Focal spasms in clusters, focal delayed myelination, and hypsarrhythmia: unusual variant of West syndrome.
We report a patient who began to have clusters of seizures characterized by brief elevation of the right arm at 6 months of age. An interictal electroencephalogram (EEG) at 7 months revealed hypsarrhythmia without definite asymmetry. Simultaneous EEG and video recording disclosed that these focal spasms were associated with fast wave bursts superimposed on slow waves most markedly in the left centro-midtemporal region. The patient became seizure-free after synthetic ACTH therapy. The patient is developmentally normal at 3 years 5 months, but magnetic resonance imaging studies revealed findings suggestive of delayed myelination in the left frontal region. This patient is considered to have had an unusual variant of West syndrome associated with focal delayed myelination.
Watanabe
K
K
Department of Pediatrics, Nagoya University School of Medicine, Japan.
Haga
T
T
Negoro
T
T
Aso
K
K
Maeda
N
N
D000293:Adolescent; D002675:Child, Preschool; D003366:Cosyntropin / Q000008:administration & dosage; D003692:Delayed-Action Preparations; D004292:Dominance, Cerebral / Q000187:drug effects / Q000502:physiology; D004569:Electroencephalography / Q000187:drug effects; D004828:Epilepsies, Partial / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D005071:Evoked Potentials / Q000187:drug effects / Q000502:physiology; D005500:Follow-Up Studies; D005625:Frontal Lobe / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D009413:Nerve Fibers, Myelinated / Q000502:physiology*; D013036:Spasms, Infantile / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*
D003692:Delayed-Action Preparations; D003366:Cosyntropin; C006418:adrenocorticotropin zinc