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Neurostimulation of the ventral intermediate thalamic nucleus alleviates hereditary essential myoclonus
  1. ANDREAS KUPSCH,
  2. THOMAS TROTTENBERG,
  3. WASSILIOS MEISSNER
  1. Department of Neurology, Humboldt-University, Charité-Virchow, Augustenburger Platz 1, 13353 Berlin, Germany
  2. Department of Neurosurgery, Free University of Berlin, Hindenburgdamm 30, 12200 Berlin, Germany
  1. PD Dr Andreas Kupsch, Department of Neurology, Charité, Campus Virchow, Humboldt-University, Augustenburger Platz 1, 13353 Berlin, Germany. Telephone 0049 30 450 60103; fax 0049 30 450 60901; emailandreas.kupsch{at}charite.de
  1. THOMAS FUNK
  1. Department of Neurology, Humboldt-University, Charité-Virchow, Augustenburger Platz 1, 13353 Berlin, Germany
  2. Department of Neurosurgery, Free University of Berlin, Hindenburgdamm 30, 12200 Berlin, Germany
  1. PD Dr Andreas Kupsch, Department of Neurology, Charité, Campus Virchow, Humboldt-University, Augustenburger Platz 1, 13353 Berlin, Germany. Telephone 0049 30 450 60103; fax 0049 30 450 60901; emailandreas.kupsch{at}charite.de

https://doi.org/10.1136/jnnp.67.3.415

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    Therapy in hereditary essential myoclonus (HEM), a disabling movement disorder, is difficult in most cases, especially in regard to the myoclonic syndrome.1 This is the first report on the amelioration (around 65%) of HEM by high frequency deep brain stimulation of the ventral intermediate thalamic nucleus (VIM) in a 61 year old male patient with medically intractable HEM.

    The jerky movement disorder began in his neck and shoulders around the age of 6 and gradually progressed, especially in the proximal right arm, leading to a severe reduction of dexterity and inability to write. He was unable to continue working as a janitor when he was 55.

    On examination he presented mainly oscillating, “lightning”, irregular, jerky, asynchronous movements of his forehead, neck, proximal right arm, less severe of the left arm, and discreetly of the upper trunk, which was exaggerated by walking, writing or drinking and could be elicited by loud acoustic stimuli. The disorder …

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