Clinical Characteristics and Management of 50 Patients with Anti-GAD Ataxia: Gluten-Free Diet Has a Major Impact.

Abstract

The objective of this study is to report the clinical characteristics and treatment of patients with progressive cerebellar ataxia associated with anti-GAD antibodies. We performed a retrospective review of all patients with anti-GAD ataxia managed at the Sheffield Ataxia Centre over the last 25 years. We identified 50 patients (62% females) with anti-GAD ataxia. The prevalence was 2.5% amongst 2000 patients with progressive ataxia of various causes. Mean age at onset was 55 and mean duration 8 years. Gaze-evoked nystagmus was present in 26%, cerebellar dysarthria in 26%, limb ataxia in 44% and gait ataxia in 100%. Nine patients (18%) had severe, 12 (24%) moderate and 29 (58%) mild ataxia. Ninety percent of patients had a history of additional autoimmune diseases. Family history of autoimmune diseases was seen in 52%. Baseline MR spectroscopy of the vermis was abnormal at presentation in 72%. Thirty-five patients (70%) had serological evidence of gluten sensitivity. All 35 went on gluten-free diet (GFD). Eighteen (51%) improved, 13 (37%) stabilised, 3 have started the GFD too recently to draw conclusions and one deteriorated. Mycophenolate was used in 16 patients, 7 (44%) improved, 2 stabilised, 6 have started the medication too recently to draw conclusions and one did not tolerate the drug. There is considerable overlap between anti-GAD ataxia and gluten ataxia. For those patients with both, strict GFD alone can be an effective treatment. Patients with anti-GAD ataxia and no gluten sensitivity respond well to immunosuppression.

• Authors: M Hadjivassiliou, P G Sarrigiannis, P D Shanmugarajah, D S Sanders, R A Grünewald, P Zis, N Hoggard
• Journal: Cerebellum (London, England) (Cerebellum) Vol. 20 Issue 2 Pg. 179-185 (Apr 2021) ISSN: 1473-4230 [Electronic] United States
• PMID: 33084997 (Publication Type: Journal Article)

Chemical References

• Autoantibodies
• Autoantigens
• Glutamate Decarboxylase

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Autoantibodies (immunology)
• Autoantigens (immunology)
• Autoimmune Diseases of the Nervous System (diet therapy, pathology)
• Cerebellar Ataxia (diet therapy, pathology)
• Diet, Gluten-Free
• Female
• Glutamate Decarboxylase (immunology)
• Humans
• Male
• Middle Aged
• Retrospective Studies
• Young Adult