West syndrome
An age-specific epileptic encephalopathy characterized by infantile spasms, hypsarrhythmia, and developmental regression.
Full Definition
West syndrome is a severe epileptic encephalopathy that typically begins in the first year of life, characterized by the clinical triad of infantile spasms (brief, sudden flexor or extensor contractions), hypsarrhythmia (a chaotic, high-amplitude EEG pattern), and psychomotor developmental arrest or regression. Named after Dr. William James West who described his own son's condition in 1841, this syndrome has multiple etiologies including structural brain abnormalities, genetic mutations, and metabolic disorders. Early recognition and treatment with specific therapies such as ACTH or vigabatrin are crucial for optimizing developmental outcomes, though the prognosis varies significantly based on the underlying etiology.
Usage
Usage note: Eponym; often leads to other epilepsy syndromes if not adequately treated.
In Context
- "Early diagnosis of West syndrome led to prompt initiation of ACTH therapy." — Pediatric neurology report
- "The infant's developmental regression and characteristic spasms suggested West syndrome, confirmed by EEG showing hypsarrhythmia." — Clinical case presentation