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症例は50歳,女性。48歳より下肢脱力出現,歩行障害が徐々に進行。神経学的に下顎反射充進,四肢腱反射充進,病的反射陽性で感覚障害なく,四肢および舌の線維束攣縮陽性,遠位筋優位の筋力低下,筋萎縮を認め針筋電図で運動ニューロン病に一致する所見であった。
検査所見にて多クローン性高IgM血症および数種の自己抗体陽性を認めた。治療として血漿交換を3回実施したところ,血清IgMの減少と歩行障害の改善を認めた。本症例の抗myelin associated glycoprotein(MAG)抗体および抗糖脂質抗体は陰性であったが,大脳白質・灰白質の蛋白に対する抗体を検討したところ,54KD蛋白に対し陽性のバンドを認めた。
運動ニューロン病の病因を考える上で重要であると考えられた。
A patient, 50-year-old female, developed progres-sive weakness of lower extremities, and gait distur-bance for 2 years.
Neurological examination revealed hyperreflexia with pathological reflex, fasciculation in the limbs and tongue, muscle weakness and atrophy in distal limbs, but no sensory disturbance. Needle EMG showed neurogenic findings compatible with motor neuron disease (MND).
Laboratory data showed polyclonal IgM hyperim-munoglobulinemia, positive several autoimmune antibodies including antisingle strand DNA anti-body (Ab), ENAab, SS-A ab and RA. There were no antibodies for gangliosides and Myelin-associat-ed glycoprotein (MAG), but positive antibody for 54KD protein of cerebral gray and white matter.
The clinical manifestations including gait distur-bance and muscle weakness, and serum IgM levelwere moderately improved by plasmapheresis which is considered important for consideration of causes of MND.
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