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Oromandibular Dystonia in Wilson's Disease
Jayantee Kalita1, Abhay Ranjan1, Usha K Misra1
1Department of Neurology Sanjay Gandhi Post Graduate Institute of Medical Sciences Lucknow India.
Movement Disorders Clinical Practice
|October 27, 2018
Summary
Oromandibular dystonia (OMD) affects 91% of Wilson
Area of Science:
- Neurology
- Genetics
- Biochemistry
Background:
- Wilson's disease (WD) commonly causes movement disorders.
- Oromandibular dystonia (OMD) has not been previously reported in WD.
- This study investigates OMD in patients with neurological manifestations of WD (WDNM).
Purpose of the Study:
- To determine the frequency and severity of OMD in WDNM.
- To explore correlations between OMD and clinical/MRI findings.
- To assess the treatment response of OMD in WDNM.
Main Methods:
- Consecutive WDNM patients were evaluated for OMD.
- Neurological severity and OMD were assessed using the Burke-Fahn-Marsden (BFM) score.
- Patients received penicillamine, zinc, and antidystonic drugs, with follow-up at 3 and 6 months.
Main Results:
- OMD was present in 91% of WDNM patients (61/67).
- OMD severity correlated with drooling, BFM score, pancytopenia, and serum ceruloplasmin.
- OMD severity improved significantly at 6 months post-treatment (P < 0.001).
Conclusions:
- OMD is a highly prevalent manifestation in WDNM.
- Treatment led to partial improvement in OMD, paralleling overall WD improvement.
- OMD requires attention in the management of neurological Wilson's disease.