Spinocerebellar Ataxia Type 10 (SCA10)

Record ID 32010000873
Authors' objectives:

The spinocerebellar ataxias (SCA) are a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by limb and gait ataxia. More than 25 different types of SCA have been described, although the causative genes have not yet been identified for all types. To date, SCA type 10 (SCA10) has only been found in populations of Amerindian origin (specifically, Mexican, Brazilian, and Argentinean). It is often a predominantly cerebellar syndrome, with prominent limb and gait ataxia, eye movement disorders, and dysarthria (slow, distorted speech). However, a subset of SCA10 patients, particularly those of Mexican descent, also experience epilepsy with generalized motor and/or partial complex seizures. Additional features of SCA10 include hyperreflexia, spasticity, peripheral neuropathy, cognitive impairment, and mood disturbances. The onset of symptoms in SCA10 patients is variable, but tends to occur in the second to fifth decade. SCA10 is caused by extreme expansions of a pentanucleatide repeat, ATTCT, in intron 9 of the ataxin-10 gene (ATXN10) located on chromosome 22 at band q13. The repeat tract in SCA10 patients may contain pure stretches of ATTCT pentanucleotides, or it may be interrupted with other penta- or septa-nucleotide sequences.

Project Status: Completed
URL for project: http://www.hayesinc.com/
Year Published: 2009
English language abstract: An English language summary is available
Publication Type: Not Assigned
Country: United States
MeSH Terms
  • Humans
  • Spinocerebellar Ataxias
Organisation Name: HAYES, Inc.
Contact Address: 157 S. Broad Street, Suite 200, Lansdale, PA 19446, USA. Tel: 215 855 0615; Fax: 215 855 5218
Contact Name: saleinfo@hayesinc.com
Contact Email: saleinfo@hayesinc.com
Copyright: 2009 Winifred S. Hayes, Inc
This is a bibliographic record of a published health technology assessment from a member of INAHTA or other HTA producer. No evaluation of the quality of this assessment has been made for the HTA database.