S. Ono et al., COLLAGEN ABNORMALITIES IN THE SPINAL-CORD FROM PATIENTS WITH AMYOTROPHIC-LATERAL-SCLEROSIS, Journal of the neurological sciences, 160(2), 1998, pp. 140-147
During the last 10 years, we have demonstrated morphological and bioch
emical abnormalities of skin extracellular matrices in amyotrophic lat
eral sclerosis (ALS). However, currently little is known concerning co
llagen of the spinal cord in ALS. We measured the amount of collagen a
nd characterized collagen at light and electron microscopic levels in
posterior funiculus, posterior half of lateral funiculus and anterior
horn of cervical enlargement of the spinal cord obtained from ten pati
ents with ALS, 11 patients with other neurologic diseases (control. gr
oup A), and ten patients without neurologic ones (control group B). In
posterior half of lateral funiculus and anterior hem, (1) by light mi
croscopy, there was no significant difference in vessel wall area betw
een ALS patients and control groups A and B; (2) ultrastructurally, co
llagen bundles were more fragmented and widely separated, and the fibr
ils were randomly oriented in the perivascular space of capillaries in
ALS patients, which were not observed in any areas of control groups
or in posterior funiculus of ALS patients; and (3) the collagen conten
ts in ALS were significantly lower (P<0.001 and P<0.001, respectively)
than those in control groups A and B. Fragmented and widely separated
collagen bundles in the interstitial tissue surrounding capillaries a
nd markedly decreased amount of collagen in posterior half of lateral
funiculus and in anterior horn of ALS could be related to the degenera
tion of the upper and lower motor neurons in the spinal cord in ALS, t
hat is, selective neuronal vulnerability in ALS. (C) 1998 Elsevier Sci
ence B.V. All rights reserved.