臨床神経学

<シンポジウム(2)―2―4>ALSの病態進行機序の新展開

筋萎縮性側索硬化症の病態進展様式と予後

木村 文治

大阪医科大学内科学講座(I)神経内科〔〒569―8686 大阪府高槻市大学町2―7〕

A misfolding pathology has been suggested to spread from onset site to neighbouring areas in a prion-like manner. We examined the pattern of spread and direction of clinical lower motor neuron involvement over time until the appearance of respiratory symptom. A total of 150 patients with sporadic ALS underwent follow-up until respiratory symptoms. Symptom appearances were determined using ALSFRS-R. The interval from onset to involvement of the second region correlated significantly with survival, independent of particular combinations. No patient with a rapid spread pattern (two regions within 3 months from onset) survived >5 years. Time from onset to spread to the second site, suggesting propagation speed, is a strong predictor for survival. In terms of cumulative occurrence, symptoms spread longitudinally to adjacent regions. In most of ALS patients, the spread appears to have a contiguous pattern rather than a random pattern of progression, although the spread to non-contiguous regions was observed in 13%. This finding supports the notion that the ALS pathology gradually spreads to adjacent regions in a longitudinal manner. Although precise mechanism of symptom spread in ALS is not understood, knowledge about the pattern of onset and the anatomical direction of spread may provide valuable prognostic insights.
Full Text of this Article in Japanese PDF (372K)

(臨床神経, 52:1062−1065, 2012)
key words:ALS評価スケール,プリオン様伝播,連続性伝播,進展様式,下位運動ニューロン

(受付日:2012年5月24日)