Predictors of alcohol responsiveness in dystonia

Johanna Junker, Valerie Brandt, Brian D. Berman, Marie Vidailhet, Emmanuel Roze, Anne Weissbach, Cynthia Comella, Irene A. Malaty, Joseph Jankovic, Mark S. LeDoux, Alfredo Berardelli, Richard Barbano, Stephen G. Reich, Joel S. Perlmutter, H.A. Jinnah, Norbert Brüggemann. Neurology, Volume 91, Issue 21, 20 November 2018, Pages e2020-e2026 Read More

Abstract

OBJECTIVE: To determine predictors of alcohol responsiveness in a large cohort of patients with dystonia. METHODS: A total of 2,159 participants with dystonia were prospectively enrolled in the cross-sectional Dystonia Coalition multicenter study. Patients with secondary, combined, or confirmed genetic dystonia (total n = 164) or unknown alcohol responsiveness (n = 737) were excluded. Patients answered a standardized questionnaire and were clinically examined using a standardized video protocol and the Burke-Fahn-Marsden Dystonia Rating Scale. Alcohol responsiveness was determined by patients’ self-report. RESULTS: A total of 1,258 patients with isolated dystonia (mean age: 59.5 ± 12.2 years; 898 women) met the inclusion criteria; 369 patients (29.3%) reported improvement of dystonia after alcohol consumption. Alcohol responsiveness was not related to sex (p = 0.742), age (p = 0.715), or severity of dystonia (p = 0.623). Age at onset was lower in patients who responded to alcohol (p < 0.001). Alcohol responsiveness differed across dystonia subgroups (multifocal/generalized > segmental [p = 0.014]; cervical and laryngeal > cranial and limb [p < 0.001]) and was related to a positive family history of movement disorders (p = 0.001), and presence of tremor (p < 0.001). CONCLUSION: The association of alcohol responsiveness with a positive family history for movement disorders, generalized dystonia, and an earlier age at onset suggests that patients with dystonia who have an underlying genetic contribution may be more likely to respond beneficially to alcohol. The fact that dystonic tremor may respond to alcohol is in keeping with the observation that the intake of GABAergic drugs may have a beneficial effect in a proportion of patients. © 2018 American Academy of Neurology.

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Posted on November 30, 2018
Posted in: HPAN, Neurodegeneration, Publications Authors: