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Decreased type IV collagen of skin and serum in patients with amyotrophic lateral sclerosis

From the Departments of Neurology (Drs. Ono, Yamano, and Shimizu) and Surgical Pathology (Dr. Nagao), Teikyo University School of Medicine, Ichihara Hospital, Ichihara; Department of Neurology (Dr. Imai), National Sanatorium Chiba-Higashi Hospital, Chiba; and Department of Neurology (Drs. Takahashi and Jinnai), National Sanatorium Hyogo Chuo Hospital, Sanda, Japan; and the Dental Research Center (Dr. Yamauchi), The University of North Carolina at Chapel Hill, NC.

Address correspondence and reprint requests to Dr. S. Ono, Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, 3426-3, Anesaki, Ichihara, Chiba 299-01, Japan.

Objective: To study type IV collagen of skin and serum in patients with ALS.

Background: Collagen abnormalities of skin have been reported in ALS patients. However, little is known concerning type IV collagen in ALS.

Methods: We studied type IV collagen immunoreactivity of skin and measured serum levels of the 7S fragment of the N-terminal domain of type IV collagen (7S collagen) in patients with ALS and control subjects.

Results: The basement membrane as well as blood vessels of skin in ALS patients was weakly positive for type IV collagen as compared with those of diseased control subjects. This weak immunostaining became more pronounced as ALS progressed. The optical density for type IV collagen immunoreactivity in ALS patients was significantly lower (p < 0.001) than in diseased control subjects and was significantly decreased with duration of illness(r = -0.85, p < 0.01). Serum 7S collagen levels in patients with ALS were significantly decreased (p < 0.01) as compared with those in diseased and healthy control subjects and were negatively and significantly associated with duration of illness (r= -0.81, p < 0.001). There was an appreciable positive correlation between concentrations of serum 7S collagen and the density for type IV collagen immunoreactivity in ALS patients (r = 0.81, p < 0.02).

Conclusions: These data suggest that a metabolic alteration of type IV collagen may take place in the skin of ALS patients and that the decreased levels of serum 7S collagen may reflect a decreased type IV collagen immunoreactivity of skin in patients with ALS.

Received October 8, 1997. Accepted in final form March 18, 1998.

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