Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Two distinct types of neuropathy associated with Sjögren's syndrome developed in one patient The importance of the selection of an appropriate therapeutic regimen

Yoshimasa Noguchi, M.D.1), Takaaki Tsuchiyama, M.D.1), Takashi Matsumoto, M.D.1), Hiroto Fujigasaki, M.D., Ph.D.1), Akira Inaba, M.D., Ph.D.2), Takanori Yokota, M.D., Ph.D.1), Takashi Kanda, M.D., Ph.D.1) and Hidehiro Mizusawa, M.D., Ph.D.1)

1)Department of Neurology and Neurological science
2)Department of Clinical Laboratory Graduate School, Tokyo Medical and Dental University

A 62-year-old woman was admitted to our hospital because of muscle weakness and sensory disturbance in extremities. She showed weakness, muscle atrophy and sensory abnormality in four limbs with patchy distribution, suggesting involvement of multiple peripheral nerve trunks. Serum titers of anti-SS-A, SS-B, and antinuclear antibody were elevated. Sural nerve biopsy showed recanalization and lymphocytic infiltration in the epineural small vessels, suggesting the presence of vasculitis. She was diagnosed as having vasculitic neuropathy complicated with Sjögren's syndrome. Methylprednisolone pulse therapy followed by oral prednisolone was started and these symptoms gradually improved in one month. At age 63, she felt dysesthesia in the right lower limb and this sensory abnormality spreaded to upper limbs. Two years later, she was admitted again due to clumsiness of hands and gait disturbance. Neurological examination showed decreased vibration and position sense of lower limbs and limb ataxia in addition to dysesthesia. Electrophysiological studies demonstrated significant decrease in amplitude of sensory nerve action potentials and delayed somatosensory evoked potentials after N13, indicating impairment of dorsal root ganglions. She was treated with intravenous immunoglobulin (400 mg/kg, total 15 g/day) for 5 days. One week later, sensory ataxia was improved. It has been known that Sjögren's syndrome is often complicate with various types of neuropathies including vasculitic neuropathy and sensory neuropathy. Our patient developed these two different types of neuropathies which were dramatically improved after two different therapeutic regimens; indicating the importance to select a suitable treatment regimen in accordance with the mechanism of neuropathy associated with Sjögren's syndrome.

(CLINICA NEUROL, 43: 539－543, 2003)
key words: Sjögren's syndrome, neuropathy, intravenous immunoglobulin, corticosteroid therapy

(Received: 16-Apr-03)