West syndrome
An age-specific epilepsy syndrome of infancy characterized by infantile spasms, hypsarrhythmia on EEG, and developmental regression.
Full Definition
West syndrome is a severe epileptic encephalopathy that typically begins in the first year of life, most commonly between 3-12 months of age. The syndrome is defined by a triad of clinical features: infantile spasms (brief, symmetric contractions of the neck, trunk, and extremities), hypsarrhythmia (a chaotic, high-amplitude EEG pattern), and psychomotor regression or developmental arrest. West syndrome can be cryptogenic (unknown cause) or symptomatic (due to underlying brain abnormalities). Early recognition and treatment with specific medications like vigabatrin or ACTH are crucial, as delayed treatment can lead to poor developmental outcomes and evolution to other epilepsy syndromes.
Usage
Usage note: Named after Dr. William James West. Always capitalize 'West' as it is an eponym.
In Context
- "The 8-month-old was diagnosed with West syndrome and started on vigabatrin therapy." — Pediatric neurology note
- "Early treatment of West syndrome is essential to prevent developmental regression and optimize outcomes." — Clinical guideline