infantile spasms
A severe epilepsy syndrome of infancy characterized by clusters of sudden muscle contractions and associated with developmental regression.
Full Definition
Infantile spasms, also known as West syndrome, is a catastrophic epilepsy syndrome typically beginning between 3-12 months of age. The condition is characterized by clusters of sudden, brief muscle contractions (flexor, extensor, or mixed spasms) that often occur upon awakening or falling asleep. The syndrome includes three components: infantile spasms, hypsarrhythmia on electroencephalogram, and developmental regression or arrest. The condition can be cryptogenic (unknown cause) or symptomatic (due to underlying brain abnormalities, metabolic disorders, or genetic conditions). Prompt recognition and treatment are crucial, as delays in therapy can worsen developmental outcomes. First-line treatments include ACTH (adrenocorticotropic hormone) or vigabatin, with other antiepileptic drugs used as adjunctive therapy.
Usage
Usage note: West syndrome refers to the complete triad. Note that 'salaam seizures' is a descriptive term but less precise.
In Context
- "Video EEG confirmed infantile spasms with associated hypsarrhythmia pattern." — Neurology consultation report