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 m...

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Bibliographic Details
Published in:Pediatric neurology 2012-09, Vol.47 (3), p.201-204
Main Authors: Asghar, Sheila J., MD, MSc, Milesi-Hallé, Alessandra, MD, PhD, Kaushik, Chavvi, MD, Glasier, Charles, MD, Sharp, Gregory B., MD
Format: Article
Language:English
Subjects:
Adolescent
Anti-Inflammatory Agents - therapeutic use
Biological and medical sciences
Brain Edema - complications
Brain Edema - physiopathology
Calcium Channel Blockers - therapeutic use
Calcium Channels - genetics
Cerebral hemispheres
Child
Children
Cognition Disorders - etiology
Cognitive ability
DNA - genetics
Edema
EEG
Electroencephalography
Female
Functional Laterality
gait
Gait Disorders, Neurologic - etiology
Genetic screening
Head
Headache
Humans
Image Processing, Computer-Assisted
Magnetic Resonance Imaging
Male
Medical sciences
Methylprednisolone - therapeutic use
Migraine
Migraine with Aura - complications
Migraine with Aura - genetics
Migraine with Aura - physiopathology
Mutation
Neurologic Examination
Neurology
Pediatrics
Skull
Sodium-Potassium-Exchanging ATPase - genetics
speech
Trauma
Vascular diseases and vascular malformations of the nervous system
Verapamil - therapeutic use
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Summary: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.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2012.05.006