Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Primary Sjögren's syndrome presenting with choreo-athetosis

Yoshihiko Nakazato, M.D., Toshimasa Yamamoto, M.D., Naotoshi Tamura, M.D., Kunio Shimazu, M.D. and Kenji Ishii, M.D.

Department of Neurology, Saitama Medical School

A 69-year-old man presenting with choreo-athetosis was proved to have primary Sjögren's syndrome. Choreo-athetosis suddenly appeared in the bilateral legs and the right arm; it was predominant in the right limbs. On the neurological examinations, there was no abnormal finding except for this involuntary movement. Brain MRI showed no abnormal finding. 18F-Fluorodeoxyglucose PET revealed that glucose metabolism was relatively increased in the bilateral striatum and thalamus (left side dominant). Dry eye and thirst were present for 4 years, and a diagnosis of primary Sjögren syndrome was confirmed by ophthalmological examinations, lip biopsy and sialography. Choreo-athetosis disappeared after thioridazine hydrochloride was started, while steroid was not effective.

(CLINICA NEUROL, 42: 946|948, 2002)
key words: choreo-athetosis, Sjögren syndrome, pernicious anemia

(Received: 13-Aug-02)