Kuzuhara & Kokubo (2005). Atypical parkinsonism of Japan: Amyotrophic lateral sclerosis-parkinsonism-dementia complex of the Kii peninsula of Japan (Muro disease): An update.

[Wise Young]

Neurodegenerative diseases are often related and share mechanisms. This is a condition where there is motoneuronal degeneration associated with striatonigral degeneration typical of Parkinson's disease. The Parkinson's is the first to show. There are similarities with Alzheimer's. However, the families that have this disease show none of the genetic abnormalities that have been associated with ALS or Alzheimer's disease.

Kuzuhara S and Kokubo Y (2005). Atypical parkinsonism of Japan: Amyotrophic lateral sclerosis-parkinsonism-dementia complex of the Kii peninsula of Japan (Muro disease): An update. Mov Disord 20 Suppl 12: S108-13. An update of the endemic parkinsonism-dementia complex (PDC) frequently associated with amyotrophic lateral sclerosis (ALS) in the high prevalence ALS focus of the Kii peninsula of Japan is presented. The initial symptom was parkinsonian gait or hypobulia/amnesia, which was followed by akinesia, rigidity, occasional tremor, bradyphrenia, abulia and amnesia, and finally by akinetic mutism. In several years, most of the patients developed ALS symptoms such as muscle atrophy, bulbar palsy, and upper motor neuron signs. Magnetic resonance imaging and computed tomography of the brain showed marked atrophy of the temporal and frontal lobes and the cerebral blood flow reduction on single-photon emission computed tomography. Marked loss of nerve cells associated with abundant neurofibrillar tangles (NFTs) in the entire central nervous system, most predominantly in the brainstem and temporal lobe was characteristic. Concomitant ALS pathology involving the upper and lower motor neurons was common, and senile plaques were absent in most cases. NFTs consisted of twisted tubules on electron microscopy. Western blot of tau protein showed three bands consisting of six tau isoforms, similar to those of Alzheimer's disease. A family history of ALS/PDC was recorded in more than 70% of patients, but no abnormal mutation or polymorphism was found in the genes of SOD1, tau, and apolipoprotein E. Familial nature and continuing morbidity of Kii ALS/PDC suggest that genetic factors may be more likely in its pathogenesis. (c) 2005 Movement Disorder Society. Department of Neurology, Mie University School of Medicine, Tsu, Japan. http://www.ncbi.nlm.nih.gov/entrez/q..._uids=16092099

[Wise Young]

The fact that this disease hit once, peaked, and then disappeared suggests strongly that something caused it, i.e. an infection, a toxin, a combination of genetics and environment? Nobody knows.

Steele JC (2005). Parkinsonism-dementia complex of Guam. Mov Disord 20 Suppl 12: S99-S107. On Guam and in two other Pacific locales, indigenous residents and immigrants are prone to familial neurodegeneration that manifests as atypical parkinsonism, dementia, motor neuron disease, or a combination of these three phenotypes. This progressive and fatal disease of the Mariana islands, the Kii peninsula of Japan, and the coastal plain of West New Guinea is similar and the pathological features have close affiliation with universal tauopathies, including progressive supranuclear palsy, Alzheimer's disease, and amyotrophic lateral sclerosis. The Chamorros of Guam call the disease lytico-bodig, and neuroscientists refer to it as the amyotrophic lateral sclerosis/Parkinsonism-dementia complex. During recent decades, its prevalence has declined progressively, and the age at onset has steadily increased. In 2004, motor neuron disease, once 100 times more common than elsewhere is rare, atypical parkinsonism is declining, and only dementia remains unusually common in elderly females. The cause of this obscure malady remains uncertain, despite 60 years of international research, but its ending implicates environmental influences rather than genetic predisposition. (c) 2005 Movement Disorder Society. Micronesian Health Study II, Tamuning, Guam. http://www.ncbi.nlm.nih.gov/entrez/q..._uids=16092098

[Sue Pendleton]

The fact that this disease hit once, peaked, and then disappeared suggests strongly that something caused it, i.e. an infection, a toxin, a combination of genetics and environment? Nobody knows.

Sixty years of research? I went to Entrez Med but it appears the abstract is all that is readily available. Could this be connected to the testing of the atomic bombs near Bikini Atoll or to the original bombs that we dropped on Japan? It would have to be exposure only to a certain amount as the west coast of New Guinea and Guam are rather far away from the wind patterns of the targeted cities. Seems I read somewhere that the bombs were first flown to Guam and then Wake Island but I could be wrong here. Where is the Aii Penisula, Wise? All these places were also, excepting possibly the Aii Pennisula, sites of fighting between the Japanese and Americans. Maybe a chemical used in weapons such as phosphorus could have caused the rather short time period of this disease?