Sporadic hemiplegic migraine presenting as acute encephalopathy |
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Authors: | Ohmura Kayo Suzuki Yasuhiro Saito Yoshiaki Wada Takahito Goto Mikio Seto Shiro |
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Affiliation: | Department of Pediatrics, Kishiwada City Hospital, Kishiwada, Japan. ohmura@h8.dion.ne.jp |
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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. |
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Keywords: | Hemiplegic migraine Neuroimaging Inflammation Interleukin-6 |
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