GUAMANIAN NEURODEGENERATIVE DISEASE - ULTRASTRUCTURAL STUDIES OF SKIN

It is evident that Guamanian amyotrophic lateral sclerosis (ALS) and p arkinsonism-dementia complex (PDC) are clinical variants of a single d isease entity and that Guamanian ALS is clinically indistinguishable f rom sporadic ALS. We studied by electron microscopy the skin tissues f rom 11 patients with Guamanian neurodegenerative disease (PDC and ALS) , 11 Chamorro control subjects, 10 Japanese patients with sporadic ALS and 11 Japanese control patients. Among patients with sporadic ALS, t here was an inverse relationship of collagen fiber diameter and the du ration of disease and a marked increase of amorphous material in the g round substance. These findings were not observed in the Guamanian pat ients or controls. Therefore, the skin studies reinforce the view of a different disease mechanism in Guamanian ALS and PDC compared to spor adic ALS. (C) 1997 Elsevier Science B.V.