title: Variable manifestations of familial hemiplegic migraine associated with reversible cerebral edema in children.
abstract: Three children with familial hemiplegic migraine presented with right-sided weakness, speech difficulty, altered mental status, and gait abnormalities. These persistent aura signs were accompanied by left-sided slowing and cerebral dysfunction, documented by electroencephalograms. Cranial magnetic resonance imaging revealed cortical edema restricted to the left cerebral hemisphere. Follow-up electroencephalogram and imaging studies produced normal results 1-4 months afterward. However, cognitive changes persisted. Genetic testing demonstrated variable results: one child manifested a CACNA1A mutation compatible with familial hemiplegic migraine type 1, whereas another demonstrated an ATP1A2 sequence alteration. No known mutations were evident in the third child, with minor head trauma thought to precipitate the familial hemiplegic migraine. These findings demonstrate the variable clinical and genetic heterogeneity of childhood familial hemiplegic migraine.
authors.affiliation: Section of Pediatric Neurology, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. asgharsheilaj@uams.edu
authors.forename: Sheila J
authors.initials: SJ
authors.lastname: Asghar
authors.forename: Alessandra
authors.initials: A
authors.lastname: Milesi-Hallé
authors.forename: Chavvi
authors.initials: C
authors.lastname: Kaushik
authors.forename: Charles
authors.initials: C
authors.lastname: Glasier
authors.forename: Gregory B
authors.initials: GB
authors.lastname: Sharp
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001929:Brain Edema / Q000150:complications / Q000503:physiopathology*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D015220:Calcium Channels / Q000235:genetics; D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D004247:DNA / Q000235:genetics; D004569:Electroencephalography; D005260:Female; D007839:Functional Laterality; D020233:Gait Disorders, Neurologic / Q000209:etiology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D020325:Migraine with Aura / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D009460:Neurologic Examination; D000254:Sodium-Potassium-Exchanging ATPase / Q000235:genetics; D014700:Verapamil / Q000627:therapeutic use; D020864:Calcium Channels, N-Type
chemical_list: D000893:Anti-Inflammatory Agents; D002121:Calcium Channel Blockers; D015220:Calcium Channels; D004247:DNA; D008775:Methylprednisolone; D000254:Sodium-Potassium-Exchanging ATPase; D014700:Verapamil; C105656:CACNA1A protein, human; D020864:Calcium Channels, N-Type; C499076:ATP1A1 protein, human