Guamanian neurodegenerative disease: ultrastructural studies of skin

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...

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Bibliographic Details
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
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Summary: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.
ISSN:0022-510X
1878-5883
DOI:10.1016/S0022-510X(96)00273-0