Abstract
Writer's cramp (focal hand dystonia) is a sporadic focal dystonia that affects a specific part of the upper limb causing excessive co-contraction of antagonistic muscles. It usually presents as a task-specific dystonia, including, among others, writing of a character or playing a musical instrument. Although treatments for writer's cramp exist, medical therapy often results in unsatisfactory outcomes in patients with this type of dystonia. However, accumulating evidence suggests that long-term and complete remission of various types of focal hand dystonia can be achieved with stereotactic ablation or deep brain stimulation of the thalamic ventral-oralis complex (Vo) nucleus, which includes both the ventralis oralis posterior and anterior nuclei of the thalamus. Following the striking therapeutic success of Vo thalamotomy in patients with medically-refractory writer's cramp, we here introduce the use of stereotactic lesioning of the thalamic Vo nucleus for the treatment of this focal type of dystonia. Our findings identified patients with disabling writer's cramp (i.e., it prevents their success in their professional careers) to be good candidates for positive outcome with this surgical technique.
Highlights
Among dystonia syndromes, focal hand dystonia (FHD) is as a sporadic focal dystonia with adult onset, typically between the ages of 30 and 50 years [1]
The magnetic resonance (MR) images with fluid-attenuated inversion recovery (FLAIR) showed a vasogenic edema surrounding the lesions on the fourth post-operative day (Figures 2D,E), which almost disappeared at 1–2 years after surgery (Figure 2F)
This study provides evidence that stereotactic ablation of the thalamic ventral-oralis complex (Vo) nucleus represents a successful therapeutic strategy, devoid of any permanent complication in patients with writer’s cramp
Summary
Focal hand dystonia (FHD) is as a sporadic focal dystonia with adult onset, typically between the ages of 30 and 50 years [1]. Considering that FHD usually manifests as a task-specific dystonia, it can be classified as an occupational dystonia, referred to the writer’s, typist’s, guitarist’s, or pianist’s cramp [2, 4] As this dystonia can have a widespread effect on muscles, it can involve different tasks, and, in some instances, it can progress to the other hand [5]. Patients with unilateral Vo thalamotomy were included in this study if they met the following criteria: [1] diagnosis of writer’s cramp according to the consensus statement of Albanese et al [1], [2] men and women, aged at 18–80 years, [3] medically-refractory writer’s cramp that prevents their success in their professional careers, and [4] written informed consent to participate in this assessment. The surface EMG examination to determine the responsible muscles was routinely performed before the surgery
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