Neurology Flashcards

1
Q

Sodium valproate: indications and side effects?

A

Use: (Think ‘generalised’)

  1. Generalised tonic clonic
  2. Absence
  3. Atypical absence
  4. Myoclonic

Side effect:

  1. Teratogen (neural tube)
  2. Hyperammonaemia
  3. Thrombocytopenia (dose-related)
  4. Weight gain
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2
Q

Carbamazepine: indications and side effects?

A

Use:
1. Focal seizures +/-secondary generalised

Side effect:

  1. Leukopenia
  2. Hepatotoxicity
  3. SIADH -> hyponatraemia
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3
Q

Phenytoin: indications and side effects?

A

Use:

  1. Status epilepticus
  2. Focal seizures +/- secondary generalised

Side effects:

  1. P450 induction
  2. Hirsutism
  3. Gum enlargement
  4. Teratogen (hydantoin syndrome)
  5. Ataxia, rash
  6. Steven Johnson syndrome
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4
Q

Ethosuximide: indications and side effect?

A

Use:
1. Absence seizures

Side effect:

  1. (F)atigue
  2. (G)I upset
  3. (H)eadache
  4. (I)tching
  5. Steven (J)ohnson
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5
Q

Phenobarbitol: indications and side effect?

A

Use: (only in infants i.e. ‘phenoBABYtol’)

  1. Generalised tonic-clonic
  2. Focal seizure

Side effect:

  1. Severe behavioural changes
  2. Impaired cognition
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6
Q

Lamotrigine: indications and side effect?

A

Use:

  1. Lennox-Gastaut
  2. Focal seizures

Side effect:

  1. Steven Johnson syndrome
  2. Haemophagocytic lymphohistiocytosis
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7
Q

Levetiracetam: indications and side effect?

A

Use:

  1. Focal seizures (with impaired consciousness >4 years)
  2. Generalised tonic-clonic (>6 years)
  3. Juvenile myoclonic epilepsy (>12 years)

Side effect:
1. Behavioural (irritability, aggression)

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8
Q

Topiramate: indications and side effect?

A

Use:

  1. Focal seizure
  2. Generalised seizure

Side effect:

  1. Kidney (s)tone
  2. (S)low cognition
  3. (S)kinny (weight loss)
  4. (S)ight threatened (glaucoma)
  5. (S)peech difficulties (word finding)
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9
Q

Oxcarbazepine: indications and treatment?

A

Use:
1. Benign Rolandic epilepsy of childhood (centrotemporal spikes)

Side effect:

  1. Ataxia
  2. Nystagmus
  3. Hyponatraemia
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10
Q

Which areas of the brain correspond to motor, adversive, somatosensory or emotional changes?

A

Focal motor = precentral gyrus

Focal adversive (forceful turning to one side) = mesial frontal lobe

Focal somatosensory = parietal

Emotional/hallucinatory = temporal

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11
Q

What is the treatment of choice for focal seizures?

A

Carbamazepine
Oxcarbazepine
Phenytoin

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12
Q

What is the treatment of infantile spasms?

A

ACTH
Vigabatrin

ACTH side effect = HTN, irritability, ionfection

Vigabatrin side effect = Vision (permanent blindness)
-> best to use with coexistant tuberous sclerosis

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13
Q

What is West syndrome?

A
Triad of: 
1. Infantile spasm 
2. Intellectual disability 
3. Hypsarrhythmia
= high voltage, irregular, slow waves
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14
Q

What is the EEG with Lennox Gastaut syndrome?

A

Generalised, bilaterally synchronous, sharp- and slow-wave complexes, occurring in repetitive fashion in long runs

a.k.a. ‘slow spike and wave’

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15
Q

What neurocutaneous syndrome is infantile spasms associated with?

A

Tuberous sclerosis

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16
Q

What neurocutaneous disease causes focal seizures contralateral to affected area?

A

Sturge-Weber
= IPSIlateral leptomeningeal angioma causing CONTRAlateral focal seizure

Triad:

  1. Port-wine stain
  2. Leptomeningeal angioma
  3. Glaucoma
17
Q

Why is spinal muscular atrophy (SMA) a disease of motor, not sensory, deficit?

A

= ANTERIOR horn disease (degeneration)

Anterior horn = motor
Posterior horn = sensory

Lethal AR disorder (chromosome 5q)

18
Q

Elevated CK is seen with what neuromuscular disease?

A

Duchenne and Becker

NOT elevated with SMA

19
Q

Which is more severe - Duchenne or Becker?

A

Duchenne
= absent or deficient dystrophin protein

‘BECKER is BETTER’
= defective but not total absence of protein

20
Q

What is the difference in myasthenia gravis vs. Lambert Eaton vs botulism?

A

MG = POST-synaptic (AChR destroyed)

LES = PRE-synaptic (voltage-gated calcium channel not releasing ACh; attacked by antibodies)

Botulism = PRE-synaptic (botulinum toxin inhibits ACh release)

21
Q

What are the types of myasthenia gravis in children?

A
  1. Transient neonatal
  2. Congenital
  3. Juvenile
22
Q

What is used to help diagnose myasthenia gravis?

A

ACh-receptor antibodies (AChR-Ab)

Muscle-specific receptor tyrosine kinase (MuSK) antibodies

EMG demonstrating electrodecrement

23
Q

What is the most common myositis in children?

A

Juvenile dermatomyositis

24
Q

What serum abnormality is seen in ataxia telangiectasia?

A

Elevated alpha fetoprotein

Decreased serum IgA

25
Q

What is the difference between vestibular neuritis vs. labarynthitis?

A

Both due to viral/postviral inflammatory disorder of vestibular portion of CN VIII

Vestibular neuritis = auditory function preserved

Labarynthitis = unilateral hearing loss

26
Q

What are the key physical findings in vestibular neuritis?

A

Nystagmus - horizontal or rotational

Positive head thrust (rapid turning of head toward lesion side -> patient unable to maintain visual fixation)

Gait instability = fall toward affected side

27
Q

Where is the most common location for brain tumours in children?

A

Posterior

28
Q

Describe the flow of CSF and their channels

A

CSF produced by choroid plexus in lateral, third and fourth ventricles

Lateral -> (foramen of Munro) -> third ventricle -> (aqueduct of Sylvius) -> fourth ventricle -> (foramina of Magendie (medial) and Luschka (lateral)) -> subarachnoid space

Absorbed by arachnoid granulatoions to dural venous sinus

29
Q

What is a communicating hydrocephalus?

A

= impairment of CSF flow within the SUBARACHNOID SPACE or impairment of ABSORPTION (at villi)

A misnomer
Think of it as EXTERNAL hydrocephalus

30
Q

How are the clinical symptoms of hydrocephalus related to anatomy?

A

Symptoms due to (1) ventricular distension; (2) increased ICP

Lateral ventricle dilation = ataxia + spasticity (lower&raquo_space; upper as leg fibers closest to ventricles)

Third ventricle dilation = endocrine dysfunction (hypothalamic region) + visual dysfunction (proximity to anterior of third ventricle)

Cerebral aqueduct dilation = sun-setting eyes (compress periaqueductal vertical gaze center)

31
Q

What type of sensory information does the dorsal column-medial lemniscus vs. anterolateral spinothalamic tract?

A

DCML

  1. Proprioception
  2. Fine touch

SPINOTHALAMIC
(Lateral)
1. Pain
2. Temperature

(Anterior)

  1. Crude touch
  2. Pressure
32
Q

What vitamin deficiency causes treatment-resistant neonatal seizures?

A

Vitamin B6 (pyridoxine)

Required for synthesis of y-amniobutyric acid (GABA), which is the principle inhibitory neurotransmitter

33
Q

What is elevated in the serum after a seizure?

A

Prolactin

But not routinely measured as per NICE

34
Q

What is the most common aura pre-seizure?

A

Epigastric pain

Feeling of fear

35
Q

What two antiepileptics are renally cleared?

A

Levetiracetam

Phenobarbitol (25%)

36
Q

What is the most common posterior fossa tumour of childhood?

A

Cerebellar astrocytoma

37
Q

What is Macewen’s sign

A

Percussion of skull -> cracked pot sound

Positive test = separation of sutures
-> seen in hydrocephalus, brain abscess

38
Q

What is the familial cause of hydrocephalus due to?

A

Aqueductal stenosis

X-linked

Note: NF-1 can also cause aqueductal stenosis (rare association)

39
Q

What is gene is involved in Dravet syndrome?

A

SCN1A