BACKGROUND: Movement disorder is common in Wilson's disease (WD), but there is no report on oromandibular dystonia (OMD). We report on frequency, severity, and MRI correlation of OMD in Wilson's disease with neurological manifestations (WDNM) and its response to treatment. METHODS: Consecutive WDNM patients were included and their clinical, hematological, serum chemistry, and MRI findings were noted. Neurological severity of WD and OMD were assessed. Burke-Fahn-Marsden (BFM) score for dystonia was noted. Patients were treated with penicillamine, zinc, and multiple antidystonic drugs. Clinical improvement at 3 and 6 months was noted. RESULTS: Overall, 61 of 67 (91%) WDNM patients had OMD, whose median age was 13.5 years. Median severity of OMD was 2.5 (range, 1-4). Thirteen patients were anarthric and 12 unable to eat. Severity of OMD correlated with drooling (r = 0.29; P = 0.02), BFM score (r = 0.63; P < 0.001), pancytopenia (r = -0.26; P = 0.04), and serum ceruloplasmin (r = 0.33; P = 0.01), but not with location and number of MRI lesions. Compared to baseline, severity of OMD improved at 6 months (P < 0.001), but not at 3 months. None became asymptomatic. Improvement in OMD paralleled with improvement in severity grade of WDNM (r = 0.26; P = 0.04) and with BFM score (r = 0.31; P = 0.02). CONCLUSION: OMD was a common manifestation of WDNM occurring in 91% patients, and OMD improved partially over the study period.
BACKGROUND: Movement disorder is common in Wilson's disease (WD), but there is no report on oromandibular dystonia (OMD). We report on frequency, severity, and MRI correlation of OMD in Wilson's disease with neurological manifestations (WDNM) and its response to treatment. METHODS: Consecutive WDNM patients were included and their clinical, hematological, serum chemistry, and MRI findings were noted. Neurological severity of WD and OMD were assessed. Burke-Fahn-Marsden (BFM) score for dystonia was noted. Patients were treated with penicillamine, zinc, and multiple antidystonic drugs. Clinical improvement at 3 and 6 months was noted. RESULTS: Overall, 61 of 67 (91%) WDNM patients had OMD, whose median age was 13.5 years. Median severity of OMD was 2.5 (range, 1-4). Thirteen patients were anarthric and 12 unable to eat. Severity of OMD correlated with drooling (r = 0.29; P = 0.02), BFM score (r = 0.63; P < 0.001), pancytopenia (r = -0.26; P = 0.04), and serum ceruloplasmin (r = 0.33; P = 0.01), but not with location and number of MRI lesions. Compared to baseline, severity of OMD improved at 6 months (P < 0.001), but not at 3 months. None became asymptomatic. Improvement in OMD paralleled with improvement in severity grade of WDNM (r = 0.26; P = 0.04) and with BFM score (r = 0.31; P = 0.02). CONCLUSION: OMD was a common manifestation of WDNM occurring in 91% patients, and OMD improved partially over the study period.
Authors: S Sinha; A B Taly; S Ravishankar; L K Prashanth; K S Venugopal; G R Arunodaya; M K Vasudev; H S Swamy Journal: Neuroradiology Date: 2006-06-03 Impact factor: 2.804
Authors: Steven E Lo; Axel J Rosengart; Roberta L Novakovic; Un Jung Kang; Darshan N Shah; Mohsin A Khan; Arif Dalvi; Fernando D Goldenberg; R Loch Macdonald; Jeffrey I Frank Journal: Neurocrit Care Date: 2005 Impact factor: 3.210
Authors: Leandro T Lucato; Maria Concepción G Otaduy; Egberto R Barbosa; Alexandre A C Machado; Alexander McKinney; Luiz A Bacheschi; Milberto Scaff; Giovanni G Cerri; Claudia C Leite Journal: AJNR Am J Neuroradiol Date: 2005-05 Impact factor: 3.825
Authors: J L Rodrigo Agudo; M Valdés Mas; A M Vargas Acosta; M L Ortiz Sánchez; M L Gil del Castillo; L F Carballo Alvarez; J A Pons Miñano Journal: Rev Esp Enferm Dig Date: 2008-08 Impact factor: 2.086