[A new case of cerebellar ataxia with anti-GAD antibodies treated with corticosteroids and initially seronegative].

Abstract

INTRODUCTION:

Cerebellar ataxia with antiglutamic acid decarboxylase antibodies (GAD-ab) is an exceptional newly recognized autoimmune disorder. The cerebellar ataxia may occur in isolation or be associated with stiff man syndrome another rare GAD-Ab induced disorder of central nervous system.

EXEGESIS:

A 38-year-old woman with a past history of Graves disease presented with insidious cerebellar symptoms including ataxic gait, dysmetria, dysarthria, and oscillopsia. A thorough survey of markers of paraneoplastic cerebellar ataxia and collagen diseases was negative. Her serum contained high level of GAD-ab (647.2 U/ml) and MRI evidenced pure cerebellar atrophy leading to diagnosis of autoimmune cerebellar ataxia. Under corticosteroids, cerebellar symptoms partially improved, but serum GAD-ab titre dramatically decreased.

CONCLUSION:

Testing for GAD-ab may be indicated in patients with idiopathic cerebellar ataxia, particularly mature women with organ-specific autoimmune diseases. Corticosteroids must be started to prevent irreversible cerebellar atrophy.

• Authors: B Birand, P Cabre, M Bonnan, S Olindo, D Smadja
• Journal: La Revue de medecine interne (Rev Med Interne) Vol. 27 Issue 8 Pg. 616-9 (Aug 2006) ISSN: 0248-8663 [Print] France
• Vernacular Title: Un nouveau cas d'ataxie cérébelleuse à anticorps anti-GAD traité par corticoïdes et initialement séronégatif.
• PMID: 16797794 (Publication Type: Case Reports, Comparative Study, English Abstract, Journal Article)

Chemical References

• Adrenal Cortex Hormones
• Autoantibodies
• Glutamate Decarboxylase

Topics

• Adrenal Cortex Hormones (administration & dosage, therapeutic use)
• Adult
• Atrophy
• Autoantibodies (blood)
• Autoimmune Diseases (drug therapy)
• Cerebellar Ataxia (diagnosis, drug therapy, immunology)
• Cerebellum (pathology)
• Female
• Glutamate Decarboxylase (immunology)
• Humans
• Magnetic Resonance Imaging
• Time Factors
• Treatment Outcome