Alcohol-sensitive hereditary essential myoclonus with dystonia: a study of 6 Brazilian patients
In: Neurological Sciences; Neurological Sciences. New York: Springer-verlag, v. 21, n. 6, 2000, S. 373-377
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Zugriff:
We present the clinical profile of a group of patients with myoclonus and dystonia sensitive to alcohol and address these cases in the context of essential myoclonus. Six patients from 4 families were selected: 4 men and 2 women with myoclonus affecting predominantly the arms. Active movements of these segments elicited the dystonic and myoclonic movements. A marked improvement with alcohol intake was seen. Laboratory findings including EEG, SSEP, and cranial CT and MRI were normal. Surface EMG recording showed bursts with duration of 30-112 ms in 3 patients. One patient showed a triphasic recording pattern (agonist-antagonist-agonist) of ballistic type. Our findings suggest that the myoclonus-dystonia disorder is present in Brazilian patients. ; Universidade Federal de São Paulo, Dept Neurol, Movement Disorders Unit, São Paulo, Brazil ; Universidade Federal de São Paulo, Dept Neurol, Movement Disorders Unit, São Paulo, Brazil ; Web of Science
| Titel: |
Alcohol-sensitive hereditary essential myoclonus with dystonia: a study of 6 Brazilian patients
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| Autor/in / Beteiligte Person: | Borges, V ; Ferraz, H. B. ; Andrade, LAF de ; Universidade Federal de São Paulo (UNIFESP) |
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| Zeitschrift: | Neurological Sciences; Neurological Sciences. New York: Springer-verlag, v. 21, n. 6, 2000, S. 373-377 |
| Veröffentlichung: | Springer, 2000 |
| Medientyp: | academicJournal |
| ISSN: | 1590-1874 (print) |
| DOI: | 10.1007/s100720070053 |
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