A Case of Myoclonus–Dystonia Responding to Low-frequency Pallidal Stimulation

Harini Sarva,Andres Deik,Joan Miravite,Brian H Kopell,William Lawrence Severt,Deborah Raymond,Matthew C Swan

doi:10.5334/tohm.359

Abstract

Background: High-frequency pallidal stimulation has been shown to improve various types of dystonia, including myoclonus-dystonia. Case Report: We report a case of epsilon sarcoglycan mutation-negative myoclonus–dystonia with response to low-frequency bilateral pallidal stimulation. Discussion: Low-frequency pallidal stimulation provides an effective means of treating various dystonias, regardless of genetic status, as in our case, as it provides increased programming options with fewer adverse effects.

Highlights

Myoclonus–dystonia (MD) is a genetically and phenotypically heterogenous condition, which presents with myoclonus of proximal muscles and dystonia, typically of the neck and arm.[1]
Several reports demonstrated improvement with high-frequency pallidal stimulation, we report a case of SGCE mutation-negative MD whose myoclonus and dystonia responded to low-frequency bilateral pallidal stimulation
Our patient had a reduction in her Burke– Fahn–Marsden Dystonia Rating Scale (BFMDS) by 96% with low-frequency, high pulse width pallidal stimulation, similar to two recent cases of MD that responded to 60 Hz pallidal stimulation.[13]

Summary

A Case of Myoclonus–Dystonia Responding to Low-frequency Pallidal Stimulation

Harini Sarva1*, Joan Miravite[2], Matthew C. Swan[2], Andres Deik[3], Deborah Raymond[2], William Lawrence Severt2 & Brian H. Kopell[4 1] Parkinson’s Disease and Movement Disorders Institute, Department of Neurology, Weill Cornell Medicine, New York, NY, USA, 2 Department of Neurology, Division of Movement Disorders, Icahn School of Medicine at Mount Sinai, Mount Sinai Beth Israel Medical Center, New York, NY, USA, 3 Parkinson Disease and Movement Disorders Center, Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA, 4 Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, Mount Sinai Medical Center, New York, NY, USA

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