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Human brain mapping in dystonia reveals both endophenotypic traits and adaptive reorganization
Author(s) -
Meunier Sabine,
Garnero Line,
Ducorps Antoine,
Mazières Leonor,
Lehéricy Stéphane,
Tézenas Du Montcel Sophie,
Renault Bernard,
Vidailhet Marie
Publication year - 2001
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.1234
Subject(s) - magnetoencephalography , dystonia , neuroscience , psychology , endophenotype , sensory system , upper limb , physical medicine and rehabilitation , medicine , electroencephalography , cognition
Dystonia has a wide clinical spectrum from early‐onset generalized to late‐onset sporadic, task‐specific forms. The genetic origin of the former has been clearly established. A critical role of repetitive skilled motor tasks has been put forward for the latter, while underlying vulnerability traits are still being searched for. Using magnetoencephalography, we looked for structural abnormalities reflecting a preexisting dysfunction. We studied finger representations of both hands in the primary sensory cortex, as compared in 23 patients with unilateral task‐specific dystonia and 20 control subjects. A dramatic disorganization of the nondystonic hand representation was found in all patients, and its amount paralleled the severity of the dystonic limb motor impairment. Abnormalities were also observed in the cortex coding the dystonic limb representation, but they were important only in the most severely affected patients. The abnormal cortical finger representations from the nondystonic limb appear to be endophenotypic traits of dystonia. That finger representations from the dystonic limb were almost normal for the less severely affected patients may be due to intrinsic beneficial remapping in reaction against the primary disorder.