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A 7-year-old boy with prior episodes of hemiplegia and family history of hemiplegic migraine presented with fevers, waxing and waning encephalopathy (lethargic and poorly interactive), and left-sided weakness persistent throughout… Click to show full abstract
A 7-year-old boy with prior episodes of hemiplegia and family history of hemiplegic migraine presented with fevers, waxing and waning encephalopathy (lethargic and poorly interactive), and left-sided weakness persistent throughout his 5-week hospitalization. Diagnostic testing revealed a suspected pathogenic mutation in ATP1A2 (c.2285G>C; p.Gly762Ala), a gene associated with a broad phenotypic spectrum encompassing familial hemiplegic migraine type 2 (FHM2) and alternating hemiplegia of childhood. Previous case series illustrate that FMH2 attacks can be prolonged, debilitating, and associated with impaired consciousness and fever.1 Cortical edema has been described, but transient diffusion restriction can be another radiologic feature (figures 1 and 2).2
Journal Title: Neurology
Year Published: 2020
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