Dentatorubral Pallidoluysian Atrophy

Common Name(s)

Dentatorubral Pallidoluysian Atrophy

Dentatorubral-pallidoluysian atrophy (DRPLA) is a progressive brain disorder that causes involuntary movements; mental and emotional problems; and a decline in thinking ability. The average age of onset of DRPLA is 30 years, but the condition can appear anytime from infancy to mid-adulthood. Specific signs and symptoms may differ among affected individuals and sometimes affects children and adults differently. DRPLA is caused by a mutation in the ATN1 gene and is inherited in an autosomal dominant manner. Treatment is symptomatic and supportive.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Dentatorubral Pallidoluysian Atrophy" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Dentatorubral Pallidoluysian Atrophy" returned 19 free, full-text research articles on human participants. First 3 results:

Radiologic and neuropathologic findings in patients in a family with dentatorubral-pallidoluysian atrophy.
 

Author(s): Y Sunami, R Koide, N Arai, M Yamada, T Mizutani, K Oyanagi

Journal: AJNR Am J Neuroradiol. 2011 Jan;32(1):109-14.

 

We describe the cases of 2 patients, a father and his son, with DRPLA who underwent MR examinations prior to death and in whom postmortem examinations of the brain were obtained. MR imaging findings had the following features: 1) atrophy of the cerebellum and brain stem were the common ...

Last Updated: 14 Jan 2011

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Dentatorubral pallidoluysian atrophy in a Turkish family.
 

Author(s): Uluç Yiş, Eray Dirik, Asli Gündoğdu-Eken, A Nazli Başak

Journal: Turk. J. Pediatr.. ;51(6):610-2.

 

Dentatorubral pallidoluysian atrophy is a neurodegenerative disease that generally presents in adulthood. Although rare, it can be observed in childhood due to extreme expansion of the triplet repeat size during spermatogenesis. The diagnosis in childhood is very difficult in the ...

Last Updated: 3 Mar 2010

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Global and region-specific analyses of apparent diffusion coefficient in dentatorubral-pallidoluysian atrophy.
 

Author(s): T Kin, M Hirano, T Taoka, Y Furiya, H Kataoka, K Kichikawa, S Ueno

Journal: AJNR Am J Neuroradiol. 2006 Aug;27(7):1463-6.

 

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant spinocerebellar ataxia. Techniques for the quantitative assessment of neurodegenerative lesions remain to be established in this disease. We attempted to quantify global and region-specific neurodegeneration in ...

Last Updated: 15 Aug 2006

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Dentatorubral Pallidoluysian Atrophy" returned 1 free, full-text review articles on human participants. First 3 results:

Molecular pathology of dentatorubral-pallidoluysian atrophy.
 

Author(s): I Kanazawa

Journal: Philos. Trans. R. Soc. Lond., B, Biol. Sci.. 1999 Jun;354(1386):1069-74.

 

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant disorder characterized clinically by myoclonus, epilepsy, cerebellar ataxia, choreoathetosis and dementia. Cardinal pathological features of DRPLA are a combined degeneration of both the dentatorubral and the pallidoluysian ...

Last Updated: 26 Aug 1999

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