A

The first patient is currently an 18-year-old woman who is moderately mentally handicapped and has two predominant seizure types – partial complex seizures and persistent infantile spasms.

She was born after a normal pregnancy to healthy, non-related parents with no family history of neurological problems. At 6 months of age she had a 4-day history of myoclonic seizures and flexor spasms occurring in clusters. Neurologic examination and development were normal for age. A routine EEG showed typical hypsarrhythmia without focal features. and a computed tomography (CT) scan was normal. A diagnosis of West syndrome was made and she was treated with adrenocorticotropic hormone (ACTH) (40 units intramuscularly) and nitrazepam (0.2 mg/kg twice daily) with an immediate, marked decrease in spasms and a normalization of the EEG over 3 weeks. However, occasional spasms continued for the next 6 weeks and ACTH was increased to 60 units/day for an additional 6 weeks. ACTH was discontinued at 8 months of age and nitrazepam at 10 months of age.