臨床神経学

<シンポジウム(2)―2―3>ALSの病態進行機序の新展開

ALS病変は連続して広がっていくか

叶内 匡1), 関口 輝彦2), 水澤 英洋2), 横田 隆徳2)

1)東京医科歯科大学医学部附属病院検査部〔〒113―8519 東京都文京区湯島1―5―45〕
2)東京医科歯科大学脳神経病態学

Using clinical information, it was investigated whether lesions in sporadic amyotrophic lateral sclerosis (sALS) spread contiguously from an onset site to the another regions in domino-like manner as hypothesized by prion-like propagation of pathogenic proteins. First, the data from medical records of 53 sALS patients with bulbar or lower limb onset showed that the symptom has noncontiguously spread from the bulbar region to the lower limbs or vice versa, skipping the upper limbs, in 18.9% of the patients. Second, in 18 patients with upper limb onset, correlation between the local progression speed of symptom severity in the onset limb and the interval from onset to involvement of the second region (lower limb) was investigated. The symptom severity was assessed by a score on "dressing and hygene", the subscale of the revised ALS functional rating scale. The two parameters should be positively correlated, if the lesion propagates contiguously from an initially affected motoneuron to the neighbouring ones within the same motoneuron pool (local progression) and then propagates to the another motoneuron pools (regional spread). However, the statistically significant correlation was not found, suggesting that there may be the different mechanisms between local progression and regional spread of ALS lesions.
Full Text of this Article in Japanese PDF (177K)

(臨床神経, 52:1059−1061, 2012)
key words:筋萎縮性側索硬化症,prion-like propagation,local progression,regional spread

(受付日:2012年5月24日)