Abstract
Three patients (2 females, one male) with infantile spasms (IS) associated with partial seizures (PS) are reported. All cases had an unremarkable perinatal history and had shown normal development until seizure onset. Using prolonged video/EEG monitoring we confirmed PS intervened during the cluster of IS in two cases and that another patient developed IS several seconds later following PS. Interictal EEG showed hypsarrythmia in all. MRI revealed a focal cystic lesion in the left temporal lobe in one case and the other case showed hypoperfusion of the left temporal region on SPECT. In all patients IS responded initially to VPA with vitamin 136 or ACTH, but relapsed. Two patients responded ACTH again, while one medically refractory girl with an anatomical lesion on MRI underwent surgical resection with excellent outcome. Pathology of the resected lesion showed a cortical dysplasia. Her development was normal at onset (DQ 91 at age 10 months), but moderately retarded just before operation (DQ 49 at age 3 years). After operation she has been seizure-free for 25 months, but continues to exhibit some degree of developmental delay (DQ 53 at age 3 years 11 months). Surgical intervention might be considered early in the medically refractory cases with anatomical lesions.
