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Neuroacanthocytosis Syndromes II pp 255–269Cite as

Neurosurgery for Neuroacanthocytosis

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Neuroacanthocytosis (NA) is a group of neurodegenerative diseases characterized by the presence of acanthocytes in peripheral blood and various types of involuntary movements which are often resistant to medical treatment. We report data concerning four NA patients who benefited from functional surgery; two had pallidotomies and two had deep brain stimulation (DBS) of the internal pallidum (GPi). The clinical outcome of pallidotomy was partial in the first patient, but clearly improved involuntary movements in the second patient without inducing side effects. In the patients who underwent DBS, GPi 40 Hz stimulation gave the best clinical benefit, improving chorea without an effect upon hypotonia. Higher frequency stimulation (130 Hz) was effective for dystonia but increased chorea, worsened dysarthria and induced drooling. Low frequency (10 Hz) GPi stimulation was ineffective. Therefore, in this small series of NA patients, both pallidotomy and pallidal stimulation were partially effective for relieving involuntary movements. However, in this progressive disease with a wide spectrum of symptoms, the decision to perform surgery must involve a full assessment of the clinical features of each patient.

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Author information

Authors and Affiliations

  1. Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fiuchru, Tokyo, Japan

    F. Yokochi

  2. Department of Clinical Neurophysiology, Centre Hospitalier Pellegrin, Place Amélie-Raba Léon, 33076, Bordeaux, France

    P. Burbaud

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  1. F. Yokochi
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  2. P. Burbaud
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Editor information

Editors and Affiliations

  1. Movement Disorders Clinic Department of Neurology, James J. Peters Veterans Affairs Medical Center, 130 W. Kingsbridge Road (127), Bronx, NY 10468

    Ruth H. Walker

  2. Department of Neurology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA

    Ruth H. Walker

  3. Department of Medical Genetics Cambridge Institute for Medical Research Wellcome Trust/MRC Building, Addenbrooke’s Hospital, Hills Road, Cambridge, CB2 2XY, UK

    Shinji Saiki

  4. Neurologische Klinik, Ludwig-Maximilians-Universität, D-81366, München, Germany

    Adrian Danek

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Yokochi, F., Burbaud, P. (2008). Neurosurgery for Neuroacanthocytosis. In: Walker, R.H., Saiki, S., Danek, A. (eds) Neuroacanthocytosis Syndromes II. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-71693-8_22

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