ESPE Abstracts (2014) 82 P-D-3-3-750

A Case of Type 1 Diabetes Associated with Cerebellar Ataxia: Stiff-Person Syndrome

Selim Kurtoğlu, Deniz Ökdemir, Nihal Hatipoğlu, Leyla Akın, Ülkü Gül, Mehmet Canpolat & Mustafa Kendirci


Erciyes University, Kayseri, Turkey


Background: Stiff-person syndrome (SPS) is a rare disorder which is characterized by muscle rigidity, spasm and cerebellar abnormalities. The etiology is not clarified yet. 80% of cases are caused by an autoantibody against GAD that inhibits synthesis of GABA. Other autoimmune diseases such as type 1 diabetes mellitus and thyroiditis are often associated.

Aim: To underline the importance of considering SPS in differential diagnosis of patients with type 1 diabetes associated with neurological abnormalities.

Case: A 10-year-old girl was referred to our department due to hyperglycemia. The diagnosis of type 1 diabetes was made upon clinical and laboratory findings. Blood glucose was 500 mg/dl, HbA1c 8%, and serum anti-GAD antibody was positive. Serum thyroid autoantibodies were also elevated. She had been diagnosed with cerebellar ataxia 1 year ago but the etiology could not be established. She had no muscle rigidity or spasm. On follow-up, cranial MRI revealed atrophic changes in cerebellum. Cerebrospinal fluid examination was negative for anti-GAD antibodies.

Conclusion: Although the etiology of SPS is undefined and clinical spectrum is large, this syndrome should be considered in the differential diagnosis of patients with type 1 diabetes associated with neurological abnormalities.

Article tools

My recent searches

No recent searches.