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neuroacanthocytosis/spasm

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Improvement of severe trunk spasms by bilateral high-frequency stimulation of the motor thalamus in a patient with chorea-acanthocytosis.

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We report on a patient with a severe form of chorea-acanthocytosis, intractable to medical treatment, who benefited from bilateral high-frequency stimulation (HFS) of the posterior ventral oral nucleus of the thalamus. The frequency of trunk spasms dramatically decreased after surgery and the

Improvement of severe trunk spasms by bilateral high-frequency stimulation of the motor thalamus in a patient with chorea-acanthocytosis.

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Successful Combination of Pallidal and Thalamic Stimulation for Intractable Involuntary Movements in Patients with Neuroacanthocytosis.

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OBJECTIVE Neuroacanthocytosis (NA) is a rare neurodegenerative disease that involves severe involuntary movements including chorea, dystonia, and trunk spasms. Current treatments are not effective for these involuntary movements. Although there are a few reports on the use of deep brain stimulation

Minimal tissue damage after stimulation of the motor thalamus in a case of chorea-acanthocytosis.

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Autopsy findings are reported from a patient with chorea-acanthocytosis treated for 2 years by deep brain stimulation (DBS) of the motor thalamus. Postoperative testing showed a progressive improvement in axial truncal spasms. Although relatively high currents were used for 2 years in this patient,

Globus pallidus interna deep brain stimulation improves chorea and functional status in a patient with chorea-acanthocytosis.

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We report a 39-year-old woman with chorea-acanthocytosis (ChAc) who was referred with refractory hyperkinetic movement and truncal bending spasm. She was diagnosed with ChAc with clinical features and laboratory findings of acanthocytosis in peripheral blood smear, and genetic studies revealed novel

Characteristic head drops and axial extension in advanced chorea-acanthocytosis.

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Chorea-acanthocytosis is a rare autosomal recessive neurodegenerative disorder with a complex clinical presentation comprising of a mixed movement disorder (mostly chorea and dystonia), seizures, neuropathy and myopathy, autonomic features as well as dementia and psychiatric features. Because the

Chorea-acanthocytosis: report of three cases from Iran.

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Chorea-acanthocythosis (ChAc) is an inherited neurodegenerative disorder characterized by movement disorders, neuropsychiatric disturbances, neuropathy, myopathy, seizures and acanthocytosis accompanied by an elevated serum creatine kinase (CK) level. Its causative gene (VPS13A) produces chorein
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