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Random Notes for Royal College Exam > Neurology > Flashcards

Neurology Flashcards

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1
Q

What is the most commonly identified precipitant of Guillain Barre Syndrome (GBS)?

A

Campylobacter (30%)

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2
Q

What are other infectious precipitants can cause GBS?

A
  1. CMV
  2. EBV
  3. Mycoplasma pneumoniae
  4. Influenza-like illnesses
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3
Q

What is Niemann-Pick disease?

A

Sphingomyelin lipidosis

  1. lysosomal enzyme def. of sphingomyelinase
  2. HSM
  3. Regression
  4. Macular degeneration w/ cherry red spots (25%)
  5. Hypotonia
  6. Areflexia
  7. Delayed nerve conduction
  8. Brain atrophy with volume loss
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4
Q

What is Gaucher disease?

A

Glucosylceramide lipidosis - enzyme defect in B-glucocerebrosidase

Common in Ashkenazi Jews

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5
Q

What is Krabbe disease?

A

Galactosylceramide lipidosis aka globoid cell leukodystrophy, 2 phenotypes (early 3-6m, later infancy)

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6
Q

What is metachromic leukodystrophy?

A

Lipidosis occurs due to deficiency of lysosomal enzyme arylsulfatase A (cerebroside sulfatase)

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7
Q

What are the clinical features metachromic leukodystrophy?

A

Symptoms usu. develop in first 2y, but may present @ 4-5yo

  1. Gait dysfunction
  2. Ataxia
  3. Weakness
  4. Neuropathy
  5. Muscle stretch reflex absent
  6. Spasticity
  7. Affects intellect
  8. Optic atrophy
  9. Seizures
  10. Cherry red spots
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8
Q

What is the inheritance pattern of Krabbe disease?

A

AR lysosomal defect

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9
Q

What are the clinical features of Krabbe disease?

A
  1. Galactocerebrocidase - WBC in serum & amniotic fluid
  2. Irritability
  3. Fever of unknown origin
  4. Rigidity leading to feeding problems
  5. Vision and hearing loss
  6. Mortality by age 2
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10
Q

What is the inheritance pattern of Gaucher disease?

A

AR

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11
Q

What are the clinical features of Gaucher disease?

A
  1. Accumulation of glucocerbrosides in brain, liver, spleen, bone marrow
  2. HSM
  3. Hyperextended posture
  4. Bone marrow suppression
  5. Bone pain
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12
Q

What is the inheritance pattern gangliosides GM-1?

A

AR

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13
Q

What are the clinical characteristics GM-1 infant type?

A
  1. Anorexia
  2. Poor suck
  3. FTT
  4. Dev’t delay
  5. Generalized seizure
  6. Spastic quadriplegia
  7. Coarse facial features
  8. Edema of hands/feet
  9. Gum hypertrophy
  10. HSM
  11. Blindness
  12. Deafness
  13. Cherry red spot
  14. Mortality by 2-3yo
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14
Q

What are the clinical characteristics of GM-1 juvenile type (presents @ 1yo)?

A
  1. Incoordination
  2. Weakness
  3. Ataxia
  4. Language regression
  5. Convulsions
  6. Spasticity
  7. Decerebrate rigidity
  8. Blindness
  9. Mortality by 10yo
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15
Q

What are the clinical characteristics of GM-1 adult type?

A
  1. Slow progressive spasticity
  2. Ataxia
  3. Dysarthria
  4. Gradual loss of cognitive function
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16
Q

What are the clinical characteristics of GM-2 Tay Sachs disease?

A
  1. Hyperacusis
  2. Increased startle response
  3. Developmental delay
  4. Poor vision
  5. Cherry red spot
  6. Myoclonic seizures
  7. Death by 3yo
17
Q

What is the inheritance pattern of Tay Sachs?

A

AR

18
Q

What is adrenoleukodystrophy?

A

Peroxisomal disorder

19
Q

What is the inheritance pattern of adrenoleukodystrophy?

A

X-linked

20
Q

What are the clinical characteristics of adrenoleukodystrophy?

A
  1. Presents males 4-8yo
  2. Progressive deterioration in schoolwork
  3. Hearing/speech problems
  4. Seizures
  5. White matter changes on MRI
  6. Addison’s (primary adrenal insufficiency)
21
Q

What is MELAS?

A

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes

22
Q

What is Werdnig-Hoffman disease?

A

SMA Type 1

23
Q

What is a 6th nerve palsy?

A

Dysfunction of abducens nerve and contraction of the lateral rectus muscle

  1. Esotropia
  2. Dipopia
24
Q

What are the most common causes of 6th nerve palsy?

A
  1. Tumors (45%) esp. posterior fossa medulloblastoma, brainstem glioma
  2. Elevated ICP (15%)
  3. Trauma (12%)
  4. Congenital (11%)
  5. Inflammatory (7%)
  6. Idiopathic (5%)
25
Q

What is the characteristic EEG findings for infantile spasms?

A

Hypsarrhythmia

26
Q

What is the characteristic EEG findings for absence seizures?

A

3Hz generalized spike and wave discharges

27
Q

What is the characteristic EEG findings for benign rolandic epilepsy?

A

Centrotemporal spikes

28
Q

Why is diazepam not commonly used in neonates?

A

Decreased liver metabolism

29
Q

Which is higher in neonates versus adolescents?

A

volume of liquid distribution

30
Q

When does Lennox-Gastaut present?

A

3-10yo

31
Q

What are the clinical characteristics of Lennox-Gastaut?

A
  1. Severe impairment
  2. Atypical absence seizures with head atonia and myoclonus during seizures
  3. Poor prognosis
32
Q

What is the characteristic EEG finding for Lennox-Gastaut?

A

1-2Hz spike and slow wave discharges

33
Q

When does Landau-Kleffner syndrome typically present?

A

3-6yo, resolves by 8-12yo

34
Q

What are the clinical characteristics of Landau-Kleffner?

A
  1. Regression in language comprehension and expressive language (verbal auditory agnosia)
  2. 80% eventually exhibit clinical seizures
35
Q

How does septra effect phenytoin?

A

It causes decreased metabolism and increases serum levels of phenytoin

36
Q

What is first line therapy for absence seizures?

A

Ethosuximide

37
Q

What is first line therapy for infantile spasms?

A

ACTH

Second: Vigabatrin

38
Q

Which antiepileptic causes hepatotoxicity, pancreatitis, and thrombocytopenia?

A

Valproic acid

39
Q

What is the most common posterior fossa tumor?

A

Medulloblastoma 35-40%
Cerebellar astrocytoma 35-40%
Brainstem glioma 10-15%
Ependymoma 10-15%

Random Notes for Royal College Exam (23 decks)

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