Choreoacanthocytosis

Chorea-acanthocytosis (ChAc) is a form of neuroacanthocytosis (see this term) and is characterized clinically by a Huntington disease-like phenotype with progressive neurological symptoms including movement disorders, psychiatric manifestations and cognitive disturbances.

Dystonia

An abnormally increased muscular tone that causes fixed abnormal postures. There is a slow, intermittent twisting motion that leads to exaggerated turning and posture of the extremities and trunk.


Total: 7

                       


(per page)
PMID (PMCID)
31191273
 OTHER
Cortico-subthalamic Coherence in a Patient With Dystonia Induced by Chorea-Acanthocytosis: A Case Report.
Cao C, Huang P, Wang T, Zhan S, Liu W, Pan Y, Wu Y, Li H, Sun B, Li D, Litvak V.
Front Hum Neurosci. 2019;13:163.
Here we report the results of simultaneous local field potential (LFP) recordings and magnetoencephalography (MEG) in a single patient who was implanted bilaterally in the STN for the treatment of dystonia induced by chorea-acanthocytosis.
31191273
 OTHER
Cortico-subthalamic Coherence in a Patient With Dystonia Induced by Chorea-Acanthocytosis: A Case Report.
Cao C, Huang P, Wang T, Zhan S, Liu W, Pan Y, Wu Y, Li H, Sun B, Li D, Litvak V.
Front Hum Neurosci. 2019;13:163.
Cortico-subthalamic Coherence in a Patient With Dystonia Induced by Chorea-Acanthocytosis: A Case Report.
24974674
 MIXED_SAMPLE Adult
Chorea-acanthocytosis presenting as dystonia.
Kobal J, Dobson-Stone C, Danek A, Fidler V, Zvan B, Zaletel M.
Acta Clin Croat. 2014;53(1):107-12.
Chorea-acanthocytosis presenting as dystonia.
21863267
 MALE Adult
Deep brain stimulation of the globus pallidus internal improves symptoms of chorea-acanthocytosis.
Li P, Huang R, Song W, Ji J, Burgunder JM, Wang X, Zhong Q, Kaelin-Lang A, Wang W, Shang HF.
Neurol Sci. 2012;33(2):269-74.
Bilateral DBS to the GPi can improve chorea and dystonia in some patients with intractable chorea-acanthocytosis.
21714011
 MALE Middle Aged
Feeding dystonia in McLeod syndrome.
Gantenbein AR, Damon-Perriere N, Bohlender JE, Chauveau M, Latxague C, Miranda M, Jung HH, Tison F.
Mov Disord. 2011;26(11):2123-6.
Another neuroacanthocytosis syndrome, the autosomal recessive chorea-acanthocytosis, has a similar presentation, but distinct clinical features, believed to be characteristic, such as tongue protrusion dystonia, feeding dystonia, and rubber-man-like appearance.
21714011
 MALE Middle Aged
Feeding dystonia in McLeod syndrome.
Gantenbein AR, Damon-Perriere N, Bohlender JE, Chauveau M, Latxague C, Miranda M, Jung HH, Tison F.
Mov Disord. 2011;26(11):2123-6.
Another neuroacanthocytosis syndrome, the autosomal recessive chorea-acanthocytosis, has a similar presentation, but distinct clinical features, believed to be characteristic, such as tongue protrusion dystonia, feeding dystonia, and rubber-man-like appearance.
20544815
 OTHER Adult
Characteristic head drops and axial extension in advanced chorea-acanthocytosis.
Schneider SA, Lang AE, Moro E, Bader B, Danek A, Bhatia KP.
Mov Disord. 2010;25(10):1487-91.
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.