Guamanian neurodegenerative disease: ultrastructural studies of skin

• Published in: Journal of the neurological sciences 1997-02, Vol.146 (1), p.35-40
• Main Authors: Ono, Seiitsu, Waring, Stephen C., Kurland, Leonard L., Katrina-Craig, Ulla, Petersen, Ronald C.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - pathology; Biological and medical sciences; Case-Control Studies; Collagen; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia - pathology; Diagnosis, Differential; Female; Guam; Humans; Male; Medical sciences; Microscopy, Electron; Middle Aged; Nerve Degeneration - physiology; Neurology; Parkinson Disease - pathology; Parkinsonism-dementia complex; Skin; Skin - pathology
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/S0022-510X(96)00273-0

It is evident that Guamanian amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) are clinical variants of a single disease entity and that Guamanian ALS is clinically indistinguishable from sporadic ALS. We studied by electron microscopy the skin tissues from 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, there was an inverse relationship of collagen fiber diameter and the duration of disease and a marked increase of amorphous material in the ground substance. These findings were not observed in the Guamanian patients or controls. Therefore, the skin studies reinforce the view of a different disease mechanism in Guamanian ALS and PDC compared to sporadic ALS. © 1997 Elsevier Science B.V. All rights reserved.