Annals of Movement Disorders (Apr 2024)

Anti-glutamic acid decarboxylase antibodies-associated cerebellar ataxia: A treatable ataxia

  • Rohan Mahale,
  • Sandeep M,
  • Anita Mahadevan,
  • Nitish Kamble,
  • Vikram Holla,
  • Ravindranadh Mundlamuri,
  • Seena Vengalil,
  • Netravathi M,
  • Atchayaram Nalini,
  • Pramod Kumar Pal,
  • Ravi Yadav

DOI
https://doi.org/10.4103/aomd.aomd_23_23
Journal volume & issue
Vol. 7, no. 1
pp. 13 – 18

Abstract

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BACKGROUND: Anti-glutamate decarboxylase 65 antibody-associated cerebellar ataxia (anti-GAD65Ab-associated CA) is the most widely studied immune-mediated CA. There are few case series evaluating the clinical, radiological, treatment profile, and outcome of anti-GAD65Ab-associated CA. OBJECTIVE: To study the clinical, radiological profile, associated neoplasm, treatment outcome, and prognosis in patients diagnosed with anti-GAD65Ab-associated CA. METHODS: A retrospective descriptive analysis of a cohort of patients diagnosed with anti-GAD65Abassociated CA was performed and analyzed. RESULTS: Thirteen patients were selected for the analysis with female predominance (70%). The mean age at presentation was 47.5 } 11.1 years (range, 29–65 years), and the median duration of the symptoms was 4 months. All 13 patients (100%) had gait ataxia. Ten patients had limb ataxia along with gait ataxia (75%). Seven patients had cerebellar dysarthria (54%). Four patients (31%) had gazeevoked jerky nystagmus. Five patients (39%) were diagnosed with type 2 diabetes mellitus, and one patient had hypothyroidism in addition to type 2 diabetes mellitus. Brain magnetic resonance imaging was normal in seven (54%) patients, and pure cerebellar atrophy was observed in six patients. One patient was detected with a colon neoplasm. All 13 patients received intravenous methylprednisolone, followed by oral steroids over 3 months. Six patients (50%) received plasma exchange along with intravenous methylprednisolone. Favorable outcomes (modified Rankin scale score 2) were observed in five patients and poor outcomes in two; six patients were lost to follow-up after the first admission. CONCLUSION: Anti-GAD65Ab-associated CA presents as subacute to chronic progressive CA with a favorable outcome with immunotherapy. Anti-GAD antibodies should be assessed in serum or cerebrospinal fluid in patients presenting subacute to chronic sporadic CA. The occurrence of a systemic neoplasm is rare in anti-GAD65Ab-associated CA.

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