1 - Stroke

Summary

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Overview and Epidemiology of Stroke

• Stroke Definition: A clinical diagnosis characterized by the sudden onset of focal neurologic deficit due to an underlying vascular pathology.
• Stroke Clinical Features: Symptoms typically include unilateral weakness (face, arm, leg), numbness, blindness in one eye, slurring of speech, dizziness, loss of balance, headache, nausea, and vomiting.
• Stroke Age Categorization:
  1. Hyperacute: 0–6 hours.
  2. Acute: 6 hours – 3 days.
  3. Subacute: 3 days – 3 weeks.
  4. Chronic: > 3 weeks.
• Stroke Mimickers: Conditions that can present like stroke include seizures (Todd’s paralysis), systemic infection, brain tumor, toxic-metabolic issues, positional vertigo, cardiac events, syncope, trauma, subdural hematoma, and herpes encephalitis.
• Todd’s Paralysis: A transient weakness occurring after a seizure, specifically after status epilepticus (>5 minutes).
• Stroke Epidemiology: A major cause of death and disability; in the Philippines, mortality is 82.8/100,000 and morbidity accounts for 81% of Daily-Adjusted Life Years (DALYs).
• Non-Modifiable Risk Factors: Increasing age, male sex, and genetics.
• Modifiable Risk Factors: Hypertension, diabetes mellitus, cardiac causes, CNS infections (e.g., TB), dyslipidemia, snoring, smoking, and physical inactivity.

Ischemic Stroke: Mechanisms and Pathophysiology

• Ischemic Stroke Core Feature: Thrombosis, often involving Virchow’s triad: blood stasis, hypercoagulability, and endothelial injury.
• Ischemic Stroke (Thrombus Formation): Endothelial injury exposes vWF → platelet GP Ib binding → platelet activation/aggregation via fibrinogen → fibrin stabilization → RBC entrapment.
• Ischemic Stroke (White vs. Red Clot): Platelet-rich clots are white clots; RBC-rich clots are red clots.
• Ischemic Stroke (Three Main Mechanisms): 1) Atherosclerosis & Thrombosis, 2) Cardioembolism, 3) Small Vessel Disease.
• Atherothrombosis (Atherosclerosis Pathogenesis): LDL oxidation attracts macrophages → Foam cells form in the intima → Atherosclerotic plaque with a thin fibrous cap forms → Luminal narrowing (stenosis).
• Atherothrombosis (Clinical Features): Often related to recurrent TIA and an intermittent "stuttering" progression of neurologic deficits.
• Atherothrombosis (Ethnic Distribution): Extracranial lesions are common in Caucasians; Intracranial lesions are common in Asians.
• Atherothrombosis (Common Sites): Plaque commonly forms at bifurcations (due to non-laminar flow and shear stress), specifically the Internal Carotid Artery (ICA), vertebral arteries, and proximal segments of the MCA, ACA, and PCA.
• Cardioembolism (Etiology): The most common risk is chronic atrial fibrillation (AF); the highest risk is valvular heart disease.
• Cardioembolism (Clinical Features): Sudden onset with neurologic deficit at its peak; no stuttering progression.
• Cardioembolism (Hemodynamic Pathophysiology): AF causes atrial-ventricular dissociation → blood stasis → Red clot formation (typically in the left atrial appendage).
• Cardioembolism (Hemorrhagic Conversion): Cardioembolic strokes have a high risk of hemorrhagic infarction (hemorrhagic conversion on CT) because red clots are friable and prone to lysis.
• Small Vessel Disease (Lacunar Stroke): Obstruction of a single small penetrating arteriole (e.g., lenticulostriate or pontine perforating arteries) supplying deep brain structures; infarct size is < 15 mm.
• Small Vessel Disease (Pathology): Primarily caused by Lipohyalinosis (wall thickening due to fibrinoid material accumulation related to hypertension).
• Ischemic Stroke (Ischemic Zones):
  1. Infarct Core: Irreversible ischemia; blood flow < 12 mL/100g/min.
  2. Penumbra: Reversibly ischemic, vulnerable tissue; the target of reperfusion therapy.
  3. Oligemia: Reduced flow (22–35 mL/100g/min) with collateral support; transient dysfunction only.

Management of Ischemic Stroke

• Stroke Diagnostics (CT Scan): Non-contrast CT is the first-line to rule out hemorrhage; it has low sensitivity for ischemia in the hyperacute stage (< 6 hours).
• Stroke Diagnostics (MRI): High sensitivity for early ischemia; DWI (Diffusion-Weighted Imaging) detects restricted water movement (cytotoxic edema) as a bright/hyperintense signal, confirmed by a low ADC (Apparent Diffusion Coefficient).
• Intravenous Thrombolysis: Administration of rTPA for candidates with stroke onset < 4.5 hours.
• Endovascular Treatment: Includes Intra-arterial thrombolysis (< 4.5 hours) and Mechanical Thrombectomy (onset < 18 hours).
• The "5H's" of Acute Stroke Care: Manage Hypoxia, Hypovolemia, Hyper/Hypotension, Hyper/Hypothermia, and metabolic derangements (Hypo/Hypernatremia or glycemia).
• Permissive Hypertension: In ischemic stroke, allow elevated BP to maintain cerebral perfusion; avoid treatment unless SBP > 220, DBP > 120, or MAP > 130.
• Hypertension Treatment Exceptions: Lower BP immediately if the patient has hypertensive encephalopathy, aortic dissection, acute renal failure, pulmonary edema, or AMI ("heart over brain").
• Secondary Prevention (Atherothrombotic/SVD): Use Antiplatelet agents (Aspirin, Clopidogrel, Cilostazol, Dipyridamole, or Triflusal).
• Secondary Prevention (Cardioembolic): Use Anticoagulants (Warfarin or NOACs/DOACs like Dabigatran, Apixaban, Rivaroxaban).
• Statins in Stroke: Used for their pleiotropic effect (plaque stabilization) rather than just lipid lowering.
• Neuroprotection: Includes Citicoline (membrane stabilization), Edaravone (oxidative stress reduction), and Cerebrolysin (neurotrophic factors).

Transient Ischemic Attack (TIA)

• TIA Definition: Transient focal neurologic deficit without evidence of infarction on neuroimaging, typically lasting < 1 hour.
• TIA Clinical Significance: Single attacks suggest embolic phenomena; recurrent attacks suggest vascular occlusion. High risk if recurrent, involving unilateral weakness, or lasting > 1 hour.
• ABCD² Score for TIA: Used to predict the 2-day stroke risk:
  • 0–3 (Low): 1% risk; outpatient possible.
  • 4–5 (Medium): 4.1% risk; admission warranted.
  • 6–7 (High): 8.1% risk; urgent admission essential.

Primary Intracerebral Hemorrhage (ICH)

• ICH Definition: Extravasation of blood into the brain parenchyma due to a ruptured arteriole; presents with sudden deficit, headache, nausea, and rapid sensorium deterioration.
• ICH Epidemiology: Incidence is higher in Asians (linked to high salt diet). Mortality and disability are higher than in ischemic stroke.
• ICH Risk Factors: Hypertension is the most important independent risk factor. Others include low LDL/TG, excessive alcohol, and sympathomimetic drugs (e.g., Phenylpropanolamine in decongestants).
• ICH Pathology: Lipohyalinosis leads to Charcot-Bouchard aneurysms (micro-aneurysms) that rupture.
• ICH Common Sites:
  1. Basal Ganglia (Putamen): 40–50% (Most common).
  2. Lobar areas (20–40%).
  3. Thalamus (10–15%).
  4. Pons (5–12%).
• ICH Diagnostics (CT Scan): Appears as hyperdensity in the hyperacute stage.
• ICH Spot Sign: Focal enhancement within the bleed on CT, indicating active bleeding and a high risk of hematoma expansion.
• ICH Treatment: Target SBP < 140 mmHg (INTERACT trial) to reduce expansion risk.
• ICH Decompression: Medical (Mannitol, Hypertonic Saline) or Surgical (Early evacuation or Hemicraniectomy for deep bleeds).

Subarachnoid Hemorrhage (SAH)

• SAH Types: 1) Traumatic (most common overall), 2) Non-traumatic (most common cause is a ruptured aneurysm).
• SAH Clinical Manifestation: "Worst headache of life" or "Thunderclap headache"; associated with photophobia, stiff neck (nuchal rigidity), and seizures.
• Non-traumatic SAH Pathology: Apoptosis of smooth muscle and degeneration of the internal elastic lamina weaken the arterial wall.
• Aneurysm Types: 1) Saccular (outpouching on one side), 2) Fusiform (circumferential enlargement of a segment).
• Aneurysm Locations:
  • ACA & ACom bifurcation: 40% (Most common).
  • MCA bifurcation: 34%.
  • MCA & PCom bifurcation: 20%.
• PCom Aneurysm Sign: Compression of Cranial Nerve III leads to a pupil-involving oculomotor nerve palsy.
• SAH Diagnostics (CT Sensitivity): 98–100% within 12 hours; drops to 57–85% by day 6.
• SAH Diagnostics (Lumbar Puncture): Performed if CT is negative but suspicion is high. Findings include Xanthochromia (yellowish CSF from bilirubin).
• Traumatic Tap vs. True SAH: In a traumatic tap, RBC count decreases from the first to the last vial; in true SAH, RBC count remains constant across vials.
• SAH Gold Standard Diagnostic: Digital Subtraction Cerebral Angiography; if negative, repeat in 7–14 days after the clot lyses.
• SAH Grading (Hunt and Hess Scale): Grades 1–2 are "good"; Grades 3–5 (drowsiness to coma) are "poor."
• SAH Complications (Re-rupture): Risk is 4% in the first 24 hours, then 1% daily. Severe disability/mortality if it occurs.
• SAH Complications (Vasospasm): Narrowing of vessels due to blood irritation; occurs within the first 21 days.
• SAH Complications (Hydrocephalus): CSF reabsorption is blocked by blood products in the subarachnoid space.
• SAH Management: Bed rest, pain control, target SBP < 150 mmHg (unsecured), and Nimodipine (60mg QID x 21 days) to prevent vasospasm.
• SAH Definitive Treatment: Clipping (surgical) or Endovascular Coiling.

Increased Intracranial Pressure (ICP) and Herniation

• Monroe-Kellie Doctrine: The skull is fixed; volume = Brain (80%) + Blood (10%) + CSF (10%). If one increases, another must decrease to keep ICP 0–20 mmHg.
• Cerebral Perfusion Pressure (CPP): CBF = MAP – ICP. Autoregulation maintains this.
• Increased ICP Features: Headache, nausea/vomiting, papilledema, and decreased sensorium.
• Cushing’s Triad (Sign of ↑ ICP): 1) Bradycardia, 2) Increased pulse pressure, 3) Increased MAP.
• Brain Herniation Types:
  1. Subfalcine: Cingulate gyrus under falx cerebri.
  2. Uncal: Temporal uncus through tentorial notch; compresses CN III.
  3. Central: Diencephalon/midbrain downward; leads to coma.
  4. Tonsillar: Cerebellar tonsils through foramen magnum; medulla compression risk.
• Kernohan Phenomenon: Uncal herniation compresses the contralateral cerebral peduncle, causing ipsilateral motor weakness (a false localizing sign) and ipsilateral CN III palsy.
• Decorticate Posturing: Arms flexed, legs extended; lesion is above the red nucleus (cortex/internal capsule).
• Decerebrate Posturing: All limbs extended; lesion is at/below the red nucleus (brainstem).
• Respiratory Patterns in ↑ ICP:
  • Cheyne-Stokes: Gradually deep/shallow with apnea.
  • Central Neurogenic Hyperventilation: Fast, deep, regular breaths.
  • Ataxic: Completely irregular/erratic (no coordination).
• ICP Emergency Management:
  1. Head elevation 15–30 degrees (midline) to enhance venous outflow.
  2. Hyperventilation (PaCO2 26–30 mmHg) for temporary vasoconstriction.
  3. Mannitol or Hypertonic Saline.
  4. Avoid hypotonic fluids.

Summary Comparisons for Exam Mastery

QA

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Overview and Epidemiology of Stroke

1. What is the clinical definition of Stroke? | Sudden focal neurologic deficit. Due to vascular pathology.
2. What are the clinical features (7) of Stroke? | 1) Unilateral weakness
   2) Numbness
   3) Blindness
   4) Slurring
   5) Dizziness
   6) Headache
   7) Nausea/Vomiting
3. What is the timeframe for a Hyperacute Stroke? | 0–6 hours.
4. What is the timeframe for an Acute Stroke? | 6 hours – 3 days.
5. What is the timeframe for a Subacute Stroke? | 3 days – 3 weeks.
6. What is the timeframe for a Chronic Stroke? | > 3 weeks.
7. List 8 Stroke Mimickers. | 1) Seizures
   2) Infection
   3) Tumor
   4) Metabolic issues
   5) Vertigo
   6) Syncope
   7) Trauma
   8) Encephalitis
8. Define the condition Todd’s Paralysis. | Transient post-seizure weakness. Occurs after status epilepticus (>5 mins).
9. What is the mortality rate of Stroke in the Philippines? | 82.8 per 100,000.
10. What is the Filipino Stroke morbidity percentage in DALYs? | 81% of DALYs. (Daily-Adjusted Life Years).
11. List 3 non-modifiable risk factors for Stroke. | 1) Increasing age
    2) Male sex
    3) Genetics
12. List 8 modifiable risk factors for Stroke. | 1) Hypertension
    2) Diabetes
    3) CNS infections
    4) Dyslipidemia
    5) Snoring
    6) Smoking
    7) Physical inactivity
    8) Cardiac causes

Ischemic Stroke: Mechanisms and Pathophysiology

13. What is the core feature of Ischemic Stroke? | Thrombosis.
14. What are the 3 components of Virchow’s triad in stroke? | 1) Blood stasis
    2) Hypercoagulability
    3) Endothelial injury
15. What is the initial step in Ischemic Stroke thrombus formation? | Endothelial injury. Exposes von Willebrand Factor (vWF).
16. To what does the platelet GP Ib bind during Ischemic Stroke clot formation? | von Willebrand Factor (vWF).
17. Which mediator is responsible for platelet aggregation in Ischemic Stroke? | Fibrinogen.
18. What is the final component that stabilizes an Ischemic Stroke clot? | RBC entrapment. (Stabilized by fibrin).
19. What is the definition of White Clots in Ischemic Stroke? | Platelet-rich clots.
20. What is the definition of Red Clots in Ischemic Stroke? | RBC-rich clots.
21. List the 3 main mechanisms of Ischemic Stroke. | 1) Atherothrombosis
    2) Cardioembolism
    3) Small vessel disease
22. What is the initial event in Atherothrombosis pathogenesis? | LDL oxidation. (Attracts macrophages).
23. Which cells are formed in the intima during Atherothrombosis? | Foam cells. (Macrophages eating oxidized LDL).
24. What is a key structural feature of a high-risk Atherosclerotic plaque? | Thin fibrous cap.
25. Describe the progression pattern of Atherothrombotic Stroke. | Stuttering progression. (Intermittent neurologic deficits).
26. Which condition often precedes Atherothrombotic Stroke? | Recurrent TIA. (Transient Ischemic Attack).
27. In which ethnic group are Extracranial lesions most common? | Caucasians.
28. In which ethnic group are Intracranial lesions most common? | Asians.
29. Where is the usual site of Atherothrombotic plaque formation? | Arterial bifurcations.
30. What is the most common bifurcation site for Internal Carotid Artery (ICA) plaque? | Carotid bifurcation. (Origin of ICA).
31. List 3 proximal segments prone to Atherothrombosis. | 1) MCA
    2) ACA
    3) PCA
32. What is the most common etiology for Cardioembolism? | Chronic atrial fibrillation.
33. Which condition carries the highest risk for Cardioembolic Stroke? | Valvular heart disease.
34. Describe the neurologic onset of Cardioembolism. | Sudden onset. (Deficit is maximal at onset).
35. What hemodynamic change in Atrial Fibrillation leads to clot formation? | Blood stasis. (Due to AV dissociation).
36. Which clot type is typically found in Cardioembolic Stroke? | Red clot. (RBC-rich).
37. What is the usual heart origin of Cardioembolic clots? | Left atrial appendage.
38. What is a common CT complication seen in Cardioembolic Stroke? | Hemorrhagic conversion. (Hemorrhagic infarction).
39. Which property of Red Clots causes hemorrhagic conversion? | Friable and prone to lysis.
40. What is the definition of a Lacunar Stroke? | Infarct size < 15 mm. (Small vessel disease).
41. Which vessels are involved in Small Vessel Disease? | Lenticulostriate or pontine perforators.
42. What is the primary pathology of Small Vessel Disease? | Lipohyalinosis.
43. What causes the wall thickening in Lipohyalinosis? | Fibrinoid material accumulation. (From chronic hypertension).
44. What is the status of the Infarct Core? | Irreversible ischemia. Blood flow < 12 mL/100g/min.
45. What is the status of the Penumbra? | Reversibly ischemic. Vulnerable tissue.
46. What is the clinical goal regarding the Penumbra? | Target of reperfusion therapy.
47. What flow rate defines Oligemia? | 22–35 mL/100g/min.
48. What is the characteristic of blood flow in Oligemia? | Transient dysfunction only. Supported by collaterals.

Management of Ischemic Stroke

49. What is the first-line diagnostic test for Stroke? | Non-contrast CT scan. (To rule out hemorrhage).
50. Describe CT scan sensitivity for hyperacute ischemia. | Low sensitivity. (< 6 hours).
51. Which MRI modality is used to detect restricted water movement? | DWI. (Diffusion-Weighted Imaging).
52. How does cytotoxic edema appear on DWI? | Bright/Hyperintense signal.
53. Which MRI modality is used to confirm the DWI signal? | Low ADC. (Apparent Diffusion Coefficient).
54. What is the time window for Intravenous Thrombolysis (rTPA)? | < 4.5 hours. From symptom onset.
55. What is the window for Intra-arterial thrombolysis? | < 4.5 hours.
56. What is the window for Mechanical Thrombectomy? | < 18 hours.
57. List the 5H's of Acute Stroke Care. | 1) Hypoxia 2) Hypovolemia 3) Hyper/Hypotension 4) Hyper/Hypothermia 5) Hyper/Hypoglycemia.
58. What is the purpose of Permissive Hypertension in Ischemic Stroke? | Maintain cerebral perfusion. To save the penumbra.
59. What SBP threshold is used to treat Permissive Hypertension? | SBP > 220 mmHg.
60. What DBP threshold is used to treat Permissive Hypertension? | DBP > 120 mmHg.
61. What MAP threshold is used to treat Permissive Hypertension? | MAP > 130 mmHg.
62. Which conditions (5) are exceptions to Permissive Hypertension? | 1) Encephalopathy
    2) Aortic dissection
    3) Renal failure
    4) Pulmonary edema
    5) AMI
63. What is the secondary prevention for Atherothrombotic Stroke? | Antiplatelet agents.
64. List 5 Antiplatelet agents used for stroke. | 1) Aspirin 2) Clopidogrel 3) Cilostazol 4) Dipyridamole 5) Triflusal.
65. What is the secondary prevention for Cardioembolic Stroke? | Anticoagulants.
66. List 3 NOACs/DOACs used for stroke prevention. | 1) Dabigatran 2) Apixaban 3) Rivaroxaban.
67. Which Vitamin K Antagonist is used for cardioembolic protection? | Warfarin.
68. What is the primary role of Statins in secondary stroke prevention? | Pleiotropic effect. (Plaque stabilization).
69. Which Neuroprotective agent is used for membrane stabilization? | Citicoline.
70. Which Neuroprotective agent is used for oxidative stress reduction? | Edaravone.
71. Which Neuroprotective agent provides neurotrophic factors? | Cerebrolysin.

Transient Ischemic Attack (TIA)

72. What is the typical duration in the definition of TIA? | < 1 hour.
73. What is the diagnostic requirement for the TIA definition? | No evidence of infarction. (On neuroimaging).
74. What is the clinical significance of Single TIA attacks? | Suggest embolic phenomena.
75. What is the clinical significance of Recurrent TIA attacks? | Suggest vascular occlusion.
76. What is the purpose of the ABCD² Score? | Predicts 2-day stroke risk.
77. What action is taken for an ABCD² Score of 0-3 (Low)? | Outpatient possible. (1% risk).
78. What action is taken for an ABCD² Score of 4-5 (Medium)? | Admission warranted. (4.1% risk).
79. What action is taken for an ABCD² Score of 6-7 (High)? | Urgent admission essential. (8.1% risk).

Primary Intracerebral Hemorrhage (ICH)

80. What is the definition of Primary ICH? | Blood extravasation into parenchyma. From a ruptured arteriole.
81. List 4 hallmark symptoms of ICH. | 1) Sudden deficit
    2) Headache
    3) Nausea
    4) Rapid sensorium drop
82. Which ethnicity is linked to higher ICH incidence? | Asians. (High salt diet).
83. What is the most important independent risk factor for ICH? | Hypertension.
84. Which lipid levels are risk factors for ICH? | Low LDL and Triglycerides.
85. Which sympathomimetic drug is a risk factor for ICH? | Phenylpropanolamine.
86. What is the underlying pathology of Primary ICH? | Lipohyalinosis.
87. What is the specific rupture site in Primary ICH? | Charcot-Bouchard aneurysms. (Micro-aneurysms).
88. What is the most common site for ICH (40-50%)? | Basal Ganglia. (Putamen).
89. Which ICH site accounts for 20-40% of cases? | Lobar areas.
90. Which ICH site accounts for 10-15% of cases? | Thalamus.
91. Which ICH site accounts for 5-12% of cases? | Pons.
92. How does ICH appear on CT scan? | Hyperdense. (Bright immediately).
93. What is the significance of the ICH Spot Sign? | Active bleeding. (Focal enhancement).
94. What risk is associated with a positive Spot Sign? | Hematoma expansion.
95. What is the systolic blood pressure target in ICH treatment? | SBP < 140 mmHg. (INTERACT trial).
96. What medical decompression is used for ICH edema? | Mannitol or Hypertonic Saline.
97. What is the surgical management for deep ICH bleeds? | Early evacuation. Or Hemicraniectomy.

Subarachnoid Hemorrhage (SAH)

98. What is the most common overall cause of SAH? | Traumatic SAH.
99. What is the most common cause of non-traumatic SAH? | Ruptured aneurysm.
100. What is the hallmark clinical manifestation of SAH? | Worst headache of life. (Thunderclap headache).
101. List 3 associated signs of SAH. | 1) Photophobia
     2) Nuchal rigidity
     3) Seizures.
102. What degenerates in non-traumatic SAH pathology? | Internal elastic lamina. (And smooth muscle apoptosis).
103. Define Saccular Aneurysm. | Outpouching on one side.
104. Define Fusiform Aneurysm. | Circumferential segment enlargement.
105. What is the most common site for Berry Aneurysms (40%)? | ACA & ACom bifurcation.
106. Where are 34% of SAH aneurysms located? | MCA bifurcation.
107. Where are 20% of SAH aneurysms located? | MCA & PCom bifurcation.
108. What is the clinical finding of a PCom Aneurysm? | Pupil-involving CN III palsy. (Oculomotor nerve).
109. What is the CT sensitivity for SAH within 12 hours? | 98–100%.
110. What is the CT sensitivity for SAH by day 6? | 57–85%.
111. When is a Lumbar Puncture indicated for suspected SAH? | Negative CT. (But high suspicion).
112. What is the diagnostic CSF finding for SAH? | Xanthochromia. (Yellowish color from bilirubin).
113. How do you distinguish a Traumatic tap vs. True SAH regarding RBCs? | Constant RBC count across vials.
114. What is the gold standard diagnostic for SAH? | Digital Subtraction Cerebral Angiography.
115. When should angiography be repeated if initially negative? | 7–14 days. (After clot lysis).
116. Which Hunt and Hess Scale grades have a "good" prognosis? | Grades 1–2.
117. Which Hunt and Hess Scale grades have a "poor" prognosis? | Grades 3–5. (Drowsiness to coma).
118. What is the risk of SAH Re-rupture in the first 24 hours? | 4% risk. (1% daily thereafter).
119. What is the timeframe for SAH Vasospasm? | First 21 days.
120. What is the pathophysiology of SAH Vasospasm? | Vessel narrowing from blood irritation.
121. What causes Hydrocephalus in SAH? | Blocked CSF reabsorption. (By blood products).
122. What is the systolic BP target for unsecured SAH? | SBP < 150 mmHg.
123. Which drug prevents SAH Vasospasm? | Nimodipine. (60mg QID x 21 days).
124. What is the surgical definitive treatment for SAH Aneurysm? | Clipping.
125. What is the endovascular definitive treatment for SAH Aneurysm? | Coiling.

Increased Intracranial Pressure (ICP) and Herniation

126. Name the 3 volume components of the Monroe-Kellie Doctrine. | 1) Brain 2) Blood 3) CSF.
127. What is the normal range for Intracranial Pressure (ICP)? | 0–20 mmHg.
128. What is the formula for Cerebral Perfusion Pressure (CPP)? | CPP = MAP – ICP.
129. List 4 clinical features of increased ICP. | 1) Papilledema 2) Headache 3) Nausea 4) Decreased sensorium.
130. What are the 3 components of Cushing’s Triad? | 1) Bradycardia 2) Incr. Pulse Pressure 3) Incr. MAP.
131. Define Subfalcine Herniation. | Cingulate gyrus under falx cerebri.
132. What is the clinical feature of Uncal Herniation? | Compresses Cranial Nerve III.
133. What is the result of Central Herniation? | Coma. (Diencephalon downward).
134. What is the danger of Tonsillar Herniation? | Medulla compression. (Through foramen magnum).
135. What is the Kernohan Phenomenon? | Ipsilateral motor weakness. (False localizing sign).
136. What is the cause of Kernohan Phenomenon? | Contralateral cerebral peduncle compression.
137. Describe Decorticate Posturing and its lesion level. | Arms flexed; Above red nucleus.
138. Describe Decerebrate Posturing and its lesion level. | All limbs extended; At/Below red nucleus.
139. Describe Cheyne-Stokes respiration. | Gradually deep/shallow with apnea.
140. Describe Central Neurogenic Hyperventilation. | Fast, deep, regular breaths.
141. Describe the Ataxic respiratory pattern. | Completely irregular/erratic.
142. What is the head elevation target for Emergency ICP management? | 15–30 degrees. (Midline).
143. What is the goal of Hyperventilation in high ICP? | PaCO2 26–30 mmHg. (Vasoconstriction).
144. Which fluids should be Avoided in high ICP management? | Hypotonic fluids.

Stroke Summary Comparisons

145. Contrast Ischemic vs ICH onset. | Ischemic: Stuttering; ICH: Sudden.
146. Contrast Ischemic vs ICH pain. | Ischemic: Painless; ICH: Severe headache.
147. Contrast Ischemic vs ICH CT appearance. | Ischemic: Hypodense; ICH: Hyperdense immediately.
148. Contrast Ischemic vs ICH BP target. | Ischemic: < 220; ICH: < 140.
149. Contrast ICH vs SAH location. | ICH: Parenchyma; SAH: Subarachnoid space.
150. Contrast ICH vs SAH pathology. | ICH: Charcot-Bouchard; SAH: Saccular.
151. Contrast ICH vs SAH hallmark diagnostics. | ICH: Spot Sign; SAH: Xanthochromia.
152. Contrast Decorticate vs Decerebrate prognosis. | Decorticate: Better than decerebrate.
153. Contrast Atherothrombotic vs Cardioembolic onset. | Atherothrombotic: Stuttering; Cardioembolic: Maximum at onset.
154. Contrast Atherothrombotic vs Cardioembolic clot. | Atherothrombotic: White; Cardioembolic: Red.
155. Contrast Atherothrombotic vs Cardioembolic secondary prevention. | Atherothrombotic: Antiplatelets; Cardioembolic: Anticoagulants.

2

Summary

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Fundamentals of Memory and Cognitive Reserve

• Memory can be divided based on duration and nature: Long-Term Memory (>1 min) is either Explicit (Declarative) or Implicit (Non-declarative).
• Explicit Memory (Declarative) includes Episodic Memory (autobiographic data managed by mesiotemporal regions) and Semantic Memory (encyclopedic knowledge regulated by temporal and parietal regions).
• Implicit Memory is demonstrated through tasks that do not require conscious processes, such as Procedural Memory (motor skills/cognitive routines) and Priming.
• Short-Term Memory (Working Memory) typically lasts 30-40 seconds and involves the Dorsolateral Prefrontal Cortex (DLPFC) and associative visual/auditory areas.
• Dementia is defined as an acquired, persistent impairment of intellectual function involving multiple spheres (Attention, Executive, Memory, Language, etc.) sufficient to interfere with daily functioning.
• Theory of Cognitive Reserve suggests that individuals with higher mental stimulation (education, occupation) can tolerate greater brain pathology before showing symptoms; thus, lower educational background is a risk factor for dementia.

Epidemiology and Classification of Dementia

• The most common causes of Dementing Disease in order of frequency are: 1) Cerebral Atrophy (Alzheimer's), 2) Multi-infarct (Vascular) Dementia, and 3) Alcoholic Dementia.
• Alzheimer’s Disease (AD) is the most common form of neurodegenerative dementia, accounting for 43% to 70% of cases.
• Classification of Dementia based on associated signs: | Category | Examples | | :--- | :--- | | Progressive (No other signs) | Alzheimer's, Frontotemporal Dementia (FTD) | | Progressive (With Neuro signs) | Huntington's (Chorea), Lewy Body (Parkinsonism), ALS complex | | Cortical Dementias | Alzheimer's, FTD, Lewy Body Dementia | | Subcortical Dementias | Parkinson Disease Dementia, Huntington's, Progressive Supranuclear Palsy |

Alzheimer’s Disease (AD): Clinical Profile

• Alzheimer’s Disease (AD) is characterized by a gradual, progressive (insidious) decline in cognitive function and activities of daily living.
• Ribot’s Law in Alzheimer's states that memories of the distant past are relatively preserved while recent information is lost first.
• Atypical Features that may suggest a diagnosis other than Alzheimer’s include early incontinence, early gait problems, movement disorders, or gaze problems.
• NINCDS-ADRDA Criteria for Alzheimer's includes: Age >40, deficits in 2+ cognitive areas with worsening, absence of disturbed consciousness, and clinical examination confirmation.
• Bedside Memory Tests used to screen for cognitive impairment in the Philippines include the MMSE (Mini-Mental State Exam) and the more sensitive MOCA (Montreal Cognitive Assessment).

Alzheimer’s Disease (AD): Biomarkers and Genetics

• Spectrum of AD progression:
  1. Pre-clinical: Changes in biomarkers (amyloid) begin without symptoms.
  2. Mild Cognitive Impairment (MCI): Noticeable memory changes but No impairment in activities of daily living.
  3. Dementia due to AD: Symptoms impair functional daily life.
• Biomarkers of Amyloid accumulation in AD include abnormal tracer retention on PET scans and Low CSF amyloid (Aβ42).
• Biomarkers of Neurodegeneration in AD include Elevated CSF Tau (total and phosphorylated), decreased FDG uptake on PET, and hippocampal/cortical atrophy on MRI.
• Apolipoprotein E (ApoE) genetics: The E4 variant (Chromosome 19) is linked to late-onset AD, while the E2 variant is protective.

Alzheimer’s Disease (AD): Pathology and Severe Signs

• Gross MRI findings in AD typically show medial temporal lobe and hippocampal atrophy with enlarged ventricles and thin gyri.
• Neuronal loss in early AD occurs significantly in the entorhinal cortex (Layer II) (leading to changes in smell) and the Nucleus Basalis of Meynert (the source of Acetylcholine).
• Neuropathological Findings in AD consist of three hallmarks:
  1. Neurofibrillary tangles: Intracellular hyperphosphorylated Tau.
  2. Neuritic (Amyloid) plaques: Extracellular Amyloid-β deposits.
  3. Granulovacuolar degeneration: At the pyramidal layer of the hippocampus.
• Severe Alzheimer's Disease symptoms include Sundowning (evening restlessness), nighttime confusion, and Frontal Release Signs (primitive reflexes like grasp, snout, and palmomental).

Frontotemporal and Lewy Body Dementias

• Frontotemporal Dementia (FTD) (Pick's Disease) is associated with Lobar Atrophy, Primary Progressive Aphasia (effortful speech), and Semantic Dementia (impaired word comprehension).
• FTD Pathology involves Argentophilic (Pick) bodies in the temporal lobes and Chromosome 17 abnormalities leading to increased Tau deposits.
• Lewy Body Dementia (LBD) is characterized by early-onset dementia accompanied by Visual Hallucinations, Parkinsonism, and Fluctuating attention.
• REM Movement Disorder (acting out dreams) is a suggestive symptom of Lewy Body Dementia or Parkinson's Disease.

Subcortical and Vascular Dementias

• Parkinson Disease Dementia (PDD) is diagnosed when cognitive deficits severe enough to impact daily life develop at least 1 year after the onset of Parkinsonism.
• Huntington’s Disease is an Autosomal Dominant (complete penetrance) disorder on Chr 4 characterized by Choreoathetosis, progressive dementia, and 39-50 CAG repeats.
• Progressive Supranuclear Palsy (PSP) presents with an early onset of falls, Vertical gaze difficulties, and "slurring/choking" bulbar problems.
• PSP Imaging hallmarks on MRI include "Mouse ears" (dorsal mesencephalon atrophy) and the "Hummingbird/Penguin sign" (midbrain atrophy with preserved pons).
• Vascular Dementia is characterized by focal neurologic signs and a stepwise progression (sudden declines with every new stroke) rather than a slow, insidious decline.

Rapidly Progressing and Reversible Dementias

• Sporadic Creutzfeldt-Jakob Disease (sCJD) is a rapidly progressive prion disease (mean survival 5 months) showing Myoclonus, EEG Periodic sharp waves, and CSF 14-3-3 protein.
• Variant Creutzfeldt-Jakob Disease (vCJD) (Mad Cow) affects a wider age range, starts with found psychiatric illness, and shows the Pulvinar sign on MRI.
• Korsakoff Amnestic Syndrome is associated with thiamine (B1) deficiency and chronic alcoholism, presenting with Confabulation (honest lying) and severe anterograde/retrograde amnesia (though never complete).
• Reversible/Curable Dementias should be ruled out first and include Vitamin B12 deficiency, Hypothyroidism, and Hepatic encephalopathy.

Pharmacotherapy and Symptomatic Management

• Cholinesterase Inhibitors used to treat Alzheimer's include:
  1. Donepezil: Long-acting, selective inhibitor of AChE.
  2. Rivastigmine: Intermediate-acting, inhibits both AChE and BuChE; available in transdermal patch.
  3. Galantamine: Dual mechanism but rarely used now due to cardiotoxicity (bradycardia/heart block).
• Memantine is an uncompetitive NMDA antagonist that treats Alzheimer's by reducing the toxic effects of excess glutamate (Excitotoxicity).
• Antipsychotics (like high-dose agents) are Not recommended for dementia-related agitation due to increased cardiovascular mortality in the elderly; low-dose Risperidone is the preferred agent if necessary.
• Agitation and Aggression in dementia, specifically FTD, are treated with SSRIs or SNRIs as first-line therapy.

Comparative Differentiating Features

• Alzheimer’s vs. Vascular Dementia: AD has an insidious, slow onset, while Vascular Dementia follows a stepwise progression linked to stroke events.
• Lewy Body Dementia (LBD) vs. Parkinson Disease Dementia (PDD): In LBD, dementia occurs before or within 1 year of parkinsonism; in PDD, dementia occurs more than 1 year after parkinsonism has been established.
• Cortical vs. Subcortical Dementia: Cortical (AD, FTD) primarily affects memory and language; Subcortical (PDD, Huntington’s, PSP) involves motor signs like chorea, rigidity, or gaze palsies earlier in the course.
• sCJD vs. vCJD MRI: sCJD often shows "cortical ribboning," while vCJD is specifically identified by the Pulvinar sign.
• Donepezil vs. Rivastigmine: Donepezil is purely an AChE inhibitor, whereas Rivastigmine inhibits both AChE and BuChE and offers a transdermal patch option for patients who forget oral meds.
• ApoE E4 vs. ApoE E2: E4 increases the risk and lowers the age of onset for AD, while E2 is the protective variant.
• Pick’s Disease (FTD) vs. Alzheimer's: Pick's focuses on early behavioral/language changes and lobar atrophy, whereas AD primarily starts with short-term memory loss and hippocampal atrophy.
• Huntington's vs. PSP: Huntington's is defined by Chorea and genetic CAG repeats, while PSP is defined by Vertical gaze palsy and early falls.
• Decorticate vs. Decerebrate Posturing: Decorticate (arms flexed) indicates a lesion above the red nucleus; Decerebrate (all limbs extended) indicates a lesion at or below the red nucleus and carries a worse prognosis.
• MMSE vs. MOCA: MMSE is a basic 30-point screen; MOCA is also 30 points but is more sensitive and specific for detecting milder forms of cognitive impairment.
• Episodic vs. Semantic Memory: Episodic is "what you did" (autobiographical); Semantic is "what things are" (encyclopedic knowledge).

QA

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Fundamentals of Memory and Cognitive Reserve

1. How is Memory divided based on duration and nature? | Long-Term and Short-Term Memory
2. What is the duration threshold for Long-Term Memory? | Greater than 1 minute
3. What are the two types of Long-Term Memory? | Explicit (Declarative) and Implicit (Non-declarative)
4. What does Explicit Memory (Declarative) include? (2) | 1) Episodic Memory
   2) Semantic Memory
5. Define Episodic Memory. | Autobiographical data
6. Which brain regions manage Episodic Memory? | Mesiotemporal regions
7. Define Semantic Memory. | Encyclopedic knowledge
8. Which brain regions regulate Semantic Memory? | Temporal and parietal regions
9. How is Implicit Memory demonstrated? | Tasks not requiring conscious processes
10. What are examples of Implicit Memory? (2) | 1) Procedural Memory
    2) Priming
11. Define Procedural Memory. | Motor skills and cognitive routines
12. What is the typical duration of Short-Term Memory (Working Memory)? | 30-40 seconds
13. Which brain region is primarily involved in Short-Term Memory? | Dorsolateral Prefrontal Cortex (DLPFC)
14. Besides the DLPFC, what other areas are involved in Short-Term Memory? | Associative visual and auditory areas
15. What is the definition of Dementia? | Acquired, persistent impairment of intellectual function
16. What functional requirement is needed for a Dementia diagnosis? | Interference with daily functioning
17. What cognitive spheres are involved in Dementia? (4+) | Attention, Executive, Memory, and Language
18. What does the Theory of Cognitive Reserve suggest? | Higher mental stimulation allows tolerance of brain pathology
19. What factors contribute to Cognitive Reserve? (2) | Education and occupation
20. Why is a lower educational background a risk factor for Dementia? | It relates to lower cognitive reserve

Epidemiology and Classification of Dementia

21. What is the most common cause of Dementing Disease? | Cerebral Atrophy (Alzheimer's Disease)
22. What is the second most common cause of Dementing Disease? | Multi-infarct (Vascular) Dementia
23. What is the third most common cause of Dementing Disease? | Alcoholic Dementia
24. What percentage of neurodegenerative dementia cases is caused by Alzheimer’s Disease (AD)? | 43% to 70%
25. What are examples of Progressive Dementia with no other signs? (2) | Alzheimer's and Frontotemporal Dementia (FTD)
26. What are examples of Progressive Dementia with neurological signs? (3) | Huntington's, Lewy Body, and ALS complex
27. List common Cortical Dementias. (3) | Alzheimer's, FTD, and Lewy Body Dementia
28. List common Subcortical Dementias. (3) | Parkinson Disease Dementia, Huntington's, and Progressive Supranuclear Palsy
29. What neurological sign is associated with Huntington's in classification? | Chorea
30. What neurological sign is associated with Lewy Body in classification? | Parkinsonism

Alzheimer’s Disease (AD): Clinical Profile

31. What is the clinical nature of Alzheimer’s Disease (AD) decline? | Gradual, progressive (insidious)
32. In what areas does Alzheimer’s Disease (AD) show decline? | Cognitive function and activities of daily living
33. Define Ribot’s Law as it pertains to Alzheimer's. | Distant memories preserved; recent information lost first
34. What are Atypical Features suggesting a non-Alzheimer's diagnosis? (4) | Early incontinence, gait problems, movement disorders, gaze problems
35. What is the age requirement for NINCDS-ADRDA Criteria in AD? | Age >40
36. According to NINCDS-ADRDA, how many cognitive areas must show deficits? | 2 or more areas
37. What must be absent for a NINCDS-ADRDA diagnosis of AD? | Disturbed consciousness
38. Name the Bedside Memory Tests used in the Philippines. (2) | MMSE and MOCA
39. Full form of MMSE. | Mini-Mental State Exam
40. Full form of MOCA. | Montreal Cognitive Assessment
41. Compare MMSE vs. MOCA in terms of sensitivity. | MOCA is more sensitive for milder impairment

Alzheimer’s Disease (AD): Biomarkers and Genetics

42. What are the three stages of the Spectrum of AD? | 1) Pre-clinical
    2) Mild Cognitive Impairment (MCI)
    3) Dementia due to AD
43. Describe the Pre-clinical stage of Alzheimer's Disease. | Biomarker changes (amyloid) without symptoms
44. Describe Mild Cognitive Impairment (MCI). | Noticeable memory changes but no daily living impairment
45. In Alzheimer's, what defines the transition to the Dementia stage? | Symptoms impair functional daily life
46. What Biomarkers indicate Amyloid accumulation in AD? (2) | Abnormal PET tracer retention and Low CSF Aβ42
47. What Biomarkers indicate Neurodegeneration in AD? (3) | Elevated CSF Tau, decreased FDG-PET, and brain atrophy
48. What specific CSF Tau findings are seen in AD? | Elevated total and phosphorylated Tau
49. What imaging finding on MRI is a Biomarker of Neurodegeneration? | Hippocampal and cortical atrophy
50. Which Apolipoprotein E variant is linked to late-onset AD? | E4 variant
51. On which chromosome is the ApoE E4 variant located? | Chromosome 19
52. Which Apolipoprotein E variant is considered protective against AD? | E2 variant

Alzheimer’s Disease (AD): Pathology and Severe Signs

53. What are the gross MRI findings in AD? (3) | Medial temporal/hippocampal atrophy, enlarged ventricles, thin gyri
54. Where does significant Neuronal loss occur in early AD? | Entorhinal cortex (Layer II)
55. What clinical change is associated with early loss in the entorhinal cortex? | Changes in smell
56. What is the significance of the Nucleus Basalis of Meynert in AD? | Source of Acetylcholine; undergoes neuronal loss
57. List the three Neuropathological hallmarks of AD. | 1) Neurofibrillary tangles
    2) Neuritic plaques
    3) Granulovacuolar degeneration
58. What are Neurofibrillary tangles composed of? | Intracellular hyperphosphorylated Tau
59. What are Neuritic (Amyloid) plaques composed of? | Extracellular Amyloid-β deposits
60. Where is Granulovacuolar degeneration found in AD? | Pyramidal layer of the hippocampus
61. Define Sundowning in severe AD. | Evening restlessness and nighttime confusion
62. What are Frontal Release Signs in severe AD? | Primitive reflexes
63. List examples of Frontal Release Signs. (3) | Grasp, snout, and palmomental reflexes

Frontotemporal and Lewy Body Dementias

64. What is the eponym for Frontotemporal Dementia (FTD)? | Pick's Disease
65. What are the clinical associations of FTD? (3) | Lobar Atrophy, Primary Progressive Aphasia, and Semantic Dementia
66. Describe Primary Progressive Aphasia in FTD. | Effortful speech
67. Describe Semantic Dementia in FTD. | Impaired word comprehension
68. What histological finding is characteristic of FTD Pathology? | Argentophilic (Pick) bodies
69. Where are Pick bodies typically located? | Temporal lobes
70. Which chromosome abnormality is linked to increased Tau in FTD? | Chromosome 17
71. What are the three core features of Lewy Body Dementia (LBD)? | Visual Hallucinations, Parkinsonism, and Fluctuating attention
72. Is the dementia in LBD early-onset or late-onset relative to AD? | Early-onset
73. What sleep disorder is suggestive of Lewy Body Dementia? | REM Movement Disorder
74. Define REM Movement Disorder. | Acting out dreams

Subcortical and Vascular Dementias

75. When is Parkinson Disease Dementia (PDD) diagnosed? | Cognitive deficits ≥1 year after parkinsonism onset
76. What is the inheritance pattern of Huntington’s Disease? | Autosomal Dominant (complete penetrance)
77. Which chromosome is affected in Huntington’s Disease? | Chromosome 4
78. What genetic abnormality is seen in Huntington’s Disease? | 39-50 CAG repeats
79. What are the clinical features of Huntington’s? (2) | Choreoathetosis and progressive dementia
80. What are the clinical hallmarks of Progressive Supranuclear Palsy (PSP)? (3) | Early falls, Vertical gaze difficulties, and bulbar problems
81. What "bulbar problems" are seen in PSP? | Slurring and choking
82. Describe the PSP Imaging hallmarks on MRI. (2) | "Mouse ears" and "Hummingbird/Penguin sign"
83. What does the Mouse ears sign in PSP represent? | Dorsal mesencephalon atrophy
84. What does the Hummingbird sign in PSP represent? | Midbrain atrophy with preserved pons
85. What are the characteristic signs of Vascular Dementia? (2) | Focal neurologic signs and stepwise progression
86. What is the mechanism of Stepwise progression in Vascular Dementia? | Sudden declines with every new stroke

Rapidly Progressing and Reversible Dementias

87. What is Sporadic Creutzfeldt-Jakob Disease (sCJD)? | Rapidly progressive prion disease
88. What is the mean survival for sCJD? | 5 months
89. List the clinical/lab findings of sCJD. (3) | Myoclonus, EEG periodic sharp waves, and CSF 14-3-3 protein
90. What is Variant Creutzfeldt-Jakob Disease (vCJD) also known as? | Mad Cow Disease
91. How does vCJD start clinically? | Profound psychiatric illness
92. What is the specific MRI sign for vCJD? | Pulvinar sign
93. What cause is associated with Korsakoff Amnestic Syndrome? | Thiamine (B1) deficiency and chronic alcoholism
94. Define Confabulation in Korsakoff Syndrome. | Honest lying
95. Describe the amnesia in Korsakoff Syndrome. | Severe anterograde/retrograde amnesia (never complete)
96. List Reversible Dementias that must be ruled out. (3) | Vitamin B12 deficiency, Hypothyroidism, and Hepatic encephalopathy

Pharmacotherapy and Symptomatic Management

97. List Cholinesterase Inhibitors used for Alzheimer's. (3) | Donepezil, Rivastigmine, and Galantamine
98. What is the mechanism and duration of Donepezil? | Long-acting, selective AChE inhibitor
99. What is the mechanism and delivery of Rivastigmine? | Inhibits AChE and BuChE; transdermal patch
100. Why is Galantamine rarely used today? | Cardiotoxicity (bradycardia/heart block)
101. What is the mechanism of Memantine? | Uncompetitive NMDA antagonist
102. How does Memantine protect the brain? | Reduces Excitotoxicity (excess glutamate)
103. Why are Antipsychotics generally not recommended in dementia? | Increased cardiovascular mortality in the elderly
104. What is the preferred low-dose Antipsychotic if necessary? | Risperidone
105. What is the first-line therapy for Agitation and Aggression in FTD? | SSRIs or SNRIs

Comparative Differentiating Features

106. Compare onset of Alzheimer’s vs. Vascular Dementia. | AD is insidious/slow; Vascular is stepwise
107. Differentiate LBD vs. PDD based on timing. | LBD: Dementia within 1 year of motor signs; PDD: Dementia >1 year after
108. Compare Cortical vs. Subcortical Dementia symptoms. | Cortical: Memory/Language; Subcortical: Early motor signs
109. Differentiate sCJD vs. vCJD on MRI. | sCJD: Cortical ribboning; vCJD: Pulvinar sign
110. Compare Donepezil vs. Rivastigmine enzyme targets. | Donepezil: AChE; Rivastigmine: AChE and BuChE
111. Compare ApoE E4 vs. ApoE E2 in AD. | E4: Risk/lower onset age; E2: Protective
112. Compare FTD vs. Alzheimer's early focus. | FTD: Behavior/Language; AD: Short-term memory
113. Compare Huntington's vs. PSP hallmarks. | Huntington's: Chorea; PSP: Vertical gaze palsy
114. Define Decorticate Posturing location. | Lesion above the red nucleus
115. Define Decerebrate Posturing location. | Lesion at or below the red nucleus
116. Which posturing carries a worse prognosis: Decorticate or Decerebrate? | Decerebrate
117. Compare Episodic vs. Semantic Memory. | Episodic: personal actions; Semantic: general facts
118. What is the diagnostic significance of 14-3-3 protein? | Rapidly progressive sCJD
119. Which AD biomarker is Low in the CSF? | Amyloid-β (Aβ42)
120. Which AD biomarker is Elevated in the CSF? | Tau (total and phosphorylated)
121. What defines Implicit Memory priming? | Unconscious exposure influencing response
122. What differentiates MCI from AD? | MCI has No impairment in activities of daily living
123. Where is Layer II of the entorhinal cortex located? | Mesiotemporal region
124. What are primitive reflexes in AD called? | Frontal Release Signs
125. In PSP, what is the midbrain finding? | Atrophy (Hummingbird/Penguin sign)
126. What is the defining movement in Huntington's? | Choreoathetosis
127. What is the hallmark EEG findings for sCJD? | Periodic sharp waves
128. What is the target of Memantine? | NMDA receptor
129. What is the consequence of excess glutamate? | Excitotoxicity
130. What Dementia shows lobar atrophy? | Frontotemporal Dementia (FTD)

3

Summary

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Neuroimaging: Noninvasive Modalities

• In MRI, use of gadolinium contrast carries a risk of Nephrogenic Systemic Fibrosis, especially in patients with preexisting renal failure; check BUN/Creatinine/GFR first.
• Cranial Magnetic Resonance Imaging (MRI) is the imaging of choice for transverse myelitis, neuropathies, and spinal cord tumors.
• Computed Tomography (CT) is superior to MRI for visualizing calcium, fat, and bone (skull base/vertebrae) and is faster for emergent herniation cases.

Neuroimaging: Invasive Modalities

• Cerebral Angiography is the invasive gold standard for visualizing small vessel tributaries and collaterals using a catheter and dye.
• CT Myelography (CTM) uses intrathecal contrast to assess spinal canal stenosis specifically when MRI is contraindicated.

Neurophysiology and Electrophysiology

• In Electromyography (EMG), a needle is used to record muscle action potentials, while Nerve Conduction Velocity (NCV) uses a probe to measure signal speed.
• Electroencephalogram (EEG) is NOT used to diagnose epilepsy (which is a clinical diagnosis) but serves as a supportive diagnostic tool.
• To enhance detection of epileptiform activity during an EEG, patients should undergo mild sleep deprivation and avoid caffeine.
• Activating procedures during an EEG include Hyperventilation (3 mins) and Photic Stimulation (strobe light at 1-20 Hz).
• In Visual Evoked Potentials (VEP), a normal response in a blind patient suggests the lesion is not in the anterior visual pathways (example: hysterical blindness).

Lumbar Puncture (LP) and CSF Analysis

• Lumbar Puncture (LP) is the gold standard for diagnosing CNS infections (bacterial, viral, or aseptic).
• The absolute contraindication for LP is infection at the site of the tap.
• Relative contraindications for LP include increased ICP (sensory changes/drowsiness), coagulopathy, and space-occupying lesions.
• The 6 layers passed during a Lumbar Puncture are: 1) Skin, 2) Subcutaneous fat, 3) Supraspinous ligament, 4) Intraspinous ligament, 5) Ligamentum flavum (1st pop), and 6) Dura (2nd pop).
• For an LP, an imaginary line between the superior iliac crests identifies the L3-L4 or L4-L5 interspaces.
• During an LP, always get baseline serum sugar first; the normal CSF-to-serum glucose ratio is approximately 0.6 (60-70%).
• The most common complication of a Lumbar Puncture (LP) is Post-dural puncture headache, which occurs when the patient is upright.
• In adults, the spinal cord typically terminates at L1, while the subarachnoid space ends at S1-S2.

Definitions of Seizure and Epilepsy

• A Seizure is an abnormal, excessive discharge of brain neurons involving hypersynchrony.
• Epileptic Seizure is a transient occurrence of signs/symptoms due to abnormal excessive or synchronous neuronal activity.
• Epilepsy is a brain disorder characterized by an enduring predisposition to generate seizures.
• Diagnosis of Epilepsy requires: 1) ≥2 unprovoked seizures >24h apart, 2) 1 unprovoked seizure with high recurrence risk (≥60%) over 10 years, or 3) an epilepsy syndrome.
• Provoked (Reactive) Seizures result from transient factors (fever, hyponatremia, trauma) in a normal brain.
• Unprovoked Seizures have no temporary/reversible factor lowering the seizure threshold.

Epidemiology and Mortality (SUDEP)

• Epilepsy is the second most common neurologic condition after headache.
• SUDEP (Sudden Unexplained Death in Epilepsy) is a diagnosis of exclusion; it often involves nocturnal generalized tonic-clonic seizures.
• Risk factors for SUDEP include Dravet syndrome, uncontrolled GTC seizures, and AED levels below therapeutic range.
• Definite SUDEP requires autopsy confirmation, while Probable SUDEP meets clinical criteria but lacks postmortem data.

Seizure Classification and Clinical Types

• Complex Partial Seizure is now termed Focal Impaired Awareness Seizure.
• Petit Mal (Absence) seizures can occur hundreds of times a day and terminate abruptly with no post-ictal confusion.
• Aura and Post-ictal confusion are hallmarks of Focal seizures that involve consciousness (Complex Partial).
• Unknown Onset (formerly Unclassified) is typical of neonatal seizures because the brain is not yet organized.

Seizure Mimics and Differentials

• Syncope is the most common mimic (44%); it is usually associated with pallor and an upright position, unlike seizures.
• Psychogenic Non-Epileptic Seizures (PNES) are often theatrical, prolonged, and occur in the presence of others with normal EEG.
• Transient Global Amnesia involves sudden loss of episodic memory in patients >50, often triggered by stress; consciousness remains intact.
• Sleep Myoclonus (Somnolescent Starts) are non-epileptic jerks occuring at sleep onset, often worsened by stress.
• Paroxysmal Abdominal Pain can be a form of epilepsy presenting as periumbilical pain with abnormal EEG.
• Benign Paroxysmal Positional Vertigo (BPPV) is diagnosed by the Dix-Hallpike maneuver and treated by the Epley maneuver.

Pharmacotherapy of Seizures

• Ethosuximide is the first-line drug for Absence Seizures.
• Valproic acid is a second-line for absence but should be avoided in pregnant females or those with PCOS due to teratogenicity and weight gain.
• Carbamazepine is the Drug of Choice (DOC) for Focal/Complex Partial Seizures.
• Carbamazepine is associated with SJS/TEN, especially in patients with the HLA-B*1502 allele (common in SE Asia).
• Carbamazepine is unique for autoinduction, inducing its own metabolism via CYP3A4.
• Oxcarbazepine carries a significant risk for hyponatremia (Serum Na+ ≤120 mEq/L can trigger seizures).

Comparative Differentiating Features

1. Compare Syncope vs. Seizure: Syncope presents with pallor and gradual onset; Seizure presents with cyanosis/normal color and sudden onset.
2. Compare Absence vs. Complex Partial: Absence lasts seconds with abrupt termination; Complex Partial lasts minutes with post-ictal confusion.
3. Compare Absence vs. Complex Partial EEG: Absence shows generalized 3 Hz spikes; Complex Partial shows focal discharges.
4. Compare CT vs. MRI speed: CT takes ~5 minutes (ideal for trauma); MRI takes 30-60 minutes.
5. Compare EEG vs. Epilepsy Diagnosis: Epilepsy is a clinical diagnosis; EEG may be normal even in confirmed epilepsy.
6. Differentiate Provoked vs. Unprovoked: Provoked has an acute systemic trigger (e.g., hyponatremia); Unprovoked suggests an enduring predisposition.
7. Compare GTC vs. Absence: GTC is convulsive with tonic-clonic phases; Absence is non-motor staring.
8. Compare Night Terrors vs. Nightmares: Night Terrors occur in NREM (deep sleep) with no memory; Nightmares occur in REM with vivid recall.
9. Compare MRI vs. X-ray energy: MRI uses nonionizing (magnets); X-ray/CT uses ionizing radiation.
10. Compare Carbamazepine vs. Oxcarbazepine side effects: Carbamazepine is linked to SJS (HLA-B*1502); Oxcarbazepine is linked to hyponatremia.
11. Compare MRA vs. Cerebral Angiogram: MRA is noninvasive (Circle of Willis); Angiogram is invasive (catheter/dye for small vessels).
12. Compare Adult vs. Pediatric Neurology Exam: Adults follow a structured head-to-toe; Pediatrics depends highly on child cooperation/observation.
13. Compare Movement Disorders vs. Seizures: Movement disorders are absent in sleep and worsen with emotion; Seizures can occur during sleep.
14. Compare Simple Focal vs. Complex Focal: Simple has preserved consciousness (Aware); Complex has impaired consciousness.
15. Compare Ethosuximide vs. Zonisamide: Ethosuximide is 1st line for absence; Zonisamide is preferred if avoiding weight gain/teratogenicity in PCOS.

QA

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1. What are the key indications (3) for a Skull X-ray? | Trauma (infants), calcifications, and bony erosions.
2. What are the hallmark findings of a Skull X-ray in infants? | Ping-pong fractures
3. In Skull X-ray, what describes benign depressed fractures in infants? | Ping-pong fractures
4. What is a significant disadvantage of using a Skull X-ray for neuroimaging? | Replaced by CT (Only shows bone/sutures).
5. What are the primary indications (2) for Cranial Ultrasound (CUS)? | Neonates and adults post-craniectomy.
6. What conditions (3) can Cranial Ultrasound (CUS) detect in neonates? | Neonatal hemorrhage, hydrocephalus, and hypoxic-ischemic events.
7. What are the advantages (4) of Cranial Ultrasound (CUS)? | Cheap, bedside, real-time, no radiation.
8. What is a disadvantage of Cranial Ultrasound (CUS) regarding anatomy? | Cannot see posterior fossa.
9. What is the imaging of choice for Trauma? | Computed Tomography (CT)
10. What are the key indications (3) for Computed Tomography (CT)? | Trauma, acute stroke, and herniation.
11. How does acute blood appear on Computed Tomography (CT)? | Hyperdense
12. How does chronic blood appear on Computed Tomography (CT)? | Hypodense
13. What is an advantage of Computed Tomography (CT) regarding speed and safety? | Fast (5 mins) and safe with metal.
14. What is the primary disadvantage of Computed Tomography (CT)? | Radiation exposure.
15. What are the key indications (3) for MRI? | White matter diseases, spinal cord tumors, and seizures.
16. What is a unique finding of MRI compared to CT? | Superior gray-white matter differentiation.
17. What are the advantages of MRI regarding energy? | No radiation (nonionizing).
18. What are the disadvantages (3) of MRI? | Slow, expensive, and dangerous with metal.
19. What are the key indications (3) for MRA? | Vascular malformations, aneurysms, and chronic headache.
20. What specific structure does MRA visualize noninvasively? | Circle of Willis
21. What is a disadvantage of MRA? | Cannot see small tributaries.
22. What are the key indications (2) for a PET Scan? | Dementia and secondary brain malignancies.
23. What does a PET Scan measure? | Metabolic activity (using radioactive glucose tracer).
24. What is the main advantage of a PET Scan? | Detects biochemical changes before structural changes.
25. What condition is a risk when using gadolinium in MRI? | Nephrogenic Systemic Fibrosis
26. Which patients are at high risk for Nephrogenic Systemic Fibrosis? | Patients with preexisting renal failure.
27. What must be checked before giving contrast for an MRI? | BUN, Creatinine, and GFR.
28. What is the imaging of choice for transverse myelitis and spinal cord tumors? | Cranial Magnetic Resonance Imaging (MRI)
29. Which modality is superior for visualizing calcium, fat, and bone? | Computed Tomography (CT)
30. What is the invasive gold standard for visualizing small vessel tributaries? | Cerebral Angiography
31. What are the techniques used in Cerebral Angiography? | Catheter and dye.
32. What modality is used for spinal canal stenosis when MRI is contraindicated? | CT Myelography (CTM)
33. What type of contrast is used in CT Myelography? | Intrathecal contrast
34. What do EMG / NCV evaluate? | Muscles and peripheral nerves.
35. What are common indications (3) for EMG / NCV? | Neuropathies, Myopathy, and Myasthenia Gravis.
36. What does an EEG evaluate? | Cerebral electrical activity.
37. What are common indications (3) for an EEG? | Epilepsy support, nonconvulsive status epilepticus, and coma.
38. What does BAER evaluate? | Brainstem auditory pathways.
39. When is BAER indicated for infants? | Infants exposed to ototoxic drugs.
40. What does SEP evaluate? | Spinal cord and brainstem sensory pathways.
41. What are common indications (3) for SEP? | Brain death, multiple sclerosis, and spinal cord lesions.
42. What does VEP evaluate? | Anterior visual pathways to occipital cortex.
43. What is VEP used to rule out in cases of vision loss? | Central/hysterical blindness
44. What is the difference between EMG and NCV technique? | EMG uses a needle; NCV uses a probe.
45. Is an EEG used to diagnose epilepsy? | No (it is a supportive tool).
46. How is epilepsy diagnosed? | Clinical diagnosis.
47. What should patients do to enhance an EEG? | Mild sleep deprivation and avoid caffeine.
48. What are the activating procedures during an EEG? (2) | Hyperventilation and Photic Stimulation.
49. What does a normal VEP response in a blind patient suggest? | Lesion is not in anterior visual pathways (e.g. hysterical blindness).
50. What is the gold standard for diagnosing CNS infections? | Lumbar Puncture (LP)
51. What is the absolute contraindication for a Lumbar Puncture? | Infection at the site of the tap.
52. What are the relative contraindications for LP? (3) | Increased ICP, coagulopathy, and space-occupying lesions.
53. Enumerate the 6 layers passed during a Lumbar Puncture. | 1) Skin, 2) Subcutaneous fat, 3) Supraspinous, 4) Intraspinous, 5) Ligamentum flavum, 6) Dura.
54. Which layer corresponds to the "first pop" during an LP? | Ligamentum flavum
55. Which layer corresponds to the "second pop" during an LP? | Dura
56. What landmark is used to identify the LP interspace? | Line between superior iliac crests.
57. Which interspaces are targeted during an LP? | L3-L4 or L4-L5.
58. What must be obtained before checking CSF glucose during an LP? | Baseline serum sugar
59. What is the normal CSF-to-serum glucose ratio? | Approximately 0.6 (60-70%).
60. What is the most common complication of a Lumbar Puncture? | Post-dural puncture headache
61. When does a Post-dural puncture headache typically occur? | When the patient is upright.
62. Where does the spinal cord terminate in adults? | L1
63. Where does the subarachnoid space end? | S1-S2
64. Define a Seizure. | Abnormal discharge of brain neurons involving hypersynchrony.
65. Define an Epileptic Seizure. | Transient occurrence due to abnormal excessive/synchronous activity.
66. Define Epilepsy. | Brain disorder with an enduring predisposition to seizures.
67. Enumerate the criteria (3) for the Diagnosis of Epilepsy. | 1) ≥2 unprovoked >24h apart
    2) 1 unprovoked with high recurrence risk
    3) Epilepsy syndrome.
68. What causes Provoked (Reactive) Seizures? | Transient factors (fever, hyponatremia) in a normal brain.
69. What characterizes Unprovoked Seizures? | No temporary/reversible factor lowering the threshold.
70. What is the second most common neurologic condition? | Epilepsy
71. What does SUDEP stand for? | Sudden Unexplained Death in Epilepsy
72. What type of seizure is often involved in SUDEP? | Nocturnal generalized tonic-clonic seizures.
73. Enumerate the risk factors (3) for SUDEP. | 1) Dravet syndrome
    2) Uncontrolled GTC seizures
    3) Sub-therapeutic AED levels.
74. What is required to confirm Definite SUDEP? | Autopsy confirmation.
75. What defines Probable SUDEP? | Meets clinical criteria; lacks postmortem data.
76. What is the involvement and consciousness of a Focal (Aware) seizure? | One hemisphere; Intact consciousness.
77. What is the involvement and consciousness of a Focal (Impaired Awareness) seizure? | One hemisphere; Lost/Impaired consciousness.
78. What are Automatisms? | Involuntary behaviors like frothing/salivation (seen in Focal Impaired Awareness).
79. What is the involvement and consciousness of a Generalized Motor seizure? | Bilateral hemispheres; Lost/Impaired consciousness.
80. Enumerate the Generalized Motor subtypes (3). | Tonic, Clonic, Atonic.
81. What characterizes an Absence (Non-motor) seizure? | Staring spells with lost consciousness for seconds.
82. What is the classic EEG finding for Absence seizures? | 3 Hz spike-and-wave.
83. What is the new term for Complex Partial Seizure? | Focal Impaired Awareness Seizure.
84. How many times a day can Petit Mal (Absence) seizures occur? | Hundreds of times.
85. What are the hallmarks of Focal seizures that involve consciousness? | Aura and Post-ictal confusion.
86. Why are neonatal seizures typically classified as Unknown Onset? | The brain is not yet organized.
87. What is the most common mimic of a Seizure? | Syncope (44%).
88. How do Syncope and seizure differ in appearance? | Syncope has pallor; seizure has cyanosis or normal color.
89. What characterizes Psychogenic Non-Epileptic Seizures (PNES)? | Theatrical, prolonged, presence of others, normal EEG.
90. What is Transient Global Amnesia? | Sudden loss of episodic memory in patients >50, triggered by stress.
91. What are Sleep Myoclonus (Somnolescent Starts)? | Non-epileptic jerks occuring at sleep onset.
92. How does Paroxysmal Abdominal Pain present? | Periumbilical pain with abnormal EEG.
93. What maneuver diagnoses BPPV? | Dix-Hallpike maneuver
94. What maneuver treats BPPV? | Epley maneuver
95. What is the first-line drug for Absence Seizures? | Ethosuximide
96. Why avoid Valproic acid in pregnant females or those with PCOS? | Teratogenicity and weight gain.
97. What is the Drug of Choice for Focal / Complex Partial Seizures? | Carbamazepine
98. What allele is associated with SJS/TEN risk in Carbamazepine? | HLA-B*1502
99. What metabolic phenomenon is unique to Carbamazepine? | Autoinduction (via CYP3A4).
100. What is a significant electrolyte risk with Oxcarbazepine? | Hyponatremia
101. Compare Syncope vs. Seizure onset and color. | Syncope: Gradual onset, pallor.
     Seizure: Sudden onset, cyanosis/normal color.
102. Compare Absence vs. Complex Partial duration and termination. | Absence: Seconds, abrupt termination.
     Complex Partial: Minutes, post-ictal confusion.
103. Compare Absence vs. Complex Partial EEG findings. | Absence: Generalized 3 Hz spikes.
     Complex Partial: Focal discharges.
104. Compare CT vs. MRI in terms of speed. | CT: ~5 minutes.
     MRI: 30-60 minutes.
105. Compare MRI vs. X-ray in terms of energy used. | MRI: Nonionizing (magnets).
     X-ray/CT: Ionizing radiation.
106. Compare Carbamazepine vs. Oxcarbazepine side effects. | Carbamazepine: SJS.
     Oxcarbazepine: Hyponatremia.
107. Compare MRA vs. Cerebral Angiogram invasiveness. | MRA: Noninvasive (Circle of Willis).
     Angiogram: Invasive (Catheter/Dye).
108. Compare Night Terrors vs. Nightmares. | Night Terrors: NREM (no memory).
     Nightmares: REM (vivid recall).
109. Compare Simple Focal vs. Complex Focal consciousness. | Simple: Preserved/Aware.
     Complex: Impaired consciousness.
110. Compare Movement Disorders vs. Seizures in sleep. | Movement Disorders: Absent in sleep.
     Seizures: Can occur in sleep.
111. Compare Ethosuximide vs. Zonisamide role in absence. | Ethosuximide: 1st line.
     Zonisamide: Preferred to avoid weight gain/PCOS issues.
112. Differentiate Provoked vs. Unprovoked seizures. | Provoked: Systemic trigger.
     Unprovoked: Enduring predisposition.
113. Compare GTC vs. Absence motor features. | GTC: Convulsive (tonic-clonic).
     Absence: Non-motor (staring).
114. Compare EEG vs. Epilepsy diagnosis relationship. | Epilepsy is clinical; EEG is supportive (can be normal).
115. Compare Adult vs Pediatric neurology exam. | Adult: Structured head-to-toe.
     Pediatric: Observation and cooperation.
116. What does ping-pong fracture refer to? | Benign depressed fractures in infants.
117. What does metabolic activity assessment in PET scan utilize? | Radioactive glucose tracer.
118. What is the "first pop" during the 6 layers of LP? | Ligamentum flavum
119. What is the "second pop" during the 6 layers of LP? | Dura
120. Which AED metabolism involves CYP3A4 autoinduction? | Carbamazepine

4

Summary

text

Topic: Approach to Epilepsy & Automatisms

Topic: Epileptic Syndromes - Classifications & Neonatal Fits

Topic: Generalized Epilepsies (Absence, JME, Jeavons)

Topic: Focal Epilepsies & Special Syndromes

Topic: Status Epilepticus (SE)

Topic: Involuntary Movement Disorders - Tremors

Topic: Chorea & Other Hyperkinetic Disorders

Differentiating Similar Entities (Comparison Review)

• CAE vs. JAE: Childhood Absence Epilepsy occurs in school-age children (peaks 6-7 years) and is very frequent; Juvenile Absence Epilepsy occurs in adolescence and carries a much higher risk (80%) of developing generalized tonic-clonic seizures.
• BINC vs. BFNC: Benign Idiopathic Neonatal Convulsions (BINC) occurs on day 5 ("5th day fits") and is usually self-limited with low recurrence; Benign Familial Neonatal Convulsions (BFNC) occurs earlier (day 2-3) and has a significant family history (Chromosome 20).
• Essential vs. Parkinsonian Tremor: Essential tremor is bilateral, postural/action-based, and responds to alcohol; Parkinsonian tremor is unilateral at onset, a resting tremor, and does not respond to alcohol but responds to L-Dopa.
• Chorea vs. Athetosis: Chorea involves rapid, jerky, "dance-like" involuntary movements; Athetosis involves slow, writhing, twisting contortions, typically affecting the distal upper limbs.
• Hemiballismus vs. Chorea: Hemiballismus involves violent, large-amplitude flinging movements of one side of the body; Chorea involves smaller, semi-purposeful "dancing" movements.
• Common AED Side Effects: Carbamazepine is associated with SJS/TEN (Check HLA-B*1502); Oxcarbazepine is notorious for causing hyponatremia; Valproic Acid causes weight gain and PCOS.
• Syncope vs. Seizure (from source 1.1 clues): Syncope usually has a gradual onset with pallor; Seizures are sudden and may involve cyanosis and "automatisms."
• Night Terrors vs. Seizures: Night Terrors occur in NREM sleep and the patient has no memory; Seizures can be confirmed with EEG and often have post-ictal states.
• Idiopathic vs. Symptomatic: Idiopathic means the patient is otherwise normal with a presumed genetic cause; Symptomatic means there is a clear structural brain insult (tumor, stroke, or old injury).
• Status Epilepticus Pharmacology: Diazepam is the fast-acting "fire extinguisher" to stop the seizure; Phenytoin/Phenobarbital are the "stabilizers" given to prevent the fire from restarting (maintenance).
• Wilson's Disease vs. Sydenham's: Wilson's is a metabolic/genetic copper disorder with liver involvement; Sydenham's is an autoimmune post-streptococcal complication with no liver involvement.
• Cerebellar vs. Movement Disorders: Cerebellar lesions cause intention tremors and ataxia (negative and positive features); Basal Ganglia disorders cause resting tremors, chorea, or rigidity.

QA

Topic: Approach to Epilepsy & Automatisms

1. Define Automatisms in terms of motor activity. | Coordinated, repetitive, motor activities.
2. Automatisms arise from what structure when cortical control is disrupted? | Brainstem central pattern generators.
3. List the types of Automatisms (5). | 1) Orofacial
   2) Manual/Gestural
   3) Hypermotor
   4) Ictal Speech
   5) Dystonic Posturing.
4. What are examples of Orofacial Automatisms? | Lip-smacking.
5. What are examples of Manual/Gestural Automatisms? | Picking or rubbing.
6. What are examples of Hypermotor Automatisms? | Pelvic thrusting or cycling.
7. Which lobe is highly sensitive to Orofacial automatisms? | Temporal Lobe.
8. Which lobe is highly specific for Hypermotor behavior? | Frontal Lobe.
9. Define "Release phenomena" in focal impaired awareness seizures. | Primitive brain centers take over.
10. In seizure management, when is 911 Emergency Criteria met regarding duration? | Seizure lasts >5 minutes.
11. List the patient conditions that trigger 911 Emergency Criteria during a seizure (3). | 1) First-time event
    2) Pregnant
    3) Diabetic.
12. In what environmental setting should 911 Emergency Criteria be called for a seizure? | Occurs in water.

Topic: Epileptic Syndromes - Classifications & Neonatal Fits

13. Define Symptomatic Epilepsy based on cause. | Known cause (e.g., tumor/bleed).
14. Define Cryptogenic Epilepsy. | Presumed genetic, cause unidentifiable.
15. Define Idiopathic Epilepsy imaging and function. | Normal imaging and function.
16. What is the alternative name for Benign Idiopathic Neonatal Convulsions (BINC)? | "Fifth Day Fits".
17. On what day of life does Benign Idiopathic Neonatal Convulsions (BINC) typically occur? | Day 5 of life.
18. What are the potential links/causes for Benign Idiopathic Neonatal Convulsions (BINC) (2)? | Low CNS zinc or Rotavirus.
19. On what days of life does Benign Familial Neonatal Convulsions (BFNC) occur? | Days 2-3 of life.
20. What is the inheritance and chromosome for Benign Familial Neonatal Convulsions (BFNC)? | Autosomal Dominant, Chromosome 20.
21. What is the future seizure risk for Benign Familial Neonatal Convulsions (BFNC)? | 11-16%.
22. Which syndrome has a higher risk of future seizures, BINC vs. BFNC? | BFNC.
23. What is the Drug of Choice for neonatal seizures? | Phenobarbital.
24. Why is Phenobarbital the Drug of Choice for neonates? | Fewer side effects than others.
25. What is the most common side effect of Phenobarbital in neonates? | Rashes.

Topic: Generalized Epilepsies (Absence, JME, Jeavons)

26. What is the peak age of onset for Childhood Absence Epilepsy (Pyknolepsy)? | Age 6-7 years.
27. Describe the clinical features of Childhood Absence Epilepsy (CAE) (2). | Staring spells and eyelid myoclonia.
28. What is the characteristic EEG pattern for Childhood Absence Epilepsy (CAE)? | 3-Hz spike-and-slow-wave.
29. What is the age of onset for Juvenile Absence Epilepsy (JAE)? | 8-26 years.
30. What percentage of Juvenile Absence Epilepsy (JAE) patients develop Tonic-Clonic seizures? | 80% of patients.
31. What is the Drug of Choice for Absence seizures? | Ethosuximide.
32. List the side effects to monitor when using Valproic Acid (3). | 1) PCOS
    2) Hepatotoxicity
    3) Weight gain.
33. Why is Valproic Acid avoided in women of child-bearing age? | Teratogenic (Neural Tube Defects).
34. Describe the clinical trigger and sign of Jeavons Syndrome. | Eyelid jerking triggered by eye closure.
35. What is the EEG finding in Jeavons Syndrome? | 3-6 Hz generalized polyspike-and-wave.
36. What sensitivity is common to all Jeavons Syndrome patients? | Photosensitivity.
37. What is the most common idiopathic epilepsy syndrome in adolescents? | Juvenile Myoclonic Epilepsy (JME).
38. What is the peak age and chromosome for Juvenile Myoclonic Epilepsy (JME)? | Peak age 15; Chromosome 6.
39. List the SSAP triggers for Juvenile Myoclonic Epilepsy (JME). | 1) Stress
    2) Sleep lack
    3) Alcohol
    4) Photic stimulation.
40. What is the 80% effective Drug of Choice for JME? | Valproic Acid.
41. What is the first-line alternative to Valproic Acid for Juvenile Myoclonic Epilepsy (JME)? | Levetiracetam.

Topic: Focal Epilepsies & Special Syndromes

42. What is the most common childhood epilepsy syndrome? | Benign Rolandic Epilepsy (BCECTS).
43. What is the peak age for Benign Rolandic Epilepsy (BCECTS)? | 8-9 years.
44. Describe the nocturnal symptoms of Benign Rolandic Epilepsy (BCECTS) (3). | 1) Facial twitching
    2) Drooling
    3) Speech arrest.
45. What is the first-line Drug of Choice for Benign Rolandic Epilepsy? | Carbamazepine.
46. Define Rasmussen’s Encephalitis in terms of laterality and age. | Unilateral inflammatory disease (Age 1-14).
47. What structural change occurs in Rasmussen’s Encephalitis? | Progressive hemisphere atrophy.
48. Which antibody is associated with Rasmussen’s Encephalitis? | GluR3 auto-antibodies.
49. What is the most effective surgery for Rasmussen’s Encephalitis? | Hemispherectomy.
50. Describe Landau-Kleffner Syndrome (Acquired Epileptic Aphasia). | Sudden failure of expressive speech.
51. What brain region is affected in Landau-Kleffner Syndrome? | Broca’s area.
52. What EEG finding is seen in Landau-Kleffner Syndrome? | Sleep-activated discharges.
53. List triggers for Reflex Seizures (4). | 1) Musicogenic
    2) Reading
    3) Hot water
    4) Photic stimulation.
54. What desensitization strategy is used for TV-provoked reflex seizures? | Watch from 2 meters away.

Topic: Status Epilepticus (SE)

55. Provide the operational definition of Status Epilepticus. | Seizures persisting >5 minutes.
56. What is the classic definition of Status Epilepticus duration? | 30 minutes.
57. What is the 1st line treatment for Status Epilepticus? | Short-acting Benzodiazepines (Diazepam/Lorazepam).
58. When is Lorazepam preferred over Diazepam in Status Epilepticus? | Cardiopulmonary issues exist.
59. List 2nd line long-acting AEDs for Status Epilepticus (4). | 1) Phenobarbital
    2) Phenytoin
    3) Valproic Acid
    4) Levetiracetam.
60. In Status Epilepticus management, if 4 drugs stop the seizure, what is the maintenance dose rule? | All 4 must be maintained.
61. What treatment is required for Refractory Status Epilepticus? | Midazolam drip or induced coma.
62. Which drugs are used for induced coma in Refractory Status Epilepticus? | Pentobarbital or Propofol.
63. What is a common cause of death in Status Epilepticus? | Intractable metabolic acidosis.
64. Why avoid antihypertensives during a Status Epilepticus attack? | Blood pressure may crash post-ictally.
65. Which nutritional supplements are given in Status Epilepticus supportive care? | Thiamine (Vit B1) and Dextrose.

Topic: Involuntary Movement Disorders - Tremors

66. Movement Disorders (Dyskinesias) are generally absent during what state? | Sleep.
67. Describe the clinical presentation of Parkinsonian Tremor. | Resting "pill-rolling" tremor, unilateral.
68. What physical signs accompany Parkinsonian Tremor? | Decreased facial expression, cogwheel rigidity.
69. What is the most common type of Tremor? | Essential Tremor.
70. Define the nature of Essential Tremor. | Postural or action tremor.
71. What are the common head motions in Essential Tremor? | "Yes-yes" or "No-no" motions.
72. List the treatments for Essential Tremor (2). | Beta Blockers (Propranolol) and Alcohol.
73. Alcohol is beneficial for which tremor type? | Essential Tremor.
74. Define Cerebellar Tremor (Intention tremor). | Slow tremor at end of purposeful movement.
75. List signs accompanying Cerebellar Tremor (3). | Ataxia, Nystagmus, and Dysarthria.
76. Define Titubation. | Specific head tremor from cerebellum.
77. How is Dystonic Tremor relieved? | Geste antagoniste (touching body part).
78. What characterizes Dystonic Tremor muscle contractions? | Sustained involuntary muscle contractions.
79. Define Orthostatic Tremor frequency and trigger. | >12 Hz; occurs upon standing.
80. When does Orthostatic Tremor disappear? | Once weight-bearing stops.
81. Describe the onset of Psychogenic Tremor. | Abrupt onset.
82. What makes Psychogenic Tremor disappear? | When the patient is distracted.

Topic: Chorea & Other Hyperkinetic Disorders

83. Sydenham’s Chorea is a feature of which infection? | Rheumatic Fever (GABHS).
84. What brain area is targeted in Sydenham’s Chorea? | Basal Ganglia (caudate and putamen).
85. Define "Milkmaid grip" in Sydenham’s Chorea. | Relapsing grip.
86. Define "Chameleon tongue" in Sydenham’s Chorea. | Darting tongue.
87. Define "Piano hand" in Sydenham’s Chorea. | Flowing finger movements.
88. What is the Drug of Choice for Sydenham’s Chorea? | Haloperidol.
89. What is the antibiotic regimen for Sydenham’s Chorea? | 10-day Penicillin then long-term prophylaxis.
90. Define Wilson’s Disease genetics. | Autosomal Recessive (Chromosome 13, ATP7B).
91. What metal accumulates in Wilson’s Disease? | Toxic copper.
92. List the diagnostic findings for Wilson’s Disease (3). | 1) Low ceruloplasmin
    2) High urine copper
    3) Kayser-Fleischer rings.
93. What is the role of Penicillamine in Wilson’s Disease? | Remove copper (Chelator).
94. What is the role of Zinc Acetate in Wilson’s Disease? | Prevent copper absorption.
95. Define Chorea Gravidarum. | Chorea occurring during pregnancy.
96. History of what condition correlates with Chorea Gravidarum? | Sydenham’s Chorea.
97. Describe RBC appearance in Neuroacanthocytosis. | "Starlike" or "thorny" (Acanthocytes).
98. What symptoms are associated with Neuroacanthocytosis? | Axonal neuropathy and chorea.
99. Define Huntington’s Disease clinical triad. | Chorea, dementia, and cortical atrophy.

Topic: Comparison Review

100. Compare CAE vs. JAE in terms of seizure frequency. | CAE is very frequent.
101. Which has a higher Tonic-Clonic risk, CAE vs. JAE? | JAE (80% risk).
102. Compare BINC vs. BFNC onset timing. | BINC (Day 5); BFNC (Day 2-3).
103. Compare Essential vs. Parkinsonian Tremor symmetry. | Essential (bilateral); Parkinsonian (unilateral onset).
104. Compare response to alcohol in Essential vs. Parkinsonian Tremor. | Essential responds; Parkinsonian does not.
105. Compare Chorea vs. Athetosis movements. | Chorea (rapid/jerky); Athetosis (slow/writhing).
106. Compare Hemiballismus vs. Chorea movement amplitude. | Hemiballismus (violent flinging); Chorea (smaller dancing).
107. Which drug is associated with SJS/TEN and requires HLA-B*1502 testing? | Carbamazepine.
108. What metabolic side effect is Oxcarbazepine notorious for? | Hyponatremia.
109. Compare Syncope vs. Seizure onset and color. | Syncope (gradual/pallor); Seizure (sudden/cyanosis).
110. Compare Night Terrors vs. Seizures memory. | Night Terrors (no memory); Seizures (post-ictal/EEG changes).
111. Compare Idiopathic vs. Symptomatic etiology. | Idiopathic (genetic); Symptomatic (structural insult).
112. Compare Diazepam vs. Phenytoin roles in Status Epilepticus. | Diazepam (fire extinguisher); Phenytoin (stabilizer).
113. Compare Wilson's Disease vs. Sydenham's liver involvement. | Wilson's (liver involved); Sydenham's (no liver involvement).
114. Compare Cerebellar vs. Basal Ganglia tremors. | Cerebellar (intention); Basal Ganglia (resting).
115. What are the manual automatisms in focal impaired awareness seizures? | Picking or fumbling.
116. What is the peak age for Childhood Absence Epilepsy? | 6-7 years.
117. Which chromosome is linked to Juvenile Myoclonic Epilepsy (JME)? | Chromosome 6.
118. Which chromosome is linked to Benign Familial Neonatal Convulsions (BFNC)? | Chromosome 20.
119. What is the most common idiopathic epilepsy in adolescents? | Juvenile Myoclonic Epilepsy (JME).
120. What is "Acquired Epileptic Aphasia"? | Landau-Kleffner Syndrome.

5

Summary

### **Topic: Seizure Classification (2025 ILAE Classification)**

- In accordance with the 2025 ILAE Classification, **<font color="red">Focal Seizures</font>** are now more clearly distinguished into **<font color="red">Focal Seizures with Preserved Awareness (FPC)</font>** and **<font color="red">Focal Seizures with Impaired Awareness (FIC)</font>**.
- The formula for **<font color="red">Seizure Clinical Documentation</font>** is defined as: **Class + Classifier + Basic Descriptor: Expanded Descriptor**.
- **<font color="red">Focal Preserved Consciousness Seizure (FPC)</font>**, formerly known as "Simple Partial Seizure," occurs while awareness and consciousness are fully preserved.
- **<font color="red">Jacksonian March</font>** is a type of Focal Motor (Frontal) seizure characterized by tonic/clonic contractions spreading from distal to proximal muscles (e.g., hand → arm → face).
- **<font color="red">Adversive Seizures</font>** involve the sustained turning of the head and eyes to the side opposite the seizure focus.
- **<font color="red">Somatosensory Seizures</font>** originate in the parietal lobe and manifest as numbness or tingling contralateral to the focus.
- **<font color="red">Visual Seizures</font>** originate in the occipital lobe and manifest as flashes of light, sparks, or darkness.
- **<font color="red">Olfactory Seizures (Uncinate Fits)</font>** originate in the medial temporal lobe/uncus and are characterized by unpleasant or foul odors.
- **<font color="red">Temporal Lobe Aura</font>** consists of subjective warnings that precede impairment, such as déjà vu, fear, or a rising epigastric sensation.
- **<font color="red">Focal-to-Bilateral Tonic-Clonic Seizure (FBTC)</font>**, formerly "Secondarily Generalized," is differentiated from primary generalized seizures by a clear focal onset (aura or focal motor sign).
- **<font color="red">Typical Absence Seizures (TA)</font>** are brief (2–10s) lapses of consciousness with a classic **3-Hz spike-and-wave** EEG pattern, often triggered by hyperventilation.
- **<font color="red">Atypical Absence Seizures (AA)</font>** have a slower onset/offset and show a slower (1–2 Hz) or irregular spike-and-wave EEG pattern.
- **<font color="red">Generalized Tonic-Clonic Seizures (GTC)</font>** follow a sequence of a **Tonic Phase** (stiffening, cyanosis, epileptic cry) followed by a **Clonic Phase** (rhythmic jerking, autonomic activation).
- **<font color="red">Generalized Atonic Seizures (GA)</font>** are also known as "drop attacks" due to the sudden loss of postural tone.
- **<font color="red">Todd’s Paralysis</font>** is a focal neurological deficit (such as weakness) that occurs in the postictal period following a seizure.

### **Topic: Tremors & Movement Disorders**

- **<font color="red">Resting Tremor</font>** occurs at rest and is a hallmark of **Parkinsonian syndromes** and drug-induced tremors from dopamine blockers like haloperidol.
- **<font color="red">Contraction Tremor</font>** is worse during active muscle contraction (e.g., making a tight fist) and is seen in essential tremor and cerebellar disorders.
- **<font color="red">Posture (Sustension) Tremor</font>** occurs when arms are elevated against gravity (e.g., 'birdwing' position), common in essential and physiologic tremors.
- **<font color="red">Intention Tremor</font>** worsens as the patient's finger approaches a target during a finger-to-nose test, typically indicating **cerebellar disorders**.
- **<font color="red">Orthostatic Tremor</font>** is characterized by fast (>12 Hz) rhythmic muscle contractions in the legs and trunk immediately upon standing.
- **<font color="red">Essential Tremor</font>** is a common tremor that may affect the voice (quiver) or head (nodding), usually occurs on its own, and disappears during sleep.
- **<font color="red">Asterixis</font>** is a "flapping tremor" associated with excessive alcohol consumption, alcohol withdrawal, or hepatic encephalopathy.

### **Topic: Huntington’s Disease (HD)**

| Feature | Huntington's Disease (HD) |
| :--- | :--- |
| **Pathogenesis** | Autosomal Dominant; **HTT gene** mutation on Chromosome 4; **CAG repeats**. |
| **Pathology** | **Neuronal loss** in Basal Ganglia (Caudate/Putamen) and Cerebral Cortex. |
| **Clinical Triad** | **Chorea**, **Dementia**, and **Psychiatric disorders** (primarily Depression). |
| **Diagnosis** | Family history, Genetic testing (>40 repeats), MRI/CT showing caudate atrophy. |
| **Treatment** | **Tetrabenazine** (FDA approved DOC); supportive care; neuroleptics. |

- **<font color="red">CAG Nucleotide Repeats</font>** in the HTT gene determine disease status: Healthy (10-35 repeats), Huntington's (40 or more repeats), and Juvenile HD (over 55 repeats).
- **<font color="red">Huntington’s Disease Prevalence</font>** is now equal between males and females, with the highest occurrence in Western European descent.
- **<font color="red">Depression</font>** is noted as the most common and often the **first symptom** of psychiatric involvement in Huntington's Disease.
- **<font color="red">Westphal Variant (Juvenile HD)</font>** is characterized by an onset < 20 years, seizures, and rigid/contracted muscles rather than pure chorea.
- **<font color="red">Tetrabenazine</font>** is the drug of choice for HD-related chorea; it works as a **VMAT2 inhibitor**, depleting dopamine.
- **<font color="red">Monro-Kellie Doctrine</font>** explains that in HD, lateral ventricles enlarge to fill the space of atrophied brain tissue (hydrocephalus ex vacuo), without increasing ICP.
- **<font color="red">Pneumonia</font>** is the most common cause of death in late-stage Huntington’s Disease.
- **<font color="red">UHDRS (Unified Huntington’s Disease Rating Scale)</font>** is the standard tool for scoring physical progression based on motor, cognitive, behavior, and functional ability.
- **<font color="red">Grade 4 HD</font>** is the most severe neuropathologic grade, where the medial surface of the caudate nucleus becomes concave on imaging.

### **Topic: Dystonia & Other Movements**

- **<font color="red">Athetosis</font>** is characterized by slow, writhing, involuntary movements, primarily affecting the **distal parts** (fingers/arms); often caused by lesions in the **corpus striatum**.
- **<font color="red">Dystonia</font>** involves **sustained** muscle contractions causing twisting, repetitive movements, and abnormal postures, typically affecting **proximal muscles** (neck, trunk).
- **<font color="red">Meige’s Syndrome (Cranial Dystonia)</font>** is the combination of **blepharospasmodic contractions** (eye) and **oromandibular dystonia** (jaw).
- **<font color="red">Dystonia of Panay (Lubag/XDP)</font>** is a sex-linked recessive disorder (TAF1 gene at Xq13.1) unique to **adult Filipino men** with ancestry from Panay Island.
- **<font color="red">Lubag (XDP)</font>** manifests in adult males as progressive torsion dystonia in the first 10-15 years, later replaced by parkinsonian features.
- **<font color="red">Dystonia Musculorum Deformans (DMD)</font>** is a rare, childhood-onset generalized dystonia associated with the **DYT1 gene**.
- **<font color="red">Hemiballismus</font>** is characterized by violent, rapid, unilateral flinging movements caused by a lesion in the **subthalamic nucleus of Luysii**.
- **<font color="red">Dyskinesias</font>** are purposeless, uncontrolled movements that worsen with emotions, are **absent during sleep**, and present only while awake.

### **Topic: Headaches**

- **<font color="red">Pain-Sensitive Cranial Structures</font>** include the cranial sinuses, arteries of the dura mater, and cranial nerves **V, VII, IX, and X**.
- **<font color="red">Headache Danger Signals</font>** in adults include sudden onset of new severe pain ("thunderclap"), progressively worsening pain, and associated memory loss or visual disturbance.
- **<font color="red">Migraine without Aura</font>** requires at least 5 attacks, lasting 4-72 hours, with characteristics like unilateral location, pulsating quality, and nausea/vomiting.
- **<font color="red">Migraine with Aura</font>** requires at least 2 attacks where aura symptoms (usually flashing lights or reversible sensory changes) develop over 4 minutes and last <60 minutes.
- **<font color="red">Episodic Tension Headache</font>** is characterized by bilateral, non-pulsating pain ("pressure") that is NOT aggravated by physical activity and lacks nausea.
- **<font color="red">Chronic Tension Headache</font>** is defined by a headache frequency of **≥ 15 days per month** for at least 6 months.
- **<font color="red">Cluster Headache</font>** attacks are severe, unilateral (ipsilateral), and associated with **conjunctival injection, lacrimation, rhinorrhea**, and **ptosis**.
- **<font color="red">Headache Neuroimaging</font>** (CT/MRI) is indicated if the headache is focal, sudden, progressive, or associated with sensory depression.
- **<font color="red">EEG for Headache</font>** is only indicated if the headache is **chronic** and has been cleared by ENT and Ophthalmology to rule out **headache seizures**.

### **Topic: Myopathies & Muscular Dystrophies**

| Category | Condition | Key Characteristics |
| :--- | :--- | :--- |
| **Myotonic** | **Myotonia Congenita** | Autosomal dominant; muscle stiffness; hypertrophied muscles. |
| **Myotonic** | **Steinert’s Disease** | Most common myotonic MD; **Hatchet Facies**; cataracts; baldness. |
| **Amyotonic** | **Duchenne (DMD)** | **Most common** MD; X-linked; **Gowers Sign**; Pseudohypertrophy. |
| **Amyotonic** | **Limb Girdle** | Autosomal recessive; onset 2nd-3rd decade; targets shoulder/pelvis. |
| **Amyotonic** | **Facio-Scapulo-Humeral** | Autosomal dominant; involves face/neck; pseudohypertrophy rare. |
| **Inflammatory** | **Polymyositis** | Female predominance (2:1); **painful muscles**; treat with **Steroids**. |

- **<font color="red">Myopathy General Manifestations</font>** include proximal and symmetrical weakness, **normal CNS** function, and the **absence of fasciculations**.
- **<font color="red">Gowers Sign</font>** is a hallmark of Duchenne Muscular Dystrophy where the patient must "walk" their hands up their legs to stand up.
- **<font color="red">Steinert's Disease (Dystrophic Myotonia)</font>** features **Hatchet Facies**, which is a thin facial appearance due to atrophy of masseter and temporalis muscles.
- **<font color="red">Metabolic Myopathies</font>** include enzyme deficiencies: **McArdle** (Myophosphorylase), **Tarui’s** (Phosphofructokinase), and **Pompe’s** (α-1,4-Glucosidase).
- **<font color="red">Muscle Biopsy</font>** in myopathies shows variable sizes of atrophy, whereas neuropathies show **group atrophy**.
- **<font color="red">Electromyography (EMG)</font>** in myopathies reveals **lower amplitude** and shorter duration motor unit potentials (AMP).

### **Topic: Myasthenia Gravis (MG)**

- **<font color="red">Myasthenia Gravis</font>** is a defect in the neuromuscular junction involving **defective ACh production, excessive Acetylcholinesterase, or competitive inhibition**.
- **<font color="red">Fatigability</font>** is the defining symptom of MG; weakness is greatest after exercise or at the end of the day, and **strength is regained by rest**.
- **<font color="red">Bulbar Symptoms</font>** in MG include ptosis, diplopia (40%), and dysphagia/dysarthria (20%).
- **<font color="red">Tensilon Test</font>** involves injecting **Edrophonium Cl**; a positive result shows a temporary, dramatic improvement in muscle strength.
- **<font color="red">Lymphorrhagia</font>** is the classic finding on muscle biopsy for a patient with Myasthenia Gravis.
- **<font color="red">Thymectomy</font>** is indicated for MG in young females (still menstruating) with a disease duration of less than 3 years.
- **<font color="red">Myasthenic Crisis</font>** is characterized by improved muscle strength after Tensilon, whereas **<font color="red">Cholinergic Crisis</font>** shows worsening or no improvement.

### **Topic: Familial Periodic Paralysis**

- **<font color="red">Familial Periodic Paralysis</font>** is an autosomal dominant condition characterized by periodic, flaccid paralysis of all four extremities **without alteration of consciousness**.
- **<font color="red">Hypokalemic Periodic Paralysis</font>** is the most common form in Orientals, often occurring in young males ("bangungot-like").
- **<font color="red">Carbohydrate Loading</font>** and heavy exercise can trigger attacks of Hypokalemic Periodic Paralysis by driving potassium into cells.
- **<font color="red">ECG monitoring</font>** is vital in periodic paralysis to check for cardiac abnormalities caused by extreme low potassium (e.g., 2.0 meq/L or below).

### **Topic: Differentiating and Comparison Points**

- **<font color="red">Athetosis vs. Dystonia</font>**: Athetosis is slow, writhing, and **distal** (fingers); Dystonia is sustained, stronger, and **proximal** (neck, trunk).
- **<font color="red">Focal vs. Generalized Seizures</font>**: Focal seizures have a localized onset (aura/focal motor); Generalized seizures involve both hemispheres from the start with immediate loss of consciousness.
- **<font color="red">Typical vs. Atypical Absence Seizure</font>**: Typical has a **3-Hz** spike-and-wave and sudden onset; Atypical has a **1-2 Hz** slow spike-and-wave and slower onset/offset.
- **<font color="red">Huntington's vs. Lubag</font>**: Huntington's is **Autosomal Dominant** (Chromosome 4); Lubag (XDP) is **X-linked Recessive** (Xq13.1) and specific to Filipino males.
- **<font color="red">Resting vs. Intention Tremor</font>**: Resting tremor occurs when the limb is supported (Parkinson’s); Intention tremor occurs during targeted movement (Cerebellar).
- **<font color="red">Migraine vs. Tension Headache</font>**: Migraines are typically **unilateral, pulsating**, and associated with nausea; Tension headaches are **bilateral, non-pulsating ("pressure")**, and lack nausea.
- **<font color="red">Episodic vs. Chronic Tension Headache</font>**: Episodic occurs < 180 days/year; Chronic occurs **≥ 15 days/month** for over 6 months.
- **<font color="red">Myopathy vs. Neuropathy (Biopsy)</font>**: Myopathy shows **variable fiber sizes**; Neuropathy shows **group atrophy**.
- **<font color="red">Myopathy vs. Neuropathy (Reflexes)</font>**: Myopathy reflexes are **normal or hypoactive** with NO Babinski; Neuropathies/LMN lesions show absent reflexes and fasciculations.
- **<font color="red">Duchenne vs. Steinert’s Disease</font>**: Duchenne is an **amyotonic** dystrophy (weakness, waddling gait); Steinert’s is a **myotonic** dystrophy (inability to relax muscle, hatchet facies).
- **<font color="red">Myasthenic vs. Cholinergic Crisis</font>**: Myasthenic crisis improves with **Tensilon (Edrophonium)**; Cholinergic crisis worsens or shows no change.
- **<font color="red">FPC vs. FIC Seizures</font>**: Focal Preserved Consciousness (FPC) means the patient is alert and aware; Focal Impaired Consciousness (FIC) involves a lack of awareness during the event.
- **<font color="red">Jacksonian March vs. Adversive Seizure</font>**: Jacksonian march is the **spread of movement** through a limb; Adversive is a **static, sustained turn** of the head.
- **<font color="red">Primary vs. Secondary Dystonia</font>**: Primary is usually genetic/idiopathic; Secondary is caused by an insult (stroke, infection, trauma) to the **basal ganglia**.
- **<font color="red">Lubag vs. Sydenham's Chorea</font>**: Lubag is **progressive** and genetic; Sydenham's is usually **self-limited** and post-infectious (Rheumatic fever).
- **<font color="red">Clonazepam vs. Diazepam in Dystonia</font>**: Clonazepam is often preferred for less sedation, but carries a higher risk of **increased oral secretions** and aspiration at high doses.
- **<font color="red">Tetrabenazine vs. Neuroleptics in HD</font>**: Tetrabenazine targets **VMAT2** (dopamine depletion); Neuroleptics target **D2 receptors** and may worsen bradykinesia/rigidity.

QA

### Topic: Seizure Classification (2025 ILAE Classification)

1. How are <b><font color="red">Focal Seizures</font></b> distinguished in the 2025 ILAE Classification? | FPC and FIC. <br>Focal Seizures with Preserved Awareness (FPC) and Focal Seizures with Impaired Awareness (FIC).
2. What is the formula for <b><font color="red">Seizure Clinical Documentation</font></b>? | Class + Classifier + Basic Descriptor: Expanded Descriptor.
3. Define <b><font color="red">Focal Preserved Consciousness Seizure (FPC)</font></b>. | Awareness and consciousness fully preserved. <br>Formerly known as "Simple Partial Seizure."
4. Describe the progression of a <b><font color="red">Jacksonian March</font></b>. | Distal to proximal spread. <br>Tonic/clonic contractions spreading from hand to arm to face.
5. What characterizes <b><font color="red">Adversive Seizures</font></b>? | Sustained head and eye turning. <br>Turning to the side opposite the seizure focus.
6. What is the origin and manifestation of <b><font color="red">Somatosensory Seizures</font></b>? | Parietal lobe; numbness/tingling. <br>Manifests contralateral to the focus.
7. What is the origin and manifestation of <b><font color="red">Visual Seizures</font></b>? | Occipital lobe; flashes/sparks/darkness.
8. What is the origin and character of <b><font color="red">Olfactory Seizures (Uncinate Fits)</font></b>? | Medial temporal lobe/uncus; foul odors.
9. What are the subjective warnings in a <b><font color="red">Temporal Lobe Aura</font></b>? (3) | 1) Déjà vu <br>2) Fear <br>3) Rising epigastric sensation
10. How is <b><font color="red">Focal-to-Bilateral Tonic-Clonic Seizure (FBTC)</font></b> differentiated from primary generalized seizures? | Clear focal onset. <br>Manifests as an aura or focal motor sign.
11. What are the features of <b><font color="red">Typical Absence Seizures (TA)</font></b>? | 3-Hz spike-and-wave. <br>Brief (2–10s) lapse of consciousness often triggered by hyperventilation.
12. What is the EEG pattern for <b><font color="red">Atypical Absence Seizures (AA)</font></b>? | 1–2 Hz spike-and-wave. <br>Pattern is slow or irregular with a slower onset/offset.
13. Describe the <b><font color="red">Tonic Phase</font></b> of a Generalized Tonic-Clonic Seizure. | Stiffening, cyanosis, and epileptic cry.
14. Describe the <b><font color="red">Clonic Phase</font></b> of a Generalized Tonic-Clonic Seizure. | Rhythmic jerking and autonomic activation.
15. What is the common name and characteristic of <b><font color="red">Generalized Atonic Seizures (GA)</font></b>? | Drop attacks. <br>Sudden loss of postural tone.
16. Define <b><font color="red">Todd’s Paralysis</font></b>. | Postictal focal neurological deficit. <br>Example: focal weakness following a seizure.

### Topic: Tremors & Movement Disorders

17. What is the hallmark of <b><font color="red">Resting Tremor</font></b>? | Parkinsonian syndromes. <br>Occurs at rest; also seen with dopamine blockers like haloperidol.
18. When does a <b><font color="red">Contraction Tremor</font></b> worsen? | During active muscle contraction. <br>Seen in essential tremor and cerebellar disorders.
19. Define <b><font color="red">Posture (Sustension) Tremor</font></b>. | Arms elevated against gravity. <br>Example: "birdwing" position; common in essential tremor.
20. What does an <b><font color="red">Intention Tremor</font></b> typically indicate? | Cerebellar disorders. <br>Worsens as the finger approaches a target.
21. What characterizes <b><font color="red">Orthostatic Tremor</font></b>? | Fast (>12 Hz) leg contractions. <br>Occurs immediately upon standing.
22. What are the common manifestations of <b><font color="red">Essential Tremor</font></b>? | Voice quiver or head nodding. <br>Disappears during sleep.
23. What is <b><font color="red">Asterixis</font></b>? | Flapping tremor. <br>Associated with hepatic encephalopathy or alcohol withdrawal.

### Topic: Huntington’s Disease (HD)

24. What is the pathogenesis of <b><font color="red">Huntington's Disease</font></b>? | HTT gene; Chromosome 4; CAG repeats. <br>Inherited in an Autosomal Dominant pattern.
25. What is the pathology of <b><font color="red">Huntington's Disease</font></b>? | Neuronal loss in Basal Ganglia. <br>Targets the Caudate/Putamen and Cerebral Cortex.
26. What is the clinical triad for <b><font color="red">Huntington's Disease</font></b>? (3) | 1) Chorea <br>2) Dementia <br>3) Psychiatric disorders (Depression)
27. How is <b><font color="red">Huntington's Disease</font></b> diagnosed? | Genetic testing (>40 repeats). <br>Also Family history and MRI showing caudate atrophy.
28. What is the FDA approved drug of choice for <b><font color="red">Huntington's Disease</font></b>? | Tetrabenazine.
29. What CAG repeat count determines <b><font color="red">Huntington's Disease</font></b> in adults? | 40 or more repeats.
30. What CAG repeat count determines <b><font color="red">Juvenile Huntington's Disease</font></b>? | Over 55 repeats.
31. What is the healthy range for <b><font color="red">CAG Nucleotide Repeats</font></b> in the HTT gene? | 10-35 repeats.
32. What is the prevalence trend of <b><font color="red">Huntington’s Disease</font></b>? | Equal in males/females. <br>Highest occurrence in Western European descent.
33. What is the most common and often first psychiatric symptom of <b><font color="red">Huntington's Disease</font></b>? | Depression.
34. What characterizes the <b><font color="red">Westphal Variant (Juvenile HD)</font></b>? | Onset < 20 years; Seizures. <br>Presents with rigid muscles rather than chorea.
35. How does <b><font color="red">Tetrabenazine</font></b> work for Huntington's Disease? | VMAT2 inhibitor. <br>Depletes dopamine.
36. Explain the <b><font color="red">Monro-Kellie Doctrine</font></b> in the context of Huntington's Disease. | Hydrocephalus ex vacuo. <br>Ventricles enlarge to fill space of atrophied tissue without increasing ICP.
37. What is the most common cause of death in <b><font color="red">Huntington’s Disease</font></b>? | Pneumonia.
38. What is the purpose of the <b><font color="red">UHDRS (Unified Huntington’s Disease Rating Scale)</font></b>? | Scoring physical progression. <br>Based on motor, cognitive, behavior, and functional ability.
39. What is the imaging hallmark of <b><font color="red">Grade 4 Huntington's Disease</font></b>? | Concave medial caudate surface.

### Topic: Dystonia & Other Movements

40. Define <b><font color="red">Athetosis</font></b>. | Slow, writhing, involuntary movements. <br>Primarily affects distal parts (fingers/arms).
41. What is the typical cause of <b><font color="red">Athetosis</font></b>? | Lesions in the corpus striatum.
42. Define <b><font color="red">Dystonia</font></b>. | Sustained muscle contractions. <br>Causes twisting and abnormal postures of proximal muscles.
43. What are the components of <b><font color="red">Meige’s Syndrome (Cranial Dystonia)</font></b>? | Blepharospasm and oromandibular dystonia. <br>Affects the eyes and jaw.
44. What is the genetic basis of <b><font color="red">Dystonia of Panay (Lubag/XDP)</font></b>? | TAF1 gene at Xq13.1. <br>Sex-linked recessive disorder.
45. Who is specifically affected by <b><font color="red">Lubag (XDP)</font></b>? | Adult Filipino men. <br>Specifically those with ancestry from Panay Island.
46. Describe the progression of <b><font color="red">Lubag (XDP)</font></b>. | Torsion dystonia then parkinsonism. <br>Dystonia in first 10-15 years, then replaced by parkinsonian features.
47. What gene is associated with <b><font color="red">Dystonia Musculorum Deformans (DMD)</font></b>? | DYT1 gene. <br>Rare, childhood-onset generalized dystonia.
48. What is the cause and character of <b><font color="red">Hemiballismus</font></b>? | Subthalamic nucleus of Luysii lesion. <br>Violent, rapid, unilateral flinging movements.
49. Define <b><font color="red">Dyskinesias</font></b>. | Purposeless movements absent during sleep. <br>Uncontrolled; worsen with emotions.

### Topic: Headaches

50. List the <b><font color="red">Pain-Sensitive Cranial Structures</font></b>. | Sinuses, arteries, and CN V, VII, IX, X.
51. What are the <b><font color="red">Headache Danger Signals</font></b> in adults? (4) | 1) Sudden "thunderclap" onset <br>2) Progressively worsening pain <br>3) Memory loss <br>4) Visual disturbance
52. What are the requirements for <b><font color="red">Migraine without Aura</font></b>? | 5+ attacks, 4-72 hours. <br>Unilateral, pulsating, with nausea/vomiting.
53. What characterize the symptoms of <b><font color="red">Migraine with Aura</font></b>? | Flashing lights or sensory changes. <br>Develop over 4 minutes; last less than 60 minutes.
54. What characterizes an <b><font color="red">Episodic Tension Headache</font></b>? | Bilateral, non-pulsating "pressure." <br>Not aggravated by activity; lacks nausea.
55. Define <b><font color="red">Chronic Tension Headache</font></b>. | Frequency ≥ 15 days/month. <br>Must persist for at least 6 months.
56. What are the clinical signs of <b><font color="red">Cluster Headache</font></b>? (4) | 1) Conjunctival injection <br>2) Lacrimation <br>3) Rhinorrhea <br>4) Ptosis
57. When is <b><font color="red">Headache Neuroimaging</font></b> (CT/MRI) indicated? | Focal, sudden, or progressive pain. <br>Also if associated with sensory depression.
58. When is an <b><font color="red">EEG for Headache</font></b> indicated? | Chronic; rules out "headache seizures." <br>Only after ENT and Ophthalmology clearance.

### Topic: Myopathies & Muscular Dystrophies

59. What are the characteristics of <b><font color="red">Myotonia Congenita</font></b>? | Muscle stiffness and hypertrophy. <br>Autosomal dominant.
60. What is the most common myotonic muscular dystrophy? | Steinert’s Disease. <br>Features Hatchet Facies, cataracts, and baldness.
61. What are the features of <b><font color="red">Duchenne Muscular Dystrophy (DMD)</font></b>? | Gowers Sign; Pseudohypertrophy. <br>Most common MD; X-linked inheritance.
62. What characterizes <b><font color="red">Limb Girdle Muscular Dystrophy</font></b>? | Targets shoulder/pelvis. <br>Autosomal recessive; onset 2nd-3rd decade.
63. Describe <b><font color="red">Facio-Scapulo-Humeral Dystrophy</font></b>. | Involves face/neck. <br>Autosomal dominant; pseudohypertrophy is rare.
64. What is the treatment and demographic for <b><font color="red">Polymyositis</font></b>? | Steroids; Female predominance (2:1). <br>Characterized by painful muscles.
65. What are the general manifestations of <b><font color="red">Myopathy</font></b>? | Proximal/symmetrical weakness; Normal CNS. <br>Absence of fasciculations.
66. Define <b><font color="red">Gowers Sign</font></b>. | Walking hands up the legs. <br>Hallmark of Duchenne Muscular Dystrophy.
67. Define <b><font color="red">Hatchet Facies</font></b> in Steinert’s Disease. | Thin face; muscle atrophy. <br>Due to atrophy of masseter and temporalis muscles.
68. Identify the enzyme deficiency in <b><font color="red">McArdle Myopathy</font></b>. | Myophosphorylase.
69. Identify the enzyme deficiency in <b><font color="red">Tarui’s Myopathy</font></b>. | Phosphofructokinase.
70. Identify the enzyme deficiency in <b><font color="red">Pompe’s Myopathy</font></b>. | α-1,4-Glucosidase.
71. Contrast <b><font color="red">Muscle Biopsy</font></b> results in myopathy vs. neuropathy. | Myopathy: Variable sizes <br>Neuropathy: Group atrophy.
72. What does <b><font color="red">Electromyography (EMG)</font></b> reveal in myopathies? | Lower amplitude motor unit potentials. <br>Also shorter duration motor unit potentials (AMP).

### Topic: Myasthenia Gravis (MG)

73. What is the pathophysiology of <b><font color="red">Myasthenia Gravis</font></b>? | Defect in neuromuscular junction. <br>Defective ACh production or excessive Acetylcholinesterase.
74. Define the <b><font color="red">Fatigability</font></b> seen in Myasthenia Gravis. | Weakness worsens after exercise. <br>Strength is regained by rest.
75. List the <b><font color="red">Bulbar Symptoms</font></b> of Myasthenia Gravis. (4) | 1) Ptosis <br>2) Diplopia <br>3) Dysphagia <br>4) Dysarthria
76. What is a positive <b><font color="red">Tensilon Test</font></b> result? | Temporary muscle strength improvement. <br>Uses injection of Edrophonium Cl.
77. What is the classic muscle biopsy finding in <b><font color="red">Myasthenia Gravis</font></b>? | Lymphorrhagia.
78. When is <b><font color="red">Thymectomy</font></b> indicated for Myasthenia Gravis? | Young females; duration < 3 years.
79. Differentiate <b><font color="red">Myasthenic vs. Cholinergic Crisis</font></b> via Tensilon. | Myasthenic: Improves <br>Cholinergic: Worsens/No change.

### Topic: Familial Periodic Paralysis

80. Define <b><font color="red">Familial Periodic Paralysis</font></b>. | Periodic, flaccid paralysis. <br>Autosomal dominant; no alteration of consciousness.
81. Which form of <b><font color="red">Periodic Paralysis</font></b> is common in Orientals? | Hypokalemic Periodic Paralysis. <br>Often occurs in young males.
82. What are triggers for <b><font color="red">Hypokalemic Periodic Paralysis</font></b>? | Carbohydrate loading and heavy exercise. <br>Drives potassium into cells.
83. Why is <b><font color="red">ECG monitoring</font></b> vital in periodic paralysis? | Check for cardiac abnormalities. <br>Detects effects of extreme low potassium (≤ 2.0 meq/L).

### Topic: Differentiating and Comparison Points

84. Compare <b><font color="red">Athetosis vs. Dystonia</font></b> by location. | Athetosis: Distal (fingers) <br>Dystonia: Proximal (neck, trunk).
85. Compare <b><font color="red">Focal vs. Generalized Seizures</font></b> by onset. | Focal: Localized (aura) <br>Generalized: Bilateral hemispheres; immediate LOC.
86. Compare <b><font color="red">Typical vs. Atypical Absence Seizure</font></b> by EEG. | Typical: 3-Hz <br>Atypical: 1-2 Hz slow spike-and-wave.
87. Compare <b><font color="red">Huntington's vs. Lubag</font></b> genetics. | HD: Autosomal Dominant (Chr 4) <br>Lubag: X-linked Recessive (Xq13.1).
88. Compare <b><font color="red">Resting vs. Intention Tremor</font></b> by state. | Resting: Supported limb (Parkinson’s) <br>Intention: Targeted movement (Cerebellar).
89. Compare <b><font color="red">Migraine vs. Tension Headache</font></b> by quality. | Migraine: Unilateral, pulsating, nausea <br>Tension: Bilateral, pressure, no nausea.
90. Compare <b><font color="red">Episodic vs. Chronic Tension Headache</font></b> frequency. | Episodic: < 180 days/year <br>Chronic: ≥ 15 days/month for > 6 months.
91. Compare <b><font color="red">Myopathy vs. Neuropathy</font></b> by reflexes. | Myopathy: Normal/Hypoactive (No Babinski) <br>Neuropathy: Absent reflexes and fasciculations.
92. Compare <b><font color="red">Duchenne vs. Steinert’s Disease</font></b> by type. | Duchenne: Amyotonic (weakness) <br>Steinert’s: Myotonic (unable to relax).
93. Compare <b><font color="red">FPC vs. FIC Seizures</font></b> by awareness. | FPC: Alert and aware <br>FIC: Lack of awareness.
94. Compare <b><font color="red">Jacksonian March vs. Adversive Seizure</font></b> by motion. | Jacksonian: Spread through limb <br>Adversive: Static/sustained head turn.
95. Compare <b><font color="red">Primary vs. Secondary Dystonia</font></b> by etiology. | Primary: Genetic/Idiopathic <br>Secondary: Basal ganglia insult (stroke/trauma).
96. Compare <b><font color="red">Lubag vs. Sydenham's Chorea</font></b> by course. | Lubag: Progressive <br>Sydenham's: Self-limited (post-infectious).
97. Compare <b><font color="red">Clonazepam vs. Diazepam</font></b> in Dystonia. | Clonazepam: Less sedation <br>Risk: Higher oral secretions/aspiration.
98. Compare <b><font color="red">Tetrabenazine vs. Neuroleptics</font></b> in HD. | Tetrabenazine: VMAT2 (DA depletion) <br>Neuroleptics: D2 receptors (may worsen rigidity).