ABSTRACT

OBJECTIVE:

Essential myoclonus is a rare and benign, frequently hereditary disorder although it may appear sporadically.

METHODS:

In this clinical picture, myoclonus is the only neurological sign, meanwhile a distonic component may accompany. Myoclonus affecting predominantly the arms and neck, occurs spontaneously or with action.

RESULTS: In this study a 45 year-old woman diagnosed as essential myoclonic dystonia was presented.

CONCLUSION:

Her complaints had started when she was 1,5 years old, and the diagnosis was made when she was 38 years old after long term follow-up and detailed electrophysiological examinations.