Predictors of alcohol responsiveness in dystonia

Johanna Junker¹, Valerie Brandt¹, Brian D Berman¹, Marie Vidailhet¹, Emmanuel Roze¹, Anne Weissbach¹, Cynthia Comella¹, Irene A Malaty¹, Joseph Jankovic¹, Mark S LeDoux¹, Alfredo Berardelli¹, Richard Barbano¹, Stephen G Reich¹, Joel S Perlmutter¹, H A Jinnah¹, Norbert Brüggemann²

Affiliations

¹ From the Institute of Neurogenetics (J. Junker, V.B., A.W., N.B.) and Department of Neurology (J. Junker, N.B.), University of Luebeck, Germany; Department of Psychology (V.B.), Centre for Innovation in Mental Health, University of Southampton, UK; Department of Neurology (B.D.B.), University of Colorado Anschutz Medical Campus, Aurora; Neurology Section (B.D.B.), Denver VA Medical Center, CO; Département de Neurologie (M.V., E.R.), Hôpital Pitié-Salpêtrière, Assistance Publique-Hopitaux de Paris; Sorbonne Universités (M.V.), Pierre Marie Curie Paris-6, Institute of Brain and Spine (ICM), Inserm U 1127, Paris, France; Department of Neurology (C.C.), Rush University Medical Center, Chicago, IL; Department of Neurology (I.A.M.), Center for Movement Disorders and Neurorestoration, University of Florida, Gainesville; Department of Neurology (J. Jankovic), Baylor College of Medicine, Houston, TX; Department of Neurology (M.S.L.), University of Tennessee Health Science Center, Memphis; Neuromed Institute (IRCCS) (A.B.), Pozzilli (IS), and Department of Neurology and Psychiatry, Sapienza University di Roma, Italy; Department of Neurology (R.B.), University of Rochester Medical Center, NY; Department of Neurology (S.G.R.), University of Maryland Medical Center, Baltimore; Department of Neurology (J.S.P.), Washington University in St. Louis, MO; and Department of Neurology and Human Genetics (H.A.J.), Emory University, Atlanta, GA.
² From the Institute of Neurogenetics (J. Junker, V.B., A.W., N.B.) and Department of Neurology (J. Junker, N.B.), University of Luebeck, Germany; Department of Psychology (V.B.), Centre for Innovation in Mental Health, University of Southampton, UK; Department of Neurology (B.D.B.), University of Colorado Anschutz Medical Campus, Aurora; Neurology Section (B.D.B.), Denver VA Medical Center, CO; Département de Neurologie (M.V., E.R.), Hôpital Pitié-Salpêtrière, Assistance Publique-Hopitaux de Paris; Sorbonne Universités (M.V.), Pierre Marie Curie Paris-6, Institute of Brain and Spine (ICM), Inserm U 1127, Paris, France; Department of Neurology (C.C.), Rush University Medical Center, Chicago, IL; Department of Neurology (I.A.M.), Center for Movement Disorders and Neurorestoration, University of Florida, Gainesville; Department of Neurology (J. Jankovic), Baylor College of Medicine, Houston, TX; Department of Neurology (M.S.L.), University of Tennessee Health Science Center, Memphis; Neuromed Institute (IRCCS) (A.B.), Pozzilli (IS), and Department of Neurology and Psychiatry, Sapienza University di Roma, Italy; Department of Neurology (R.B.), University of Rochester Medical Center, NY; Department of Neurology (S.G.R.), University of Maryland Medical Center, Baltimore; Department of Neurology (J.S.P.), Washington University in St. Louis, MO; and Department of Neurology and Human Genetics (H.A.J.), Emory University, Atlanta, GA. norbert.brueggemann@neuro.uni-luebeck.de.

PMID: 30341158
PMCID: PMC6260199
DOI: 10.1212/WNL.0000000000006551

Multicenter Study

Predictors of alcohol responsiveness in dystonia

Johanna Junker et al. Neurology. 2018.

. 2018 Nov 20;91(21):e2020-e2026.

doi: 10.1212/WNL.0000000000006551. Epub 2018 Oct 19.

Authors

Affiliations

¹ From the Institute of Neurogenetics (J. Junker, V.B., A.W., N.B.) and Department of Neurology (J. Junker, N.B.), University of Luebeck, Germany; Department of Psychology (V.B.), Centre for Innovation in Mental Health, University of Southampton, UK; Department of Neurology (B.D.B.), University of Colorado Anschutz Medical Campus, Aurora; Neurology Section (B.D.B.), Denver VA Medical Center, CO; Département de Neurologie (M.V., E.R.), Hôpital Pitié-Salpêtrière, Assistance Publique-Hopitaux de Paris; Sorbonne Universités (M.V.), Pierre Marie Curie Paris-6, Institute of Brain and Spine (ICM), Inserm U 1127, Paris, France; Department of Neurology (C.C.), Rush University Medical Center, Chicago, IL; Department of Neurology (I.A.M.), Center for Movement Disorders and Neurorestoration, University of Florida, Gainesville; Department of Neurology (J. Jankovic), Baylor College of Medicine, Houston, TX; Department of Neurology (M.S.L.), University of Tennessee Health Science Center, Memphis; Neuromed Institute (IRCCS) (A.B.), Pozzilli (IS), and Department of Neurology and Psychiatry, Sapienza University di Roma, Italy; Department of Neurology (R.B.), University of Rochester Medical Center, NY; Department of Neurology (S.G.R.), University of Maryland Medical Center, Baltimore; Department of Neurology (J.S.P.), Washington University in St. Louis, MO; and Department of Neurology and Human Genetics (H.A.J.), Emory University, Atlanta, GA.
² From the Institute of Neurogenetics (J. Junker, V.B., A.W., N.B.) and Department of Neurology (J. Junker, N.B.), University of Luebeck, Germany; Department of Psychology (V.B.), Centre for Innovation in Mental Health, University of Southampton, UK; Department of Neurology (B.D.B.), University of Colorado Anschutz Medical Campus, Aurora; Neurology Section (B.D.B.), Denver VA Medical Center, CO; Département de Neurologie (M.V., E.R.), Hôpital Pitié-Salpêtrière, Assistance Publique-Hopitaux de Paris; Sorbonne Universités (M.V.), Pierre Marie Curie Paris-6, Institute of Brain and Spine (ICM), Inserm U 1127, Paris, France; Department of Neurology (C.C.), Rush University Medical Center, Chicago, IL; Department of Neurology (I.A.M.), Center for Movement Disorders and Neurorestoration, University of Florida, Gainesville; Department of Neurology (J. Jankovic), Baylor College of Medicine, Houston, TX; Department of Neurology (M.S.L.), University of Tennessee Health Science Center, Memphis; Neuromed Institute (IRCCS) (A.B.), Pozzilli (IS), and Department of Neurology and Psychiatry, Sapienza University di Roma, Italy; Department of Neurology (R.B.), University of Rochester Medical Center, NY; Department of Neurology (S.G.R.), University of Maryland Medical Center, Baltimore; Department of Neurology (J.S.P.), Washington University in St. Louis, MO; and Department of Neurology and Human Genetics (H.A.J.), Emory University, Atlanta, GA. norbert.brueggemann@neuro.uni-luebeck.de.

PMID: 30341158
PMCID: PMC6260199
DOI: 10.1212/WNL.0000000000006551

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.

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Figures

**Figure. Degree of alcohol responsiveness across different dystonia groups**
Patients with multifocal/generalized dystonia had a greater likelihood of response than patients with segmental dystonia (A). Among subgroups of focal dystonia, patients with either cervical or laryngeal dystonia had a greater likelihood of response than patients with limb or cranial dystonia (B). Among the subgroups of cranial dystonia, patients with oromandibular/lingual dystonia responded more frequently than patients with blepharospasm (C). *Bonferroni-corrected p: (A) p ≤ 0.017, (B) p ≤ 0.008, (C) p ≤ 0.017; ***p < 0.001.

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References

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Predictors of alcohol responsiveness in dystonia

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Predictors of alcohol responsiveness in dystonia

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