Guamanian neurodegenerative disease: Ultrastructural studies of skin

Seiitsu Ono, Stephen C. Waring, Leonard L. Kurland, Ulla Katrina-Craig, Ronald C. Petersen

Research output: Contribution to journalArticle

8 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)35-40
Number of pages6
JournalJournal of the neurological sciences
Volume146
Issue number1
DOIs
StatePublished - Feb 27 1997

Keywords

  • Parkinsonism-dementia complex
  • amyotrophic lateral sclerosis
  • collagen
  • skin

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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