Objective: To establish the frequency and types of dystonia in our cohort of Wilson’s disease patients.

Background: Wilson’s disease (WD) belongs to movement disorders with complex combination of neurological symptoms (tremor, dystonia, parkinsonism, ataxia). Dystonia is a common feature of WD. However, previous studies reported dystonia in the wide range of frequencies (11-65%). Thus, the aim of the present study was to establish the frequency and types of dystonia in our cohort of Wilson’s disease patients.

Methods: We analyzed 108 WD patients with neurological features at the time of diagnosis between 2005 and 2015. The assessment was based on the Unified Wilson’s Disease Score Scale (UWDRS). The patients were divided into 6 groups according predominant neurological feature: tremor, ataxia, parkinsonism, dystonia, mixed. In the UWDRS, dystonia is characterized as oromandibular, cervical, arm, hand, trunk, and leg dystonia. All types of dystonia were assessed for four stages of severity (0=normal, 4=severe).

Results: Among analyzed neurological patients with WD dystonia was reported in 39 (36%) patients with WD. Ten patients (25%) out of 39 had neurologic feature with predominant dystonia. Remaining 29 cases had mixed feature. The oromandibular dystonia was most common (76%; 30/39), less frequently observed was foot and limb dystonia (46%, 18/39), cervical dystonia (33%; 13/39), 8% trunk dystonia (25%; 10/39), arm and hand dystonia (23%; 9/39). Generalized dystonia was observed in four patients (10%).

Conclusions: Dystonia is common clinical feature of WD. The most common is focal dystonia. The most disabling generalized dystonia is less frequent observed.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/frequency-and-types-of-dystonia-in-wilsons-disease-patients/