Alterations of skin glycosaminoglycans in patients with ALS.
OBJECTIVE
Collagen abnormalities of skin have been reported among patients with ALS. However, little is known concerning glycosaminoglycans of the skin in ALS. Our objective was to clarify morphologic and biochemical findings of skin glycosaminoglycans among patients with ALS.


METHODS
We performed morphologic studies and biochemical analysis of glycosaminoglycans of skin from 8 patients with ALS, 6 patients with other neurologic or muscular diseases (control group A), and 7 patients without neurologic disorders (control group B).


RESULTS
The wide spaces that separate collagen bundles reacted strongly with Alcian blue stain in skin from patients with ALS and stained more markedly as ALS progressed. Staining with Alcian blue was virtually eliminated by Streptomyces hyaluronidase. The content of hyaluronic acid was significantly higher (p < 0.001) among patients with ALS than in control groups A and B. There was a significant positive correlation between content of hyaluronic acid and duration of illness among patients with ALS (r = 0.88, p < 0.01). However, there was no significant difference in content of dermatan sulfate, chondroitin sulfate-4S, or chondroitin sulfate-6S between patients with ALS and control groups A and B. There was also an appreciable positive correlation between optical density of Alcian blue and content of hyaluronic acid among patients with ALS (r = 0.92, p < 0.01).


CONCLUSIONS
The data suggest that a metabolic alteration of glycosaminoglycans related to the increased amount of hyaluronic acid may take place in the skin of patients with ALS.
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
S
S
Ono
T
T
Imai
A
A
Aso
T
T
Yamano
N
N
Shimizu
K
K
Nagao
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism*; D002851:Chromatography, High Pressure Liquid; D004187:Disaccharides / Q000378:metabolism; D005260:Female; D006025:Glycosaminoglycans / Q000378:metabolism*; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D008875:Middle Aged; D010783:Photometry; D012680:Sensitivity and Specificity; D012867:Skin / Q000378:metabolism*
D004187:Disaccharides; D006025:Glycosaminoglycans