Paediatric Neurology Flashcards
Explain features of infantile spasms/West’s syndrome?
Breif spasms which occur in first few months of line.
1. Flexion of head, trunk and limbs (extension of arms)
2. Progressive mental handicap.
3, EEG - hypsarrhythmias.
Usually secondary to serious neurological abnormality or may be cryptogenic.
First line treatment - prednisolone or vigabatrin.
Explain features of Lennox-Gastaut syndrome
Extension of infantile spasms - onset age 1-5 years.
Atypical absences, falls or jerks.
90% have mod-severe mental handicap.
EEG: slow spikes
Ketogenic diet may help
Explain features of benign rolandic epilepsy
Paraesthesia, seizures characteristically occur at night.
Features of juvenile myoclonic epilepsy (Janz syndrome_
Typical onset in teens.
1. Infrequent generalized seizures (more common in morning).
2. Daytime absences.
3. Sudden, shock-like myoclonic seizures
Good response to sodium valproate
Features of cyanotic breath holding spells
Occurs when child is really upset - after letting out long cry they stop breathing, become cyanotic and lose consciousness
Features of reflex anoxic seizures?
Occurs when child is startled. Vagus nerve sends signal to heart which causes it to stop beating.
Child suddenly goes pale, loses consciousness and may have some seizure type muscle twitching.
Explain features of abdominal migraine
Symptoms occur in children before they develop traditional migraine.
Presents with episode of central abdominal pain lasting > 1 hour. May have associated symptoms of N+V, anorexia, headaches and pallor
Managements of migraines in children?
Rest, fluids and low stimulus environment,
Paracetamol, ibuprofen or sumatriptan.
Antiemetic
Prophylaxis - propanolol, pizotifen (drowsiness), topiramate.
Congenital causes of hydrocephalus?
Most common - aqueductal stenosis.
Arachnoid cysts.
Arnold-chiari malformation.
Chromosomal abnormalities
Presentation and management of hydrocephalus in paeds?
Presentation - enlarged occipito-frontal circumference, bulging anterior fontanelle, poor feeding and vomiting, poor tone and sleepiness.
Rx - VP shunt. Complications include infection, blockage, excessive drainage, IV haemorrhage and outgrowing them
What is craniosynostosis?
Skull sutures close prematurely
What is the head shape in the follow types of synostosis?
Saggital
Coronal
Metopic
Lambdoid
Saggital synostosis - long and narrow from front to back.
Coronal - Blging on one side of the forehead.
Metopic - pointy triangular forehead
Lambdoid - flattening on one side of occiput.
Investigations and management of craniosynostosis?
Ix - skull x-ray (first line), head CT if doubt on x-ray.
Rx - surgical reconstruction of skull
Explain features of duchennes muscular dystrophy
Defect gene for dystrophin on X-chromosome.
Presents age 3-5 years with weakness in muscles around pelvic. Usually wheelchair bound by teenager. Life expectancy 23-35 years.
Oral steroids slows progression of muscle weakness. Creatine supplement may improve muscle strength.
Associated with dilated cardiomyopathy
Features of Becker muscular dystrophy
Dystrophin gene less severely affected.
Onset of symptoms 8-12 years. may be wheelchair bound by later 20-30s.
