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ARTICLES: D. Galasko, D. P. Salmon, U.-K. Craig, L. J. Thal, G. Schellenberg, and W. Wiederholt Clinical features and changing patterns of neurodegenerative disorders on Guam, 1997–2000 Neurology 2002; 58: 90-97 [Abstract] [Full text] [PDF]

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Reply to Letter to the Editor

Douglas Galasko, David Salmon, Ulla-Katrina Craig, Daniel Perl and Gerard Schellenberg   (6 May 2002)

Clinical features and changing patterns of neurodegenerative disorders on Guam,

John C Steele, John Hardy, Katrina Gwinn-Hardy, Patrick McGeer, Huw Morris, and Jordi Perez-Tur   (6 May 2002)

Reply to Letter to the Editor 6 May 2002
Douglas Galasko VA Medical Center San Diego, CA, David Salmon, Ulla-Katrina Craig, Daniel Perl and Gerard Schellenberg Send Correspondence to journal: Re: Reply to Letter to the Editor dgalasko{at}ucsd.edu Douglas Galasko, et al.	We thank Hardy et al for their historical notes and comments. As a small point, it is the recollection of many long time researchers and staff on Guam that the term Mariana dementia was coined by the late Dr Leonard Kurland several years before the Lavine et al study. We wish to emphasize that the cohort of patients with dementia that we have clinically characterized differs from patients in the majority of past studies on Guam for several reasons. Life expectancy on Guam has increased, so that we have now been able to recruit and evaluate a reasonable sized group of people aged 70 and over throughout the island. As the incidence of PDC has declined, other potential causes of dementia have gained importance. Late-life dementia was not systematically studied in prior research on Guam that focused on PDC, much of which was carried out before the research tools were developed and validated to enable cross -cultural clinicopathological studies of Alzheimer's disease AD and aging. Autopsy examination of brain tissue is indeed an aim of our studies. The autopsy rate in the past 5 years has been relatively low, which hampers these efforts. Our limited experience of neuropathological examination of a small number of brains of patients with late-life (Mariana) dementia has shown an interesting range of findings, including Alzheimer's disease, strokes, Lewy bodies, or tangle-predominant pathology. When we have had the opportunity to examine brain tissue from a sufficient number of clinically well-characterized patients with late-life dementia, we will be very interested in publishing the findings.
Clinical features and changing patterns of neurodegenerative disorders on Guam, 6 May 2002
John C Steele Fellow American College of Physicians Tamuning Guam, John Hardy, Katrina Gwinn-Hardy, Patrick McGeer, Huw Morris, and Jordi Perez-Tur Send Correspondence to journal: Re: Clinical features and changing patterns of neurodegenerative disorders on Guam, jsteele{at}kuentos.guam.net John C Steele, et al.	Mariana's dementia (MD) was first brought to attention during house- to-house surveys of neurodegenerative disease in southern villages of Guam during 1987-1988. [1] Lavine et al identified 55 cases of progressive neurodegeneration among 3579 residents. Thirty-six had ALS or parkinsonism, but 19 cases (35%) had only dementia. Because such a "pure" dementia had not previously been identified as a clinical entity on Guam, Lavine et al coined the term Marianas dementia (MD). They were not certain then if this was the same dementia that accompanies parkinsonism in the parkinsonism-dementia complex (PDC) (2), or if it was a different and separate, age-related dementia of late life. During 1997-2000, Galasko et al. used current clinical research methods to evaluate 83 Guamanaian Chamorros with MD and 90 subjects with PDC. They find the psychometric characteristics of MD and the dementia of PDC are the same, and also meet criteria for probable or possible AD. They concur with the earlier proposition of Lavine et al that Mariana's dementia is a distinct clinical entity (phenotype): this phenotype occurs in late life, is more common in women, and is not accompanied by parkinsonism or ALS. Hirano and Malamud (1960) were the first to investigate the pathology of dementia and parkinsonism on Guam. [4] In 17 cases they observed Alzheimer's neurofibrillary tangles more widespread than those seen in typical AD. Furthermore none of the 17 had senile plaques that are the hallmark of AD. By 1979, 293 Guamanian Chamorro cases of parkinsonism and dementia (PDC) had been found to have prominent and widespread neurofibrillary degeneration in cerebral cortex, basal ganglia, brain stem and cerebellum [5]. Senile plaques and Lewy bodies were more frequent findings in those dying during the last decade (1970-79), although overall they occurred in only 6 to 7% of all cases, including those of advanced age. Remarkably, classic neuropathological Alzheimer's and Parkinson's disease (PD) were never diagnosed in these studies. The article of Galasko et al. provides a current update of the clinical features and changing patterns of neurodegenerative disorders on Guam. However, what is sorely lacking is the neuropathological description of this cohort of the Chamorro population. It is crucial to know whether the changing clinical features of neurodegeneration on Guam are mirrored by the neuropathology. The key question is to determine whether, as the clinical features of neurodegeneration in the Guam population become more like those of the mainland United States, the pathological features also become more similar. REFERENCES: 1) Lavine L, Steele J, Wolfe N, Calne D, O'Brien P, Williams D, Kurland L, Schoenberg B. Amyotrophic lateral sclerosis/parkinsonism- dementia complex in Southern Guam: Is it disappearing? Advances in Neurology Vol.56: Amyotrophic lateral sclerosis and other motor neuron diseases. Edited by Lewis Rowland Raven Press 1991;Chapter 23:271-285. 2) Hirano A, Kurland L, Krooth R, Lessell S. Parkinsonism-dementia complex, an endemic disease on the island of Guam: I Clinical features. Brain 1961; 84:642-661. 3) Galasko D, Salmon D, Craig UK, Thal L, Schellenberg G, Wiederholt W. Clinical features and changing patterns of neurodegenerative disorders on Guam, 1997-2000. Neurology 2002;58(1):90-97. 4) Hirano A, Malamud N, Kurland L. Parkinsonism-dementia complex, an endemic disease on the island of Guam: II- Pathological features. Brain 1961; 84:662-679. 5) Rogers-Johnson P, Garruto R, Yanagihara R, Chen KM, Gajdusek DC, Gibbs C Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam: A 30 year evaluation of clinical and neuropathological trends. Neurology 1986;36:7-13.