Parkinsonism-Dementia Complex, an Endemic Disease on the Island of Guam

HIRANO, ASAO; Malamud, Nathan; Kurland, Leonard T.

doi:10.1093/brain/84.4.662

Cited by 280 publications

(148 citation statements)

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“…Identical tangles are found in large numbers in the cortex and deep grey matter in the Parkinsonism dementia complex of Guam Hirano et al 1961;Hirano, 1974), in the anterior temporal cortex and substantia nigra in dementia pugilistica (Corsellis et al 1973;Wisniewski et al 19766). in the substantia nigra and locus coeruleus of post-encephalitic Parkinsonism (Wisniewski et al 1970 a), in the cortex of occasional cases of subacute sclerosing pan-encephalitis (Manydbur et al 1977;Paula-Barbosa et al 1979), and extensively in the majority of cases of Down's syndrome surviving to middle life (Olson & Shaw, 1969;Burger & Vogel, 1973;Solitare & Lamarche, 1966;Jervis, 1948).…”

mentioning

confidence: 97%

Plaques, tangles and Alzheimer's disease

Tomlinson¹

1982

Psychol. Med.

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confidence: 97%

Plaques, tangles and Alzheimer's disease

Tomlinson¹

1982

Psychol. Med.

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“…In both conditions the neurofibrillary tangles were conspicuous and consisted of 10 nm filaments and corresponded to the regions of neuronal loss and gliosis. 25 -30 - 34 The distribution of tangleaffected neurons throughout the nervous system in the two conditions was essentially the same (Table 2). This was especially evident when comparison was made with progressive supranuclear palsy and Alzheimer's disease where the distribution appears quite different.…”

Section: Pathological Featuresmentioning

confidence: 85%

Similarities of Guamanian ALS/PD to Post-Encephalitic Parkinsonism/ALS: Possible Viral Cause

Hudson

Rice

1990

Can. j. neurol. sci.

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Guamanian amyotrophic lateral sclerosis with parkinsonism-dementia (ALS/PD) has been the subject of intensive study since its discovery in 1947 because of its extraordinarily high incidence in a small ethnic group (Chamorros) whose dietary lack and customs have suggested possible causes. As yet, these and other suspected causes have eluded proof. Because of marked similarities between Guamanian ALS/PD and late onset post-encephalitic (encephalitis lethargica) parkinsonism and ALS it is suggested that they have a common cause. The parkinsonism and ALS in the two disorders are clinically very similar and neuropathological studies have shown a very similar distribution of neurofibrillary tangles in neurons. Some clinical differences, such as ocular features in the post-encephalitic cases and dementia in Guamanian ALS/PD, can be explained by differences in the severity of infection and the interval between the encephalitis and onset of sequelae. Although unproven, influenza A (HswilNl strain) has long been suspected as the cause of encephalitis lethargica because of simultaneous pandemics of the two diseases in the 1920s. Because influenza A can persistently infect cells and has a marked propensity to mutate it is an optimal candidate among other RNA viruses for delayed nervous system infection as a possible cause of ALS/PD. RESUME: Ressemblances de la SLA/MP de Guam et du parkinsonisme/SLA post-encephalitique: une cause virale possible La sclerose laterale amyotrophique avec parkinsonisme et demence de Guam (SLA/MP) a fait I'objet d'6tudes intensives depuis sa decouverte en 1947, a cause de son incidence extraordinairement elevee dans un petit groupe ethnique (Chamorros) ou Ton a soupconne des deficiences dietetiques et certaines coutumes comme causes possibles de la maladie. A date t il a ete impossible d'apporter des preuves en ce sens ou de confirmer le role de toutc autre cause qu'on a coupgonne. A cause d'une grande ressemblance entre la SLA/MP de Guam et du parkinsonisme postencephalitique (enc£phalite lethargique) a debut tardif avec SLA, nous suggerons qu'ils ont une cause commune. Lc parkinsonisme et la SLA dans les deux maladies sont cliniquement tres semblables et les etudes neuropathologiqucs ont montre' une distribution tres similaire des amas neurofibrillaires dans les neurones. Certaines differences cliniqucs, telles les manifestations oculaires dans les cas post-encephalitiques et la demence dans la SLA/MP de Guam, peuvent etre expliquees par des differences dans la severite de l'infection et l'intervalle entre l'encephalite et l'appartion des s6quelles. Bien qu'on n'en ait aucune preuve, le virus de 1'influenza A (la souche HswilNl) a longtempts ete soup£onne comme etant la cause de l'encephalite lethargique a cause de pandemies simultanees de ces deux maladies dans les anndes 1920. Parce que le virus de I'influenza A peut causer une infection cellulaire persistante et qu'il a une tendance marquee a la mutation, il est un candidat de choix comme cause possible de la SLA/MP parmi les virus...

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“…Although the aetiology of this disease is still unknown, the descriptions of hereditary amyotrophic lateral sclerosis (reviewed by Espinosa, Okihiro, Mulder, and Sayre, 1962) and the high incidence of motor neurone disease occurring in the Chamorros of the Mariana Islands, with and without the Parkinsonism-dementia syndrome, have reawakened interest in these disorders with particular regard to a possible genetic aetiology (Engel, Kurland, and Klatzo, 1959;Hirano, Kurland, Krooth, and Lessell, 1961).…”

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confidence: 99%