BACKGROUND: West syndrome consists of infantile spasms with hypsarrhythmia and is perceived as a disorder of infants. METHODS: We describe 10 patients with West syndrome with spasms that remitted, started again, and persisted (followed up for 8-25 years). RESULTS: In all, West syndrome developed at younger than 17 months (five cryptogenic, six symptomatic). With initial treatment, spasms completely stopped for 4.5 months to 6 years, when epileptic spasms returned. Recurrent spasms were typical with brief arm extension, eye elevation, and head drop without falling. Spasms lasted 2-6 s in rhythmic strings over 20- to 60-min periods and occurred daily throughout follow-up. Persistent spasms were particularly troublesome, because of incontinence in one and postictal confusion in several. During the string of spasms, most refused to interact, and several would wander off. Up to 15 antiepileptic drugs did not render any patient spasm free. Only two had persistent spasms as the only seizure type; six also had intractable complex partial seizures, and three had occasional grand mal convulsions. Interictal EEGs showed multifocal spikes. Ictal recordings in six showed electrodecremental events. CONCLUSIONS: Recurrent spasms after remission of West syndrome represent an extremely resistant, distressing form of epilepsy. The onset of West syndrome is age related, but it does not reliably vanish.
BACKGROUND:West syndrome consists of infantile spasms with hypsarrhythmia and is perceived as a disorder of infants. METHODS: We describe 10 patients with West syndrome with spasms that remitted, started again, and persisted (followed up for 8-25 years). RESULTS: In all, West syndrome developed at younger than 17 months (five cryptogenic, six symptomatic). With initial treatment, spasms completely stopped for 4.5 months to 6 years, when epilepticspasms returned. Recurrent spasms were typical with brief arm extension, eye elevation, and head drop without falling. Spasms lasted 2-6 s in rhythmic strings over 20- to 60-min periods and occurred daily throughout follow-up. Persistent spasms were particularly troublesome, because of incontinence in one and postictal confusion in several. During the string of spasms, most refused to interact, and several would wander off. Up to 15 antiepileptic drugs did not render any patientspasm free. Only two had persistent spasms as the only seizure type; six also had intractable complex partial seizures, and three had occasional grand mal convulsions. Interictal EEGs showed multifocal spikes. Ictal recordings in six showed electrodecremental events. CONCLUSIONS: Recurrent spasms after remission of West syndrome represent an extremely resistant, distressing form of epilepsy. The onset of West syndrome is age related, but it does not reliably vanish.
Authors: Carlos J Ballester-Rosado; John T Le; Trang T Lam; Carrie A Mohila; Sandi Lam; Anne E Anderson; James D Frost; John W Swann Journal: Ann Neurol Date: 2022-05-10 Impact factor: 11.274