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HOME > J Mov Disord > Volume 15(3); 2022 > Article
Review Article Treatable Ataxias: How to Find the Needle in the Haystack?
Albert Stezin1,2orcid , Pramod Kumar Pal1orcid
Journal of Movement Disorders 2022;15(3):206-226
DOI: https://doi.org/10.14802/jmd.22069
Published online: September 7, 2022
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1Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, India
2Centre for Brain Research, Indian Institute of Science, Bengaluru, India
Corresponding author:  Pramod Kumar Pal, Tel: +91-80-26995147, Fax: +91-80-26564830, 
Email: palpramod@hotmail.com
Received: 7 March 2022   • Revised: 2 July 2022   • Accepted: 5 July 2022

Treatable ataxias are a group of ataxic disorders with specific treatments. These disorders include genetic and metabolic disorders, immune-mediated ataxic disorders, and ataxic disorders associated with infectious and parainfectious etiology, vascular causes, toxins and chemicals, and endocrinopathies. This review provides a comprehensive overview of different treatable ataxias. The major metabolic and genetic treatable ataxic disorders include ataxia with vitamin E deficiency, abetalipoproteinemia, cerebrotendinous xanthomatosis, Niemann-Pick disease type C, autosomal recessive cerebellar ataxia due to coenzyme Q10 deficiency, glucose transporter type 1 deficiency, and episodic ataxia type 2. The treatment of these disorders includes the replacement of deficient cofactors and vitamins, dietary modifications, and other specific treatments. Treatable ataxias with immune-mediated etiologies include gluten ataxia, anti-glutamic acid decarboxylase antibody-associated ataxia, steroid-responsive encephalopathy associated with autoimmune thyroiditis, Miller-Fisher syndrome, multiple sclerosis, and paraneoplastic cerebellar degeneration. Although dietary modification with a gluten-free diet is adequate in gluten ataxia, other autoimmune ataxias are managed by short-course steroids, plasma exchange, or immunomodulation. For autoimmune ataxias secondary to malignancy, treatment of tumor can reduce ataxic symptoms. Chronic alcohol consumption, antiepileptics, anticancer drugs, exposure to insecticides, heavy metals, and recreational drugs are potentially avoidable and treatable causes of ataxia. Infective and parainfectious causes of cerebellar ataxias include acute cerebellitis, postinfectious ataxia, Whipple’s disease, meningoencephalitis, and progressive multifocal leukoencephalopathy. These disorders are treated with steroids and antibiotics. Recognizing treatable disorders is of paramount importance when dealing with ataxias given that early treatment can prevent permanent neurological sequelae.

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