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Oct 29, 2018

7th Congress of the European Academy of Paediatric Societies

0978 - CEREBELLAR ATAXIA WITH ANTI-GAD AUTOANTIBODIES: A PEDIATRIC CASE

cerebellar ataxia

diabetes

glutamic acid decarboxylase auto-antibodies

Abstract

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Abstract

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Keywords

cerebellar ataxia

diabetes

glutamic acid decarboxylase auto-antibodies

Abstract

Background: Cerebellar ataxia with GAD (glutamic acid decarboxylase )auto-antibodies is a very rare condition. Since its first description, only a few cases have been reported in the literature. The treatment is still poorly codified. We report a new case whose particularity is the age of onset. Case presentation: It was a 3 years-old girl, known as type 1 diabetes, who presented 11 months after the discovery of his diabetes a static and kinetic cerebellar syndrome with progressive dysarthria. In the context of the etiological assessment, several radiological and biological explorations have been made . Autoimmune cerebellar ataxia was suspected on the elevated serum levels of anti-GAD antibodies (> 1500U / ml), absence of cerebellar lesions on MRI and elimination of other causes of cerebellar ataxia.The serology of celiac disease had returned positive. The patient received 6 monthly courses of veinoglobulin with bolus methylprednisolone followed by prolonged corticosteroid treatment with a gluten-free diet; allowing a partial regression of symptoms. Conclusion: Cerebellar ataxia with anti-GAD antibodies is a rare condition. The potential severity of the disease and the risk of perpetuation of the disorders imply the need for rapid diagnosis and the establishment of effective therapy. Repeated dosing of anti-GAD antibodies should be performed for any unexplained cerebellar syndrome including normal imaging. The frequent presence of insulin-dependent diabetes mellitus should not contraindicate the initiation of potentially beneficial early corticosteroid therapy to prevent cerebellar atrophy.

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© Copyright 2019 Morressier GmbH.
All rights reserved.