Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of ataxic sensory neuronopathy associated with Sjögren's syndrome

Masanori Sawamura, M.D.1)2), Wataru Oishi, M.D.1), Etsuro Nakanishi, M.D.1), Shinichiro Maruhama, M.D.1), Gan Kim, M.D.1) and Kiyoshi Harada, M.D., Ph.D.1)

1)Department of Neurology, Shizuoka General Hospital
2)Present Address: Department of Neurology, Sumitomo Hospital

A 71-year-old woman developed advanced thermal hypoalgesia, bathyesthesia, and significant sensory ataxia 1 year ago. She also had difficulty maintaining a sitting posture. Patchy and reduced thermal nociception corresponding to a dermatome was found in her four extremities and trunk. On the basis of several tests, she was diagnosed with ataxic sensory neuronopathy due to dorsal root ganglionitis associated with Sjögren's syndrome. Generally, dorsal root ganglionitis associated with Sjögren's syndrome is refractory. After treatment with simple plasmapheresis, she was able to maintain a sitting posture. Finally, her symptoms stabilized after the inclusion of oral D-penicillamine to her treatment regimen. Although the clinical course was observed for about one year, we report this case because of its valuable finding, i.e., her symptoms improved after simple plasmapheresis and oral administration of D-penicillamine.
Full Text of this Article in Japanese PDF (2545K)

(CLINICA NEUROL, 54: 413|416, 2014)
key words: Sjögren's syndrome, ataxic sensory neuronopathy, dorsal-root ganglionitis

(Received: 16-May-14)