High-dose intravenous immunoglobulin in the treatment of sensory ataxic neuropathy with Sjogren's syndrome: a case report

摘要

We report herein a case of sensory ataxic neuropathy with Sjogren's syndrome (SS-SAN) who became dramatically improved in response to high-dose intravenous immunoglobulin treatment (IVIg). An 81-year-old man began to feel numbness in his hands and feet in August 2002. Because he became unsteady and could not do skillfull movement, he was admitted to our hospital in May 2003. On neurological examination, all tendon reflexes were absent. His vibratory and position senses were severely impaired to knees and elbows. Touch, temperature, and pinprick sensations were mildly disturbed in a glove-stocking distribution. Coordination was clumsy in all limbs because of sensory loss. He had gait ataxia with Romberg sign. Nerve conduction study revealed that sensory nerve action potentials were absent. He was diagnosed as having SS-SAN because Schirmer test, Saxon test and both SS-A and SS-B antibodies were positive. Thereafter, Mg, 400 mg/kg daily for 5 days, was administered. His sensory impairment began to improve 2 days after Mg. Subsequently, he could walk steadily without ataxia. It is considered that IVIg may be an effective treatment for SS-SAN.