A. Munchau et al., Arm tremor in cervical dystonia differs from essential tremor and can be classified by onset age and spread of symptoms, BRAIN, 124, 2001, pp. 1765-1776
The pathophysiology of arm tremor in patients with cervical dystonia (CD) a
nd its relationship to other types of tremor is unclear. In the present stu
dy, we have compared the tremor in these patients with that seen in patient
s with essential tremor (ET) using two neurophysiological techniques: the t
riphasic EMG pattern accompanying ballistic wrist flexion movements; and re
ciprocal inhibition between forearm muscles. During ballistic wrist flexion
movements, the latency of the second agonist EMG burst was later in ET tha
n CD patients. This suggests that the mechanism of the arm tremor in CD may
differ from that in ET. There was no group difference between reciprocal i
nhibition in patients with ET or CD. However, there was much more variabili
ty in the data from patients with CD. Because of this, we subdivided the CD
patients into two groups, group A with normal levels of presynaptic inhibi
tion and group B with reduced or absent presynaptic inhibition. A posterior
i, it turned out that the patients in these two subgroups had similar clini
cal symptoms, but different clinical histories. The arm tremor of patients
in group A started simultaneously with torticollis (mean onset age of arm t
remor 40 years +/- 20.7 SD, interval between onset of arm tremor and tortic
ollis 0 +/- 2.9 years) whereas it began much earlier (mean onset age 14 yea
rs +/- 6 SD) and preceded onset of torticollis by a longer interval (21.6 /- 17.5 years) in patients of group B. Patients in group A also had less co
-contraction in their ballistic wrist movements between the first agonist a
nd the antagonist burst than those patients in group B. We conclude that ar
m tremor in patients with CD may have a mechanism different from that seen
in patients with ET. Moreover, the data imply that there are two subgroups
of CD patients with arm tremor, one with a late and simultaneous onset of a
rm tremor and torticollis (group A), and another with an early onset of arm
tremor and later development of torticollis (group B). These groups do not
correspond to the currently proposed clinical subdivision of 'dystonic tre
mor' and 'tremor associated with dystonia'.