Neurology Flashcards
What are the EEG changes in infantile spasms?
Hypsarrhythmia
A triad of high amplitude, disorganised background and multifocal discharges
What is the treatment of infantile spasms?
ACTH
Vigabatrin
What is the typical features of infantile spasms?
Age of onset - between 4-7 months
Occur on clusters
Rapid flexion of the trunk, neck and extremities
Often occurs at beginning or end of sleep
What are the EEG findings of absence seizures?
3Hz generalised spike-wave discharges
What is the treatment of absence seizures?
Ethosuximide
What is the prognosis of absence seizures?
Majority of children will grow out of them by adolescences
What are the features of absence seizures?
Presentation between 4-10 years of age
Staring, lack of a postictal state
Automatisms
What are the features of benign Rolandic epilepsy?
Seizures soon after sleep onset or just before awakening
Onset between 4-11 years
Facial twitching and drooling
What is the treatment of benign rolandic epilepsy?
Spontaneously remit
If Tx is considered - 1st line is carbamazepine
Which condition presents with jerking movements of the upper extremities upon awakening?
Juvenile myoclonic epilepsy
What are the EEG findings in juvenile myoclonic epilepsy?
EEG 4-6Hz (spike and polyspike wave)
What is the treatment of juvenile myoclonic epilepsy?
Sodium valproate
May also consider lamotrigine or levetiracetam
What is the treatment of choice for partial seizures?
1st line - Carbamazepine
2nd line - Levetiracetam (can be given alongside COCP)
What is the treatment of choice for generalised tonic clonic seizures?
1st line - Sodium valproate
2nd line - Levetiracetam
What are the common side effects of ethosuximide?
GI upset
Leukopenia
What are the common side effects of carbamazepine?
Rash - SJS
SIADH/ hyponatraemia
Hepatotoxicity
Leucopenia
What are the common side effects of phenytoin?
Zero order kinetics - the rate of elimination is constant regardless of the plasma concentration of the drug
Hirsutism
Gum hypertrophy
Teratogenic
What are the common side effects of Lamotrigine?
Stevens-Johnson syndrome
What are the side effects of sodium valproate?
Weight gain
Tremor
Hair loss
Dose related thrombocytopenia
Hepatotoxicity
Hyperammonaemic encephalopathy
What are the side effects of topiramate?
Closed angle glaucoma
What is Sandifier syndrome?
Back arching, dystonic posturing of the limbs - may be provoked by feeding or lying flat (may be alleviated by sitting up)
Due to GORD
What is the origin of the nerves and skin?
Ectoderm
Where do neurocutaneous conditions originate embryologically from?
Ectoderm
What is the inheritance of NF1?
Autosomal dominant
Mutation on chromosome 17
Onset in childhood
What are the diagnostic criteria of NF1?
2 or more of the following:
- 6 or more cafe-au-lait spots (>5mm in pre-pubertal, >15mm in post-pubertal)
- Axillary of inguinal freckling
- Two or more iris/ lisch nodules
- Distinctive bony lesion (sphenoid dysplasia)
- Two or more neurofibromas
- Optic glioma (<6 years)
- 1st degree relative with NF1
What complications can occur in neurofibromatosis type 1?
Renal vascular stenosis
Phaeochromocytoma
What is the inheritance of NF2?
Autosomal dominant
Mutation in chromosome 22
What is the inheritance of tuberous sclerosis?
Autosomal dominant
Mutation on chromosome 9 and 16
What are the main features of tuberous sclerosis?
Ash leaf spots
Shagreen patch
Periungal fibromas
Facial angiofibromas
Cardiac rhambdomyoma
Renal and pulmonary angiomyolipomas
Subependymal giant cell astrocytomas
What are the clinical features of Sturge-Weber Syndrome?
Facial port wine stain in V1, V2 or even V3 distribution of the trigeminal nerve
Glaucoma
Seizures
May develop ipsilateral cerebral leptomeningealangioma - may calcify over time and cause tram track/ railroad calcifications on CT scan
Which conditions are commonly associated with Sturge-Weber syndrome?
Developmental delay
Learning disabilities
ADHD
Contralateral hemiparesis
Soft tissue hypertrophy
Hemianopia
What are the EEG findings in subacute sclerosing panencephalitis?
Burst suppression
What is Brown-Sequard syndrome?
Ipsilateral loss of vibration, weakness and position sense and contralateral loss of temperature and pain sensation
Is Bells palsy upper or lower motor neurone related?
Lower motor neurone palsy
Forehead is included
