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The autosomal dominant heredoataxias are separated into spinocerebellar ataxias (SCA1-8, 10-15, 17-23, 25-30, and dentato-rubro-pallido-luysian atrophy), episodic ataxias (EA1-7), and autosomal dominant mitochondrial heredoataxias (Leigh syndrome, MIRAS, ADOAD, and AD-CPEO). The autosomal recessive ataxias are separated into Friedreich ataxia, ataxia due to vitamin E deficiency, ataxia due to Abeta-lipoproteinemia, Refsum disease, late-onset Tay-Sachs disease, cerebrotendineous xanthomatosis, spinocerebellar ataxia with axonal neuropathy, ataxia telangiectasia, ataxia telangiectasia-like disorder, ataxia with oculomotor apraxia 1 and 2, spastic ataxia of Charlevoix-Saguenay, Cayman ataxia, Marinesco-Sj\u00f6gren syndrome, and autosomal recessive mitochondrial ataxias (AR-CPEO, SANDO, SCAE, AHS, IOSCA, MEMSA, LBSL CoQ-deficiency, PDC-deficiency). Only two of the heredoataxias, fragile X\/tremor\/ataxia syndrome, and XLSA\/A are transmitted via an X-linked trait. Maternally inherited heredoataxias are due to point mutations in genes encoding for tRNAs, rRNAs, respiratory chain subunits or single large scale deletions\/duplications of the mitochondrial DNA and include MELAS, MERRF, KSS, PS, MILS, NARP, and non-syndromic mitochondrial disorders. Treatment of heredoataxias is symptomatic and supportive and may have a beneficial effect in single patients.<\/jats:p><jats:p>**Please see page 424 for abbreviation list.<\/jats:p>","DOI":"10.1017\/s0317167100007733","type":"journal-article","created":{"date-parts":[[2014,12,12]],"date-time":"2014-12-12T10:13:14Z","timestamp":1418379194000},"page":"409-428","source":"Crossref","is-referenced-by-count":32,"title":["Ataxias with Autosomal, X-Chromosomal or Maternal Inheritance"],"prefix":"10.1017","volume":"36","author":[{"given":"Josef","family":"Finsterer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2014,12,2]]},"reference":[{"key":"S0317167100007733_ref023","first-page":"393","article-title":"Therapeutic strategies for the polyglutamine diseases","volume":"59","author":"Nagai","year":"2007","journal-title":"Brain 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