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Treatable Ataxias: How to Find the Needle in the Haystack?

Stezin, A and Pal, PK (2022) Treatable Ataxias: How to Find the Needle in the Haystack? In: Journal of Movement Disorders, 15 (3). pp. 206-226.

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Official URL: https://doi.org/10.14802/jmd.22069


Treatable ataxias are a group of ataxic disorders with specific treatments. These disorders include genetic and metabolic disor-ders, 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 atax-ias. 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 defi-ciency, 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 multi-focal leukoencephalopathy. These disorders are treated with steroids and antibiotics. Recognizing treatable disorders is of para-mount importance when dealing with ataxias given that early treatment can prevent permanent neurological sequelae.

Item Type: Journal Article
Publication: Journal of Movement Disorders
Publisher: Korean Movement Disorder Society
Additional Information: The copyright for this article belongs to the Authors.
Keywords: aaAcquired ataxia; Cerebellar ataxia; Genetic ataxia; Movement disorders; Treatable ataxia
Department/Centre: Autonomous Societies / Centres > Centre for Brain Research
Date Deposited: 13 Oct 2022 06:13
Last Modified: 13 Oct 2022 06:13
URI: https://eprints.iisc.ac.in/id/eprint/77292

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