Abstract

GRIPPO, Jorge  and  GRIPPO, Tomás M.. Canalopatías en pediatría: hemiplejías episódicas. Arch. argent. pediatr. [online]. 2007, vol.105, n.2, pp.154-158. ISSN 0325-0075.

Introduction. Molecular advances are developed to study channels function and its alterations (channelopathies). The last decade, several paroxysmal manifestations were described on this pathology. The occurrence of transient attacks of hemiplegia involving alternatively either side of the body is an uncommon event in infant and children. Transient hemiplegia is associated to tonic or dystonic attacks and paroxysmal nistagmus. The disease is progressive with delayed cognitive development or mental retardation. Alternating Hemiplegia probably is a result of a mutation in ATP1A2 gen. Treatment with flunarizine and other drugs remains improven whereas its effect on the duration and severity of attacks is not established. Familial Hemiplegic Migraine, associated to ataxia (FHM2) or without ataxia (FHM1) depends on mutation of ATP1A2 and CACNA1A gene respectivelly. Migraine appears with visual aura (scotoma, photophobia, diplopia), language difficulty and hemiplegia of variable duration (hours or days) and headhache. MRI spectrography shows modifications in aspartate glutamate and mioinositol on cerebellar vermis. Treatment is proved with acetozolamide, calcium channel blocking agents.

Keywords : Channelopathies; Alternating hemiplegia; Familial hemiplegic migraine; Molecular basis.

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