Loss of topographic specificity of LTD-like plasticity is a trait marker in focal dystonia.
In focal hand dystonia, long-term potentiation (LTP) and depression (LTD)-like neuronal plasticity, as assessed by paired associative stimulation (PAS) targeting the hand-associated motor cortex, is enhanced and the topographic organization of plasticity is lost. However, if any of these abnormalities alone is sufficient to cause focal dystonia (FD) remains unknown. Ten patients with cervical dystonia (CD), 9 with blepharospasm (BS) and 16 age- and sex-matched controls were examined. PAS was performed by combining repetitively electric stimulation of the median nerve with subsequent transcranial magnetic stimulation of the contralateral motor cortex at 21.5ms (PAS21.5) and 10ms (PAS10). Corticospinal excitability was indexed by the magnitude of motor evoked potentials (MEPs) recorded from abductor pollicis brevis (APB) and abductor digiti minimi (ADM) muscles. In controls, MEP size of the homotopically conditioned APB increased after PAS21.5 whereas the MEP size of the heterotopically conditioned ADM remained stable. PAS10 led to a decrease of MEP size of the APB and to an increase of the heterotopic ADM. In contrast, after PAS21.5 and PAS10 in CD and BS MEP size increased and decreased, respectively, in both muscles. The magnitude of excitability changes, however, did not differ between dystonic patients and healthy controls. In FD the topographic organization of PAS21.5 and PAS10-induced plasticity is deranged in cortical areas not involved in the control of the dystonic body part. Somatotopical disorganization of plasticity may represent an endophenotypic trait in FD but may not be sufficient to generate a dystonic phenotype. Development of a dystonic phenotype may require that the gain of plasticity is additionally enhanced. This article is part of a Special Issue entitled "Advances in dystonia".
Department of Neurology, University of Wuerzburg, Josef-Schneider Str. 11, 97080 Wuerzburg, Germany.
David
D
Weise
Axel
A
Schramm
Marcus
M
Beck
Karlheinz
K
Reiners
Joseph
J
Classen
D000328:Adult; D000368:Aged; D001764:Blepharospasm / Q000503:physiopathology; D020821:Dystonic Disorders / Q000503:physiopathology*; D004558:Electric Stimulation; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006801:Humans; D017774:Long-Term Potentiation / Q000502:physiology*; D008297:Male; D008475:Median Nerve / Q000502:physiology; D008875:Middle Aged; D009044:Motor Cortex / Q000503:physiopathology*; D010641:Phenotype; D050781:Transcranial Magnetic Stimulation