P.012 Bilateral pallidal deep brain stimulation in a patient with chorea-acanthocytosis

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Globus Pallidus Interna Deep Brain Stimulation for Chorea-Acanthocytosis

Chorea-acanthocytosis (ChAc) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis. Pharmacotherapy for control of involuntary movements has generally been of limited benefit. Deep brain stimulation (DBS) has recently been used for treatment of some refractory cases of ChAc. We report here on the effect of bilateral high-frequency DBS of...

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Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis

BACKGROUND Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the shor...

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Pallidal Deep Brain Stimulation in the Treatment of Huntington’s Chorea

Despite the success of deep brain stimulation (DBS) in various movement disorders, its use in Huntington ́s Disease (HD) has been limited. So far, promising results of pallidal DBS have been reported in 7 patients with HD. We performed bilateral pallidal DBS in a 59 year old woman with HD since 12 years and severe motor symptoms. At the evaluation after 12 months the effect was deemed satisfacto...

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Intractable Blepharospasm Treated with Bilateral Pallidal Deep Brain Stimulation

BACKGROUND Blepharospasm can be present as an isolated dystonia or in conjunction with other forms of cranial dystonia, causing significant disability. CASE REPORT We report a case of a 69-year-old male with craniocervical dystonia, manifesting primarily as incapacitating blepharospasm refractory to medical treatments. He underwent bilateral globus pallidus (GP) deep brain stimulation (DBS) w...

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Eye movements in chorea-acanthocytosis.

PURPOSE To describe the eye movement abnormalities in patients with chorea-acanthocytosis (ChAc), a form of neuroacanthocytosis. This autosomal recessive, neurodegenerative disorder with aberrant erythrocyte morphology (acanthocytosis) is caused by mutations in the VPS13A gene. In contrast to Huntington's disease (for which ChAc has occasionally been mistaken), ocular involvement in ChAc has no...

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ژورنال

عنوان ژورنال: Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques

سال: 2019

ISSN: 0317-1671,2057-0155

DOI: 10.1017/cjn.2019.113