West syndrome/infantile spasms
- This term is being used less frequently as it appears not to be a homogeneous group and as specific etiologies for infantile spasms are identified.
- Triad of
- Mental retardation
- Infantile spasm
- Classically a seizure disorder that usually appears in first year of life, and consists of recurrent, gross flexion and occasionally extension of the trunk and limbs (massive myoclonus, AKA infantile spasms, AKA salaam seizures, AKA jackknife spasms).
- Seizures tend to diminish with age, often abating by 5 yrs.
- 50% may develop complex-partial seizures, some of the rest may develop Lennox-Gastaut syndrome.
- Hyperarrhythmia on EEG
- Huge spike/wave plus slow wave resembling muscle artefact
- An associated brain lesion may be found in some.
- Treatment:
- ACTH OR
- Corticosteroids
Others
- Myoclonic epilepsy of infancy
- Benign infantile epilepsy
- Benign familial infantile epilepsy
- Dravet syndrome
- Epilepsy of infancy with migrating focal seizures
- Myoclonic encephalopathy in non-progressive disorders
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