A case of autoimmune polyglandular syndrome type 3 with concomitant autoimmune cerebellar ataxia positive for anti-glutamic acid decarboxylase antibody and anti-gliadin antibody

Abstract

[Case] The patient was a 42-year old woman who was diagnosed as Graves' disease (GD) at 14 years of age. She developed slowly progressive insulin-dependent diabetes mellitus (SPIDDM) at 35 years of age, temporal lobe epilepsy at 40 years of age. In February on the year of presentation, she began to feel dizziness, and was hospitalized with ketosis as a resolt of not entiny due to sickness and the deterioration of GD in April. After discharge with the improvement of her symptoms, she developed diplopia, nystagmus and cerebellar ataxia, and was admitted to hospital again for examination and treatment in June. Based on the detection of anti- glutamic acid decarboxylase antibodies (GAD-Ab) and anti-gliadin antibodies in her serum and cerebrospinal fluid, she was diagnosed with autoimmune cerebellar ataxia. Treatment with plasma exchange, steroid pulse therapy, oral prednisolone and a gluten-free diet gradually improved the symptoms of ataxia, and she was fi- nally discharged. The therapy improved the patient's GD and antithyroid drugs could be discontinued; how- ever, her insulin dose was higher than that before admission. [Discussion] Since this is a very rare case of a patient with autoimmune cerebellar ataxia who was found to be positive for GAD-Ab and anti-gliadin anti- bodies during the course of APS type 3, we report the clinical course and influence of SPIDDM and GD. If a patient with autoimmune endocrine disease develops cerebellar ataxia, autoimmune cerebellar ataxia should be suspected and investigated early.