Alcohol improves cerebellar learning deficit in myoclonus-dystonia: A clinical and electrophysiological investigation
| Autor: | Vera Tadic, Christine Klein, Elisa M Werner, Alexander Münchau, Tobias Bäumer, Dagmar Timmann, Sinem Tunc, Julien F. Bally, Sebastian Löns, Norbert Brüggemann, Anthony E. Lang, Anne Weissbach, Kirsten E. Zeuner |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Dystonia Gene mutation medicine.disease 3. Good health Associative learning 03 medical and health sciences 030104 developmental biology 0302 clinical medicine Neurology Eyeblink conditioning Anesthesia medicine Neurology (clinical) Analysis of variance Corneal reflex medicine.symptom Psychology Myoclonus 030217 neurology & neurosurgery Dystonic disorder |
| Zdroj: | Annals of Neurology. 82:543-553 |
| ISSN: | 0364-5134 |
| Popis: | Objectives: To characterize neurophysiological subcortical abnormalities in myoclonus-dystonia and their modulation by alcohol administration. Methods: Cerebellar associative learning and basal ganglia-brainstem interaction were investigated in 17 myoclonus-dystonia patients with epsilon-sarcoglycan (SGCE) gene mutation and 21 age- and sex-matched healthy controls by means of classical eyeblink conditioning and blink reflex recovery cycle before and after alcohol intake resulting in a breath alcohol concentration of 0.08% (0.8 g/l). The alcohol responsiveness of clinical symptoms was evaluated by three blinded raters with a standardized video protocol and clinical rating scales including the Unified Myoclonus Rating Scale (UMRS) and the Burke Fahn Marsden Dystonia Rating Scale (BFMDRS). Results: Patients showed a significantly reduced number of conditioned eyeblink responses before alcohol administration compared to controls. While the conditioning response rate decreased under alcohol intake in controls, it increased in patients (ANOVA: alcohol state x Group p = 0.004). Blink reflex recovery cycle before and after alcohol intake did not differ between groups. Myoclonus improved significantly after alcohol intake (p = 0.016). The severity of action myoclonus at baseline correlated negatively with the conditioning response in classical eyeblink conditioning in patients. Interpretation: The combination of findings with reduced baseline acquisition of conditioned eyeblink responses and normal blink reflex recovery cycle in patients that improved significantly by alcohol intake suggests a crucial role of cerebellar networks in the generation of symptoms in these patients. This article is protected by copyright. All rights reserved. |
| Databáze: | OpenAIRE |
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