The intermuscular 3-7 Hz drive is not affected by distal proprioceptive input in myoclonus-dystonia.
In dystonia, both sensory malfunctioning and an abnormal intermuscular low-frequency drive of 3-7 Hz have been found, although cause and effect are unknown. It is hypothesized that sensory processing is primarily disturbed and induces this drive. Accordingly, experimenter-controlled sensory input should be able to influence the frequency of the drive. In six genetically confirmed myoclonus-dystonia (MD) patients and six matched controls, the low-frequency drive was studied with intermuscular coherence analysis. External perturbations were applied mechanically to the wrist joint in small frequency bands (0-4, 4-8 and 8-12 Hz; 'angle' protocol) and at single frequencies (1, 5, 7 and 9 Hz; 'torque' protocol). The low-frequency drive was found in the neck muscles of 4 MD patients. In these patients, its frequency did not shift due to the perturbation. In the torque protocol, the externally applied frequencies could be detected in all controls and in the two patients without the common drive. The common low-frequency drive was not be affected by external perturbations in MD patients. Furthermore, the torque protocol did not induce intermuscular coherences at the applied frequencies in these patients, as was the case in healthy controls and in patients without the drive. This suggests that the dystonic 3-7 Hz drive is caused by a sensory-independent motor drive and sensory malfunctioning in MD might rather be a consequence than a cause of dystonia.
Department of Neurology and Clinical Neurophysiology, Academic Medical Centre, Amsterdam, The Netherlands.
J N
JN
van der Meer
A C
AC
Schouten
L J
LJ
Bour
E
E
de Vlugt
A F
AF
van Rootselaar
F C T
FC
van der Helm
M A J
MA
Tijssen
D000328:Adult; D000344:Afferent Pathways / Q000503:physiopathology; D000368:Aged; D004421:Dystonia / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009119:Muscle Contraction / Q000502:physiology*; D018482:Muscle, Skeletal / Q000294:innervation / Q000503:physiopathology*; D009207:Myoclonus / Q000503:physiopathology*; D010812:Physical Stimulation / Q000379:methods; D011434:Proprioception / Q000502:physiology*; D013997:Time Factors; D019415:Torque; D014953:Wrist / Q000503:physiopathology