ISSN:
1590-3478
Keywords:
Key words Myoclonus-dystonia
;
Essential myoclonus
;
Dystonia
;
Alcohol
;
Genetics
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract 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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s100720070053