Alterations of cortical excitability and central motor conduction time in Wilson's disease.

Wilson's disease (WD) leads to widespread structural alterations of central nervous system and our objectives were to determine the cortical excitability changes in WD by using transcranial magnetic stimulation (TMS). Thirteen patients with WD, diagnosed by the presence of Kayser-Fleischer ring and biochemical tests, were studied. TMS was performed using a figure-of-eight coil attached to Magstim 200 stimulator. Motor evoked potentials (MEP) were recorded from right first dorsal interosseous at rest. Resting motor threshold (RMT) was determined using standard techniques and central motor conduction time (CMCT) by 'F' wave method. Comparison was made with control data of our laboratory. Dysarthria was the presenting symptom in 5 patients (38.5%) and chorea, tremors, dystonia and abnormal gait in 2 patients each (15.4%). RMT was recordable in 10 patients and not recordable in 3. Compared to controls, patients in whom RMT was recordable, had significantly higher mean RMT (80.9 ± 14.8 vs. 41.1 ± 7, p<0.0001) and CMCT (6.7 ± 0.5 ms vs. 4.8 ± 0.6 ms; p<0.0001). In 2 of the 3 patients with non-recordable RMT, MEP could be obtained with active contraction. CMCT in these 2 patients was also prolonged. Patients with WD have reduced cortical excitability and prolonged CMCT which may be due to the intracortical presynaptic motor dysfunction.

Alterations in cortical excitability and central motor conduction time in spinocerebellar ataxias 1, 2 and 3: a comparative study.

INTRODUCTION: Spinocerebellar ataxias 1, 2 and 3 (SCA1, SCA2 and SCA3) are CAG repeat disorders. The prevalence of changes in the cortical excitability and central motor conduction time (CMCT) in these disorders is largely unknown, and there are few studies which have compared these findings in the subtypes of SCA. The objectives of this study were to measure the cortical resting motor threshold (RMT) and CMCT using transcranial magnetic stimulation in patients with SCA1, SCA2, and SCA3. METHODS: The subjects of this study were 32 genetically confirmed patients with SCA (SCA1 = 15, SCA2 = 11, SCA3 = 6). Transcranial magnetic stimulation (TMS) was performed using a figure-of-eight coil attached to Magstim 200 stimulator. Motor evoked potentials were recorded from first dorsal interosseous at rest. RMT was determined using standard techniques and the CMCT by 'F' wave method. Comparison was made with data from 32 healthy controls. RESULTS: Compared to controls, the patients with SCA had significantly higher mean RMT as well as CMCT (RMT: 49.9 ± 9.1 vs. 41.5 ± 6.6, p < 0.0001; CMCT: 7.7 ± 2.3 ms vs. 4.8 ± 0.6 ms; p < 0.0001). When compared separately with the controls, while all the three subtypes of SCAs had significantly prolonged CMCT, only SCA1 and SCA3, but not SCA2 had significantly greater RMT. RMT and CMCT between patients with SCA2 and SCA3, and between SCA1 and SCA3 did not differ significantly, while SCA1 had significantly higher RMT and CMCT than SCA2. CONCLUSIONS: Patients with SCA have reduced cortical excitability and prolonged central motor conduction time, which was most evident in SCA1 and least in SCA2.