Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of motor dominant neuropathy and focal segmental glomerulosclerosis associated with Sjögren's syndrome

Atsushi Yamamoto, M.D.1), Keisuke Imai, M.D.1), Masashi Hamanaka, M.D.1),Takehiro Yamada, M.D.1), Hidekazu Yamazaki, M.D.1), Kazuma Tsuto, M.D.1),Yukiko Tsuji, M.D.2), Noriyuki Yamashita, M.D.3) and Masatoshi Kadoya M.D., Ph.D.4)

1)Department of Neurology and Stroke Treatment, Kyoto First Red Cross Hospital
2)Department of Neurology, Kyoto Prefectural University of Medicine
3)Department of Nephrology, Kyoto First Red Cross Hospital
4)Department of Rheumatology, Kyoto First Red Cross Hospital

A 49-year-old woman was admitted to our hospital with gradually progressive weakness of the limbs for about 20 days. She presented with weakness of the limbs, predominantly in the proximal portion, and slight dysesthesia of the limbs, predominantly in the distal portion. Repeated nerve conduction examination revealed axonopathy dominantly in the motor neurons. Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögren's syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögren's syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögren's syndrome.
Full Text of this Article in Japanese PDF (655K)

(CLINICA NEUROL, 55: 732－736, 2015)
key words: Sjögren's syndrome, motor dominant neuropathy, immunoglobulin therapy, steroid therapy, focal segmental glomerulosclerosis

(Received: 16-Mar-15)