大量免疫グロブリン療法が著効したシェーグレン症候群に伴うsensory ataxic neuropathyの高齢発症の1例

High-dose Intravenous Immunoglobulin in the Treatment of Sensory Ataxic Neuropathy with Sjgren's Syndrome : A Case Report

　要旨　症例は81歳男性。2002年8月頃より右手にしびれが出現し，その後しびれは四肢末梢より両肘および両膝まで拡がった。2003年より巧緻運動障害が出現し，歩行も不安定になった。四肢の遠位部優位に表在感覚の低下と深部感覚障害を認め，協調運動障害を伴った。深部腱反射は消失し，歩行は失調性であった。髄液検査で蛋白増加はなかったが，神経伝導検査で感覚神経伝導速度は導出不能であった。シルマー試験陽性，サクソン試験陽性，抗SS-A/Ro・抗SS-B/La抗体は陽性であり，シェーグレン症候群に伴う感覚性運動失調型ニューロパチー（SS-SAN）と診断した。大量免疫グロブリン療法（IVIg療法）を5日間施行し，施行2日目よりしびれ感は減少し，その後失調性歩行も改善した。IVIg療法はSS-SANに対し有効な治療法であると考えた。

　We report herein a case of sensory ataxic neuropathy with Sögren'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, IVIg, 400mg/kg daily for 5 days, was administered. His sensory impairment began to improve 2 days after IVIg. Subsequently, he could walk steadily without ataxia. It is considered that IVIg may be an effective treatment for SS-SAN.

（Received : December 22, 2003）