Hemiplegic migraine is a rare variety of migraine with aura, characterized by motor deficits during the aura, often beginning in childhood. The hemiplegic attacks can be severe and prolonged but the prognosis is usually good. Data on neuroimaging, including diffusion-weighted images (DWI) and spectroscopy, during prolonged attacks of HM are quite limited, particularly in children. We present an 8-year-old female with a prolonged attack of sporadic hemiplegic migraine characterized by right-sided hemiplegia, global aphasia, fever and impairment of consciousness. An extensive and multimodal neuroimaging follow-up study was undertaken. The first magnetic resonance imaging (MRI) was negative while the following MRI (day 4 and 11) documented a progressive increase in cortical swelling in the left hemisphere with mild hyperintensity in the DWI and mild reduction of apparent diffusion coefficient values, suggesting intracellular edema. Proton MRI spectroscopy (MRS) (day 15) showed a decrease in the N-acetylaspartate/creatine ratio in the affected hemisphere, consistent with reduced neuronal metabolic activity and neuronal loss. 99mTc- ECD brain perfusion single photon emission tomography (SPET) (day 27) showed marked left hemispheric hypoperfusion, suggesting primary neuronal dysfunction, and slight hypoperfusion of the right cerebellar cortex, consistent with crossed cerebellar diaschisis. The patient recovered completely after 40 days and the neuroimaging follow-up (MRI and SPET) after 6 months was normal. Molecular analysis disclosed a missense mutation of the ATP1A2 gene. Multimodal neuroimaging (MRI, DWI, MRS and SPET) provides a significant contribution to understanding the pathogenesis of a prolonged HM attack, supporting evidence for a primary neuronal dysfunction.

Multimodal neuroimaging in a child with sporadic hemiplegic migraine: A contribution to understanding pathogenesis.

TOLDO, IRENE;CECCHIN, DIEGO;SARTORI S;DRIGO, PAOLA;BATTISTELLA, PIER ANTONIO
2011

Abstract

Hemiplegic migraine is a rare variety of migraine with aura, characterized by motor deficits during the aura, often beginning in childhood. The hemiplegic attacks can be severe and prolonged but the prognosis is usually good. Data on neuroimaging, including diffusion-weighted images (DWI) and spectroscopy, during prolonged attacks of HM are quite limited, particularly in children. We present an 8-year-old female with a prolonged attack of sporadic hemiplegic migraine characterized by right-sided hemiplegia, global aphasia, fever and impairment of consciousness. An extensive and multimodal neuroimaging follow-up study was undertaken. The first magnetic resonance imaging (MRI) was negative while the following MRI (day 4 and 11) documented a progressive increase in cortical swelling in the left hemisphere with mild hyperintensity in the DWI and mild reduction of apparent diffusion coefficient values, suggesting intracellular edema. Proton MRI spectroscopy (MRS) (day 15) showed a decrease in the N-acetylaspartate/creatine ratio in the affected hemisphere, consistent with reduced neuronal metabolic activity and neuronal loss. 99mTc- ECD brain perfusion single photon emission tomography (SPET) (day 27) showed marked left hemispheric hypoperfusion, suggesting primary neuronal dysfunction, and slight hypoperfusion of the right cerebellar cortex, consistent with crossed cerebellar diaschisis. The patient recovered completely after 40 days and the neuroimaging follow-up (MRI and SPET) after 6 months was normal. Molecular analysis disclosed a missense mutation of the ATP1A2 gene. Multimodal neuroimaging (MRI, DWI, MRS and SPET) provides a significant contribution to understanding the pathogenesis of a prolonged HM attack, supporting evidence for a primary neuronal dysfunction.
2011
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11577/2449132
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