Acta Neuropathologica

, Volume 88, Issue 5, pp 405–412 | Cite as

Amyotrophic lateral sclerosis of Guam: the nature of the neuropathological findings

  • Kiyomitsu Oyanagi
  • Takao Makifuchi
  • Takashi Ohtoh
  • Kwang Ming Chen
  • Tjeert van der Schaaf
  • D. Carlton Gajdusek
  • Thomas N. Chase
  • Fusahiro Ikuta
Regular Paper

Abstract

To elucidate the fundamental differences and similarities of the neuropathological features and etiopathogenesis of the amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) of Guam, we conducted a topographic, quantitative and histological investigation of tau-containing neurons, neurofibrillary tangles (NFTs), Bunina bodies and ubiquitinated inclusion bodies in 27 non-ALS non-PDC Guamanian subjects, as well as 10 Guam ALS patients, 28 PDC patients, and 5 patients with combined ALS and PDC (ALS-PDC). The topographic distribution of NFTs was basically the same in each disease and also in the non-ALS non-PDC group. There were relatively few, if any, NFTs in non-ALS non-PDC subjects and ALS patients, but there were many, especially in the frontal and temporal cortex, in Guam PDC and ALS-PDC patients. The histological and ultrastructural features of Bunina bodies in Guam ALS and ALS-PDC patients were similar to those reported in classic ALS. The ratio of occurrence of the inclusion in Guam ALS and ALS-PDC patients was similar to that reported so far in classic ALS. Ubiquitinated skein-like inclusion bodies were observed in the spinal anterior horn cells in Guam ALS and ALS-PDC patients. These findings indicate that classic ALS does exist on Guam, that NFTs in Guam ALS patients are merely a background feature widely dispersed in the population, that the mechanism of neuronal degeneration of Guam ALS is basically different from that of PDC, and that Guam ALS occurs initially as classic ALS.

Key words

Amyotrophic lateral sclerosis Parkinsonism-dementia complex Guam Neuropathology Quantitative study 

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Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • Kiyomitsu Oyanagi
    • 1
  • Takao Makifuchi
    • 1
  • Takashi Ohtoh
    • 2
  • Kwang Ming Chen
    • 3
  • Tjeert van der Schaaf
    • 4
  • D. Carlton Gajdusek
    • 4
  • Thomas N. Chase
    • 4
  • Fusahiro Ikuta
    • 2
  1. 1.The Center for Materials of Brain Diseases, Brain Research InstituteNiigata UniversityNiigataJapan
  2. 2.Department of Pathology, Brain Research InstituteNiigata UniversityNiigataJapan
  3. 3.Guam Memorial HospitalTamuningU.S.A.
  4. 4.National Institute of Neurological Disorders and StrokeNational Institutes of HealthBethesdaU.S.A.

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