Neurology Flashcards
What age group is affected by febrile convulsions?
6mo-6yr
What is the criteria for a seizure to be Simple Febrile seizure
- 95%
Brief repeated symmetric contractions of neck, trunk, extremities (flexion and extension) lasting 10-30s
Infantile Spasm
What is West Syndrome?
Infantile spasm, Developmental regression, hypsarhythmia on EEG
What age group is affected by infantile spasm?
4-8mo
80% of infantile spasms are due to ________ abnormalities, _____ or are associated with_____
80% due to metabolic or developmental abnormalities, encephalopathies or are associated with neurocutaneous syndromes (
What is seen on a Typical EEG: hypsarrhytmia
High voltage slow waves, spikes and polyspikes, background disorganization
Management of infantile spasms:
ACTH, benzodiazepines, Corticosteroids (for an acute period of time), vigabatrin (if diagnosed dura sclerosis)
Centrotemporal sharp waves on EEG?
Benign Focal epilepsy (rolandic spikes)
Focal motor seizures involving tongue, mouth, face, upper extremity usually occurring in sleep-wake transition states
Benign Rolandic Epilepsy
What age group affected by Benign Rolandic Epilepsy?
5-10 years of age - remit spontaneously in adolescence
Manage Benign Rolandic epilepsy with _______
Carbamazepine,
What age group affected by Childhood absence seizures?
6-7 years and usually grow out of it by teenage years
What medication is used for Childhood absence seizures?
and if there’s also generalised seizures?
Ethosuximide for just absent
Valproic acid (if also generalized)
Typical EEG: 3Hz spike and wave
Childhood absence seizures
Normal EEG in between seizures
Multiple daily absence seizures lasting
Childhood absence seizures
Myoclonus particularly in morning; frequently presents as generalized tonic-clonic seizures
Juvenile Myoclonic Epilepsy (Janz Syndrome)
- Can also have absence seizures
What age group is affected by Janz Syndrome
12-16year olds, autosomal dominance with variable penentrance
What would exacerbate Janz Syndrome?
Sleep deprivation, Illness, photic stimulation
Triad for Lennox-Gastraut Syndrome?
- Multiple seizure types
- Diffuse congnitive dysfunction
- Slow generalised spike and slow EEG wave
Causes for Lennox Gastraut syndrome?
Seen with underlying encephalopathy and brain malformations
Management of Janz Syndrome?
Life long treatment - Valproic acid most commonly.
Mx of Lennox-Gastraut syndrome?
Valproic acid, benzodiazepines and ketogenic diet; however, response often poor
infantile acquired aphasia post seizure?
Landau Kleffner Syndrome:
Steven Johnson syndrome associated with ______ especially with given with carbamazepine
Lamatrogine
Why do you get non-communicating hydrocephalus?
Because of failure to communicate within the brain?
“Sun setting sign” - eye gaze downwards?
Hydrocephalus
Adenoma sebaceum is related to ______
Tuberous sclerosis
What is a port wine stain related to?
Sturge Weber disease
NF 1 is more common/uncommon?
Autosomal dominant/recessive
More common - dominant
Bilateral acoustic neuroma is related to _____
Neurofibromatosis 2
Small angiokeratoms seen on face - often in malar distribution are called adenoma sebaceum. This is related to _____
Tuberous sclerosis