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.
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Supported in part by a Grant-in-Aid for Scientific Research (c) 05680653 from the Ministry of Education, Science and Culture and a research grant for CNS degenerative diseases from the Ministry of Health and Welfare, Japan
Medical student of Leiden University, Holland, in 1981. He stayed on Guam and accomplished his thesis on part of this study under the guidance of Drs. K.-M. Chen and K. Oyanagi.
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Oyanagi, K., Makifuchi, T., Ohtoh, T. et al. Amyotrophic lateral sclerosis of Guam: the nature of the neuropathological findings. Acta Neuropathol 88, 405–412 (1994). https://doi.org/10.1007/BF00389491
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DOI: https://doi.org/10.1007/BF00389491