Vitamin B6 (VB6) is used frequently as one of the first-choice
drug for the treatment of
West syndrome (WS) in Japan. We report 2 cases of symptomatic WS who had a good response to readministration of VB6. Case 1 was a 3-year-old girl diagnosed as having severe hydroencephalus. She developed WS at the age of 9 months. She was treated with
ACTH, but relapsed when she was 1 year old. Despite treatment with various conventional drugs and second
ACTH therapy, her
seizures were not suppressed. We reviewed past treatment records in another hospital, and found that VB, treatment was stopped because her EEG did not improve within a week. We then retried VB6
therapy when she was 3 years and 6 months old, and as a result she became seizure-free and
hypsarrhythmia disappeared on EEG within a month. Case 2 was a boy with severe
cerebral palsy who was diagnosed as having WS at the age of 9 months. His MRI revealed bilateral
subdural hematoma. Treatment was started with VB6, and he became seizure-free within several days. But 7 days after starting VB6, treatment was stopped because of the side effects. VPA was started instead, but his EEG showed gradual worsening. Then, we added a smaller dose of VB6 to VPA. His EEG improved dramatically, and his
seizures have been controlled without any side effects. The present cases indicate the possible clinical usefulness of successful VB6 retrials even in older patients with severe organic lesions, by means of combined
therapy with other
drug, and alternative design of the dosing. However, we suspect there might be many cases in which the efficacy of VB6
therapy has not been properly assessed due to the short observation period and/or side effects.