title: Current concepts in the treatment of hereditary ataxias.
abstract: Hereditary ataxias (HA) represents an extensive group of clinically and genetically heterogeneous neurodegenerative diseases, characterized by progressive ataxia combined with extra-cerebellar and multi-systemic involvements, including peripheral neuropathy, pyramidal signs, movement disorders, seizures, and cognitive dysfunction. There is no effective treatment for HA, and management remains supportive and symptomatic. In this review, we will focus on the symptomatic treatment of the main autosomal recessive ataxias, autosomal dominant ataxias, X-linked cerebellar ataxias and mitochondrial ataxias. We describe management for different clinical symptoms, mechanism-based approaches, rehabilitation therapy, disease modifying therapy, future clinical trials and perspectives, genetic counseling and preimplantation genetic diagnosis.
authors.affiliation: Center of Health Sciences, Universidade Estadual do Ceará, Fortaleza, CE, Brazil.
authors.forename: Pedro
authors.initials: P
authors.lastname: Braga Neto
authors.affiliation: Departmento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
authors.forename: José Luiz
authors.initials: JL
authors.lastname: Pedroso
authors.affiliation: Department of Neurology, Columbia University, New York, NY, United States.
authors.forename: Sheng-Han
authors.initials: SH
authors.lastname: Kuo
authors.affiliation: Departmento de Neurologia, Universidade Estadual de Campinas, Campinas, SP, Brazil.
authors.forename: C França
authors.initials: CF
authors.lastname: Marcondes Junior
authors.affiliation: Hospital de Clinicas, Serviço de Neurologia, Universidade Federal do Paraná, Curitiba, PR, Brazil.
authors.forename: Hélio Afonso Ghizoni
authors.initials: HA
authors.lastname: Teive
authors.affiliation: Departmento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
authors.forename: Orlando Graziani Povoas
authors.initials: OG
authors.lastname: Barsottini
mesh_terms: D005817:Genetic Counseling; D006801:Humans; D013132:Spinocerebellar Degenerations / Q000145:classification / Q000235:genetics / Q000628:therapy*