Guidelines for treatment of immune-mediated cerebellar ataxias

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Abstract

Immune-mediated cerebellar ataxias include gluten ataxia, paraneoplastic cerebellar degeneration, GAD antibody associated cerebellar ataxia, and Hashimoto’s encephalopathy. Despite the identification of an increasing number of immune-mediated cerebellar ataxias, there is no proposed standardized therapy. We evaluated the efficacies of immunotherapies in reported cases using a common scale of daily activity. The analysis highlighted the importance of removal of autoimmune triggering factors (e.g., gluten or cancer) and the need for immunotherapy evaluation (e.g., corticosteroids, intravenous immunoglobulin, immunosuppressants) and adaptation according to each subtype.

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Cerebellar ataxia with anti-glutamic acid decarboxylase antibodies: study of 14 patients.

Florent P Trouillas, Pilar Sáiz, N Fabien … (2001)

Antibodies to glutamic acid decarboxylase (GAD-Ab) are described in patients with insulin-dependent (type 1) diabetes mellitus (IDDM), in stiff-man syndrome, and, recently, in a few patients with cerebellar ataxia. To show a link between GAD-Ab and some patients with cerebellar ataxia and to clarify their clinical and immunologic profiles. Serum samples were selected from 9000 samples of 4 laboratories. The selection criterion was an immunohistochemical pattern compatible with GAD-Ab that was confirmed by radioimmunoassay. We identified 22 patients with stiff-man syndrome and 14 with cerebellar ataxia and GAD-Ab. Thirteen of the 14 patients with cerebellar ataxia and GAD-Ab were women, and 11 had late-onset IDDM. Patients did not have clinical or radiologic evidence of brainstem involvement. Ten patients had oligoclonal IgG bands in the cerebrospinal fluid, and intrathecal GAD-Ab synthesis was observed in 5 of the 6 patients studied. The level of GAD-Ab of these patients was similar to those with stiff-man syndrome and significantly higher than those with IDDM or with polyendocrine autoimmunity (P<.001). However, the GAD-Ab levels of 6 of the 9 patients with polyendocrine autoimmunity overlapped with those of patients with cerebellar ataxia. These results suggest a link between high level of GAD-Ab and some cases of cerebellar ataxia, particularly women with IDDM. If high serum levels of GAD-Ab are detected, the cerebrospinal fluid should be evaluated for the presence of oligoclonal IgG bands and intrathecal synthesis of GAD-Ab to further prove an autoimmune origin of the syndrome.

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Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone.

J Honnorat, Yvo Graus, F Keime-Guibert … (2000)

To evaluate the effect of a combination of immunoglobulins (IVIg), cyclophosphamide (CTX), and methylprednisolone (MP) on the clinical course of patients with paraneoplastic neurological syndrome (PNS) and antineuronal antibodies (Abs). Seventeen patients with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/SN) with anti-Hu Abs (n = 10) or cerebellar degeneration (PCD) with anti-Yo Abs (n = 7) received one to nine cycles (mean 3.5) of a combination of IVIg (0.5 g/kg/day from days 1 to 5), CTX (600 mg/m2 at day 1) and MP (1g/day from day 1 to 3). The Rankin scale (RS) was used to evaluate the response. A positive response was considered as either improvement or stabilisation in patients who were still ambulatory (RS or =4). Tolerance was good and no patient experienced grade 3/4 toxicity (World Health Organisation). Sixteen patients were evaluable for response. Of the seven patients with RS> or =4, none improved. Of the nine patients with RS< or =3, none improved but three (two SN and one PCD) stabilised for 4, 35, and 16 months. This study suggests that vigorous immunosuppressive treatment is not useful in severely disabled PNS patients with antineuronal Abs. In a minority of patients (mainly with SN) who are not severely disabled at the onset of treatment, a transient stabilisation is possible and deserves further evaluation.

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Dietary treatment of gluten ataxia.

R Grunewald, S. Sanders, Kirsty A Douglas … (2003)

Gluten ataxia is an immune mediated disease, part of the spectrum of gluten sensitivity, and accounts for up to 40% of cases of idiopathic sporadic ataxia. No systematic study of the effect of gluten-free diet on gluten ataxia has ever been undertaken. To study the effect of gluten-free diet on patients presenting with ataxia caused by gluten sensitivity. 43 patients with gluten ataxia were studied. All were offered a gluten-free diet and monitored every six months. All patients underwent a battery of tests to assess their ataxia at baseline and after one year on diet. Twenty six patients (treatment group) adhered to the gluten-free diet and had evidence of elimination of antigliadin antibodies by one year. Fourteen patients refused the diet (control group). Three patients had persistently raised antigliadin antibodies despite adherence to the diet and were therefore excluded from the analysis. After one year there was improvement in ataxia reflected in all of the ataxia tests in the treatment group. This was significant when compared with the control group. The diet associated improvement was apparent irrespective of the presence of an enteropathy. Gluten ataxia responds to a strict gluten-free diet even in the absence of an enteropathy. The diagnosis of gluten ataxia is vital as it is one of the very few treatable causes of sporadic ataxia.

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Author and article information

Contributors

Hiroshi Mitoma: mitoma@tokyo-med.ac.jp

Journal

Journal ID (nlm-ta): Cerebellum Ataxias

Journal ID (iso-abbrev): Cerebellum Ataxias

Title: Cerebellum & Ataxias

Publisher: BioMed Central (London )

ISSN (Electronic): 2053-8871

Publication date (Electronic): 10 November 2015

Publication date PMC-release: 10 November 2015

Publication date Collection: 2015

Volume: 2

Electronic Location Identifier: 14

Affiliations

[ ]Department of Medical Education, Tokyo Medical University, Tokyo, Japan

[ ]Academic Department of Neurosciences, Royal Hallamshire Hospital, Sheffield, UK

[ ]University Lyon 1, University Lyon, Rue Guillaume Paradin, 69372 Lyon, Cedex 08 France

[ ]INSERM, UMR-S1028, CNRS, UMR-5292, Lyon Neuroscience Research Center, Neuro-Oncology and Neuro-Inflammation Team, 7, Rue Guillaume Paradin, 69372 Lyon, Cedex 08 France

[ ]National Reference Centre for Paraneoplastic Neurological Diseases, Hospices civils de Lyon, Hôpital neurologique, 69677 Bron, France

[ ]Hospices Civils de Lyon, Neuro-oncology, Hôpital Neurologique, 69677 Bron, France

Article

Publisher ID: 34

DOI: 10.1186/s40673-015-0034-y

PMC ID: 4641375

PubMed ID: 26561527

SO-VID: b8aca074-a768-46a5-8027-39dc2dc00e92

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Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 9 October 2015

Date accepted : 5 November 2015

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Keywords: cerebellar ataxias,gluten ataxia,paraneoplastic cerebellar degeneration,hashimoto’s encephalopathy,anti-gad antibodies,immunotherapy

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Keywords: cerebellar ataxias, gluten ataxia, paraneoplastic cerebellar degeneration, hashimoto’s encephalopathy, anti-gad antibodies, immunotherapy

Guidelines for treatment of immune-mediated cerebellar ataxias

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HLA-G and immune tolerance in pregnancy

Most cited references 58

Cerebellar ataxia with anti-glutamic acid decarboxylase antibodies: study of 14 patients.

Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone.

Dietary treatment of gluten ataxia.

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