title: Sporadic hemiplegic migraine presenting as acute encephalopathy.
abstract: A 10-year-old boy with psychomotor developmental delay and cerebellar vermis atrophy developed right hemiplegia with vomiting, unconsciousness, convulsions, and late-onset fever. Slow delta activity was noted over the left hemisphere on electroencephalography, and neuroimaging revealed swelling of the left temporo-occipital cerebral cortex with restricted diffusivity, successive transient cortical atrophy, and hyperperfusion over the left cerebral hemisphere. Interleukin-6 was elevated in the cerebrospinal fluid. The acute symptoms resolved completely within 3 weeks after onset, but hypoperfusion persisted in the left posterior cortex thereafter. Another episode with transient left hemiplegia appeared 7 months later, followed by recurrence of migraine attacks. Analysis of the CACNA1A gene revealed a mutation of c.1997 C>T (p.T666M). None of his family members had migraine. This case represents an unusual evolution of sporadic hemiplegic migraine with manifestations of acute encephalopathy, for which the role of migraine-related inflammatory process is assumed.
authors.affiliation: Department of Pediatrics, Kishiwada City Hospital, Kishiwada, Japan. ohmura@h8.dion.ne.jp
authors.forename: Kayo
authors.initials: K
authors.lastname: Ohmura
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.forename: Takahito
authors.initials: T
authors.lastname: Wada
authors.forename: Mikio
authors.initials: M
authors.lastname: Goto
authors.forename: Shiro
authors.initials: S
authors.lastname: Seto
mesh_terms: D001927:Brain Diseases / Q000175:diagnosis*; D015220:Calcium Channels / Q000235:genetics*; D002648:Child; D002658:Developmental Disabilities / Q000150:complications; D003937:Diagnosis, Differential; D004569:Electroencephalography; D006429:Hemiplegia / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D008297:Male; D008881:Migraine Disorders / Q000150:complications / Q000175:diagnosis* / Q000235:genetics*; D015899:Tomography, Emission-Computed, Single-Photon; D020864:Calcium Channels, N-Type
chemical_list: D015220:Calcium Channels; C105656:CACNA1A protein, human; D020864:Calcium Channels, N-Type