Intermediate
Technical
IVT
West syndrome
Epileptic encephalopathy characterized by infantile spasms, hypsarrhythmia, and developmental regression.
Full Definition
A severe epileptic encephalopathy that typically manifests in the first year of life, characterized by the triad of infantile spasms, hypsarrhythmia on EEG, and developmental regression or arrest. Named after Dr. William James West, who first described the condition in his own son in 1841. The syndrome can be cryptogenic (unknown cause) or symptomatic (due to underlying brain pathology), and early treatment with ACTH or vigabatrin is crucial for optimal outcomes.
Usage
Usage note: Always capitalize 'West' as it is an eponymous syndrome.
In Context
- "The patient was diagnosed with West syndrome at 6 months of age following onset of clustering spasms." — Pediatric neurology consultation
- "ACTH therapy was initiated promptly for treatment of West syndrome." — Treatment plan
Also known as
infantile spasm syndrome
Don't confuse with
Lennox-Gastaut syndrome
Dravet syndrome