Triad of:
- Epilepsy in infant, sudden flexion in tonic fashion
- hypsarrhythmia on EEG
- mental retardation
West Syndrome
infantile spasms also known as Salaam spasm
Hemifacial contortions, inability speak, abnormal feelings in tongue
Benign rolandic epilepsy (Centrotemporal spikes)
Episodic visual distortions/ hallucinations, migraine like headaches
Benign occipital epilepsy
Drop attacks + mental retardation
Age 1-3
Lennox Gastaut syndrome
Intractable seizures (severe myoclonic epilepsy in infancy)
Dravet Syndrome
3Hz spike wave
Brought on y hyperventilation
Small Motor Sx (lip smacking, eyelid flickering)
Absence seizure
seizures after waking
Juvenile myoclonic epilepsy
Port wine stain
Sturge Weber Syndrome
Cafe au last spots + Lisch nodules
Neurofibromatosis Type 1
Lisch nodules = gold spots in iris
Bilateral vestibular schwannoma
Neurofibromatosis Type 2
Ash leaf patch + Shagreen patches + angiofibroma + seizure
Tuberous sclerosis complex
Telangiectasia + increased susceptibility to infection + ataxia
Ataxia telangiectasia
Champagne bottle legs + onion bulb appearance on nerve biopsy
Charcot Marie syndrome
Worsening ataxia + absent lower limb reflexes + extensor plantar response arises in 2nd decade of life
Friedrich’s ataxia
Clasp knife muscle tone
Spastic cerebral palsy
What is cerebral palsy?
Persistent unchanging disorder to movement and posture due to a defect/lesion in developing brain
Non progressive + motor / posture problems + insult to developing brain (<5)
Types of Cerebral Palsy (3)
- Spastic - 70% - damage to motor cortex or corticospinal tracts. Triad: limb tone +++, brisk reflexes + extensor plantar responses
- Diskinetic - damage to basal ganglia resulting in involuntary movements in whole body. Triad: dystonia, athetosis, chorea
- Ataxic - damage to cerebellum. Fine tremor worse on movement initiation
