Sodium valproate: indications and side effects?
Use: (Think ‘generalised’)
Side effect:
Carbamazepine: indications and side effects?
Use:
1. Focal seizures +/-secondary generalised
Side effect:
Phenytoin: indications and side effects?
Use:
Side effects:
Ethosuximide: indications and side effect?
Use:
1. Absence seizures
Side effect:
Phenobarbitol: indications and side effect?
Use: (only in infants i.e. ‘phenoBABYtol’)
Side effect:
Lamotrigine: indications and side effect?
Use:
Side effect:
Levetiracetam: indications and side effect?
Use:
Side effect:
1. Behavioural (irritability, aggression)
Topiramate: indications and side effect?
Use:
Side effect:
Oxcarbazepine: indications and treatment?
Use:
1. Benign Rolandic epilepsy of childhood (centrotemporal spikes)
Side effect:
Which areas of the brain correspond to motor, adversive, somatosensory or emotional changes?
Focal motor = precentral gyrus
Focal adversive (forceful turning to one side) = mesial frontal lobe
Focal somatosensory = parietal
Emotional/hallucinatory = temporal
What is the treatment of choice for focal seizures?
Carbamazepine
Oxcarbazepine
Phenytoin
What is the treatment of infantile spasms?
ACTH
Vigabatrin
ACTH side effect = HTN, irritability, ionfection
Vigabatrin side effect = Vision (permanent blindness)
-> best to use with coexistant tuberous sclerosis
What is West syndrome?
Triad of: 1. Infantile spasm 2. Intellectual disability 3. Hypsarrhythmia = high voltage, irregular, slow waves
What is the EEG with Lennox Gastaut syndrome?
Generalised, bilaterally synchronous, sharp- and slow-wave complexes, occurring in repetitive fashion in long runs
a.k.a. ‘slow spike and wave’
What neurocutaneous syndrome is infantile spasms associated with?
Tuberous sclerosis
What neurocutaneous disease causes focal seizures contralateral to affected area?
Sturge-Weber
= IPSIlateral leptomeningeal angioma causing CONTRAlateral focal seizure
Triad:
Why is spinal muscular atrophy (SMA) a disease of motor, not sensory, deficit?
= ANTERIOR horn disease (degeneration)
Anterior horn = motor
Posterior horn = sensory
Lethal AR disorder (chromosome 5q)
Elevated CK is seen with what neuromuscular disease?
Duchenne and Becker
NOT elevated with SMA
Which is more severe - Duchenne or Becker?
Duchenne
= absent or deficient dystrophin protein
‘BECKER is BETTER’
= defective but not total absence of protein
What is the difference in myasthenia gravis vs. Lambert Eaton vs botulism?
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)
What are the types of myasthenia gravis in children?
What is used to help diagnose myasthenia gravis?
ACh-receptor antibodies (AChR-Ab)
Muscle-specific receptor tyrosine kinase (MuSK) antibodies
EMG demonstrating electrodecrement
What is the most common myositis in children?
Juvenile dermatomyositis
What serum abnormality is seen in ataxia telangiectasia?
Elevated alpha fetoprotein
Decreased serum IgA