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Paeds BPT Written Exam > Neurology > Flashcards

Neurology Flashcards

1
Q

Features of Juvenile Myoclonic Epilepsy

A
  • Morning myoclonic jerks (often dropping things)
  • +/- absence seizures
  • Normal intelligence
  • Family hx
  • Onset 8-20yrs
  • Generalised tonic-clonic seizures occurring just after waking or during sleep
  • Increased seizures with sleep deprivation, stress, alcohol
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2
Q

Features of Absence seizures

A
  • Short lapses in awareness (10-20sec)
  • Absence of aura
  • Amnesia during the episodes
  • Abrupt onset and end
  • Staring and behavioural arrest +/- flickering eyelids, eye rolling, mouth automaticisms
    No post ictal period
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3
Q

EEG finding of 3Hz spike and wave is characteristic of what?

A

Absence seizures / Childhood absence epilepsy

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4
Q

Management of focal seizures

A

Carbamazepine

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5
Q

Which medication should be avoided in absence seizures and can worsen absence seizures

A

Carbamazepine

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6
Q

What genetic condition commonly causes infantile spasms (30%)

A

Tuberous sclerosis

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7
Q

Features of Lennox Gastaut Syndrome

A
  • multiple seizure types
  • intellectual disability
  • EEG: “slow spike and wave”
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8
Q

What causes gelastic seizures (laughing seizures)

A

Hypothalamic hamartomas

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9
Q

Side effects of sodium valproate

A

Fat (weight gain)
Bald (hair loss)
Thrombocytopenia (dose related)
Pancreatitis
Incr Ammionia
Neural tube defects

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10
Q

What is vigabatrin used for and what is its main AEs

A

Used in West Syndrome (Infantile spasms)

AEs
- can cause irreversible visual field defects
- also causes weight gain

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11
Q

Side effects of carbamazepine?

A
  • Severe leukopenia
  • Hepatotoxicity
  • SIADH and hyponatremia
  • N, V, D
  • Rash
  • Diplopia
  • Dizziness and drowsiness
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12
Q

Which AED is most teratogenic?

A

Sodium valproate

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13
Q

Hallmark EEG feature of Infantile spasms?

A

Hypsarrhythmia

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14
Q

Which anti-convulsant should be avoided in Chinese people with HLA-B1502 allele?

A

Carbamazepine and lamotrigine

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15
Q

Mutation found in Dravet syndrome

A

Loss of function mutation in SCN1A gene

Dravet syndrome often begins with febrile seizures which are more prolonged, more frequent, may be focal and come in clusters then develop afebrile seizures or various types

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16
Q

Features of Benign familial neonatal seizures

A
  • Autosomal dominant, usually due to genes in KCNQ2 and KCNQ3 genes
    • Onset ~ 2-4days old
    • Usually remit ~ 2-15weeks of age
    • Seizures consist of ocular deviation, tonic posturing, clonic jerks and motor automaticisms
    • Interictal EEG usually normal
      Approx 16% develop lateral epilepsy
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17
Q

Features of CNIII paralysis

A

Ptosis
Dilation of the pupil
Displacement of the eye down and out
Impaired adduction and elevation

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18
Q

Features of CNVI paralysis

A

Medial deviation of the eye
Inability to abduct beyond the midline

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19
Q

Difference between spasticity and rigidity

A

Spasticity is characterised by initial resistance and then sudden release (clasp-knife) and is due to UMN lesion. Velocity dependent

Rigidity is characterised by complete resistance to flexion and extension (lead-pipe) and may be due to lesion in basal ganglia

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20
Q

Which AED requires serial measurements of serum levels because of a narrow therapeutic index?

A

Phenytoin

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21
Q

Lamotrigine drug interactions?

A
  • Topiramate may increase toxicity of sodium valproate.
  • Topiramate concentration may be decreased by carbamazepine.
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22
Q

Inflammatory demyelination typically of the periventricular deep white matter with POSTERIOR-PREDOMINANT pattern?

A

Adrenoleukodystrophy (X-linked)

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23
Q

Bilateral symmetrical areas of periventricular deep white matter signal change around the atria and frontal horns forming a “BUTTERFLY PATTERN”?

A

Metachromatic leukodystrophy

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24
Q

Urine succinylacetone is present in which condition?

A

Tyrosinemia

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25
Q

What is the recurrence risk of myelomeningocoele in subsequent pregnancies?

A

2-3% if 1 affected child
10% if 2 affected children!

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26
Q

Difference between meningocele and myelomeningocoele

A

Meningocele is a herniation of the meninges (not the cord) through a defect in posterior vertebral arches
Less severe, less common

Myelomeningocoele is a herniation of nerve roots, spinal cord, meninges through a defect in the spinal cord. They cause a downward displacement of brainstem and cerebellar tonsils into the spinal column (Chiari II or ‘Arnold Chiari’ malformation) ->hydrocephalus

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27
Q

Which antenatal blood test could suggest an open neural tube defect (anencephaly or encephalocoele, myelomeningocoele) if elevated?

A

AFP

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28
Q

Causes of communicating hydrocephalus (impaired absorption)?

A

Meningitis
Subarachnoid haemorrhage
Congenital infections eg CMV, toxo, rubella

Anything that irritates the lining of the arachnoid granulations

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29
Q

Causes of non-communicating (obstructive) hydrocephalus

A

Congenital
- Dandy Walker malformation
- Congenital aqueductal stenosis
- X linked aqueduct stenosis (L1CAM mutation)
- Type 1 Chiari malformation
- Type 2 Chiari malformation

Acquired
- Brain tumours
- Intraventricular clots, abscesses
- Vein of Galen malformation

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30
Q

What is Chiari I malformation?

A

Downward displacement of cerebellar tonsils or vermis below the foramen magnum

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31
Q

What is Chiari II malformation (Arnold Chiari)?

A

Downward displacement of cerebellum AND 4th ventricle and lower medulla below the level of the foramen magnum. This blocks CSF outflow and 85% have hydrocephalus. Unlike type 1, surgical intervention usually required

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32
Q

What is polymicrogyria?

A

Multiple small gyri (folds) - cerebral cortex has an irregular, pebble like appearance. Can be focal or diffuse

33
Q

What is lissencephaly?

A

Agyria
- Cerebral surface is smooth with lack of folds (gyri) and grooves (sulk)

34
Q

Features of lissencephaly?

A
  • Microcephaly
  • Developmental delay
  • Severe epilepsy
  • Blindness

CT/MRI shows abnormally thick cortical ribbon

35
Q

What is schizencephaly?

A

“split brain”
- unilateral or bilateral clefts within the cerebral hemisphere

36
Q

What is holoprosencephaly?

A
  • Failure of formation of 2 hemispheres
  • often have facial anomalies: flat midface and nasal bridge, hypotelorism, medial cleft lip, single central incisor
  • often have DI, hypopituitarism, epilepsy, vision impairment
37
Q

Features of Erbs Palsy (C5,6 and sometimes 7)

A

“Waiters tip”
- Sagging shoulder
- Internally rotated arm
- Flexed wrist
- Absent biceps reflex

Can have phrenic nerve involvement -> unilateral diaphragm palsy, asymmetric chest expansion, hypoxia

38
Q

Features of Klumpkes palsy (C8-T1)

A

“Claw hand”
- extension of MCP, flexion of DIP and PIPs
- Decreased sensation on ulnar side of hand
- Can experience Horner sydndrome (ptosis, miosis, anhydrous)

39
Q

How does nusinursen (Spinraza) for SMA work?

A

It is an oligosense nucleotide which modifies splicing of SMN2 gene to increase production of SMN protein

40
Q

How does onasemnogene (Zolgensma) for SMA work?

A

It is an SMN1 gene replacement delivered via an AAV9 viral envelope

Given once only IV

41
Q

Differences between dystrophies and myopathies?

A

Dystrophies
- High CK (vs normal -mildly increased)
- abnormal extracellular proteins (vs intracellular proteins)
- progressive weakness (vs more stable weakness)

42
Q

Management of muscular dystrophy

A

Steroids - prednisolone or deflazacort **many benefits
Plus allied health, orthotics etc

43
Q

Cause of myotonic dystrophy?

A

Autosomally dominant inherited trinucleotide expansion of CTG of DMPK gene which encodes serine-threonine protein kinase

  • Premutation: 37-50 repeats
  • Mildly affected 50-150 repeats
  • Classic disease 100-1000 repeats
44
Q

Features of myotonic dystrophy

A

Hypotonia
Weakness
Wasting
Myotonia
Myopathic facies: long face, V shaped upper lip
Intellectual impairment
Cataracts
Low IgG

45
Q

What causes juvenile myasthenia gravis

A

Antibodies bind to post-synaptic ACh receptor at the NMJ, blocking ACh (acetylcholine) from binding

AChR or MuSK antibodies probably originate in the thymus

46
Q

Key features of myasthenia gravis

A

Fatiguable weakness
Ptosis
Ophthalmoplegia

47
Q

CSF findings in Guillain Barre Syndrome

A

Very high protein
Low WBCs
(aka cytoalbuminologic dissociation)

48
Q

Which inflammatory CNS condition may be associated with ovarian teratoma?

A

Anti-NMDA receptor encephalitis

49
Q

Triplet repeat disorder of fraxatin (FXN) gene causing ataxia?

A

Friedrich’s Ataxia (AR inheritence)

50
Q

Which neurologic condition is associated with increased sensitivity to ionising radiation?

A

Ataxia-Telangiectasia

51
Q

Test and management of botulism

A

Test: stool Clostridium botulism (or C botulism toxin)
Management: ICU, anti-toxin

52
Q

Clinical features of botulism

A

Constipation
Descending weakness (cranial nerves first)
Absent reflexes: pupils, gag, deep tendon
Dry mouth

53
Q

Side effects of phenytoin

A
  • Hirsutism
  • Gum hypertrophy
  • Ataxia and Nystagmus
  • SJS
  • Hepatic dysfunction (idiosyncratic hypersensitivity)
  • Drowsiness
  • Blood dyscrasias
  • Macrocytosis (reduced folate absorption)
  • p450 inducer- lots of drug interactions
54
Q

Contraindications to sodium valproate?

A

Teen females (teratogenic)
Metabolic/ mitochondrial disorder (risk of severe hepatotoxicity)

55
Q

Side effects of topiramate

A
  • Kidney stones
  • Metabolic acidosis
  • Slowed cognition
  • Acute angle-closure glaucoma
  • Weight loss
  • Reduced sweat/ heat intolerance
56
Q

Orofacial seizures think..

A

Benign childhood epilepsy with centrotemporal spikes - wakes at night with focal seizure on one side of face, drooling, inability to speak but preserved awareness. May have secondary generalisation

57
Q

First line treatment of childhood absence epilepsy?

A

Ethosuximide or sodium valproate

58
Q

Remission rate of childhood and juvenile absence epilepsy?

A

CAE= 60%
JAE= lifelong but seizures can be controlled in 70-80%

59
Q

Features of Self-limited epilepsy with autonomic seizures (SeLEAS) - previously Panayiotopoulos

A

Onset 3-6yrs
Focal seizures with autonomic features- VOMITING, pallor, flushing, abdominal pain, often head or eye deviation
Often only 1x seizure (25%) or remission within 1-2yrs

60
Q

Which infection most commonly precedes Guillain Barre syndrome?

A

Campylobacter

(CMV, EBV, Mycoplasma)

61
Q

Management of Guillain Barre Syndrome?

A

IVIG or plasma exchange

(NOT steroids- they have shown no benefit)

Eculizumab (complement blocker) being trialled

62
Q

What are anti-GQ1b antibodies associated with?

A

Variants of Guillain barre syndrome: Miller Fisher syndrome, Bickerstaff brainstem encephalitis

63
Q

3 features of Aicardi Syndrome?

A

Infantile spasms
Agenesis of corpus callosum
Chorioretinal lacunae (small holes in the retina)

It is X-linked dominant

64
Q

What can happen if Na+ is corrected to quickly?

A

From low -> high: pontine demyelinosis
From high -> low: cerebral oedema

65
Q

Diagnostic criteria for NF1?

A

2 or more if parent not diagnosed:
- 6+ cafe au lait macules >5mm (prepubertal)
- Axillary or inguinal freckling
- 2+ neurofibromas of any type or 1+ plexiform neurofibroma
- Optic pathway glioma
- 2+ Lisch Nodules
- Distinctive osseous lesion eg bowing of tibia, sphenoid dysplasia
- Heterozyogus pathogenic NF1 variant

66
Q

Major features of tuberous sclerosis

A
  • 3+ Hypomelanotic macules >5mm
  • 3+ Angiofibromas
  • 2+ ungal fibromas
  • Shagreen patch
  • Multiple retinal hamartomas
  • Multiple cortical tubers and/or radial migration lines
  • 2+ Subependymal nodules
  • Subepnedeymal giant cell astrocytoma (SEGA)
  • Cardiac rhabdomyoma
  • Angiomyolioma
    Lymphangioleiomyomatosis (LAM)
67
Q

Mutation causing Sturge Weber syndrome

A

GNAQ gene

68
Q

Acquired epileptic aphasia

A

Landau Kleffner syndrome

(onset 3-7yrs with language regression, ADHD like behaviour +/- focal motor seizures)

69
Q

MOA of levetiracetam?

A

Binding to synaptic vesicle
protein 2A (SV2A)in the brain resulting in a change in neurotransmitter release

70
Q

When do symptoms of Guillain Barre syndrome peak?

A

Usually ~7-10 days

71
Q

Which infections commonly precede Guillain Barre syndrome?

A

Commonly have preceding infection 3-6 weeks earlier with campylobacter (30%), EBV, CMV, mycoplasma

72
Q

Which antibodies are associated with neuromyelitis optica spectrum disease?

A

AQ-4 antibodies

(aggressive disease with high chance of release, blindness and wheelchair dependency at 5yrs)

73
Q

How does MOG-antibody demyelinating disease present?

A
  • ADEM (60%) or
  • Optic neuritis (unilateral or bilateral) (37%)
  • Transverse myelitis (13%) or
  • Neuromyelitis optica spectrum disorder (50%)
74
Q

Management of ADEM

A

High dose steroids (usually 5d Methylpred)
Cover with abx and acyclovir until infection excluded
If not responsive to steroids -> IVIG and PLEX

75
Q

Management of Guillain Barre Syndrome?

A

IVIG
Plasma exchange

Steroids not effective

76
Q

Weakness of which muscles causes trendelenburg gait?

A

Weakness of hip ABDUCTORS -> dropping of the pelvis to the contralateral side

77
Q

Features of Aicardi syndrome?

A

X-linked dominant so females affected

Triad of:
agenesis of corpus callosum,
chorioretinal lacunae (often see coloboma)
seizures (incl infantile spasms)

78
Q
A

Paeds BPT Written Exam (18 decks)

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