Hetereogeneity of Neurodegenerative Diseases on Guam (P05.037)

Abstract

Objective: To characterize the full spectrum of dementia in 36 patients in Guam. Background The amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC) is a clinically-defined syndrome endemic to Guam. While Guam ALS is clinically indistinguishable from ALS seen outside Guam, PDC is distinctive syndrome consisting of parkinsonism, dementia, and oculomotor findings reminiscent of progressive supranuclear palsy. As most clinical series of dementia in Guam have predated current dementia research criteria and validated measures for evaluating cognitive and behavioral deficits, we sought to provide detailed clinical characterization of Guam9s various neurodegenerative syndromes. Design/Methods: We performed neurological evaluations on 36 consecutive patients with dementia; a subset received neuropsychological testing and brain MRI scans. Ten were from Umatac (historically exhibiting the highest prevalence of ALS/PDC on Guam) and 26 were recruited from a dementia daycare center (DDC) serving the entire island. We classified patients using current published clinical research criteria. Results: In Umatac, diagnoses included: PDC (5), choreiform disorders (2), PDC/ALS (1), behavioral variant FTD (bvFTD) (1), and amnestic/executive mild cognitive impairment (1). All patients from Umatac had a family history of neurodegenerative diseases, including PDC, ALS, ALS/PDC, and Parkinson9s disease (PD). Patients attending the DDC included: AD/vascular (7), AD (5), vascular dementia (3), frontal AD (2), dementia with Lewy bodies (DLB, 2), PDC (2), PDC/vascular (1), ALS/PDC (1), AD/DLB (1), PD (1), and semantic variant primary progressive aphasia (svPPA, 1). Among those seen at the DDC, 38% had a family history of dementia. Conclusions: Dementia on Guam is hetereogeneous and includes common diagnoses such as AD and vascular dementia. Although most ALS/PDC was seen in Umatac, PDC was also observed in a community sample. Among 8 patients with PDC and one with ALS/PDC, 8 had a family history of neurodegenerative disease. Disclosure: Dr. Lee has nothing to disclose. Dr. Tartaglia has nothing to disclose. Dr. Geschwind has received personal compensation for activities with Gerson Lehrman Group, Clinical Advisors, MedaCorp and Esai, Inc. as a consultant and/or speaker. Dr. Perry has nothing to disclose. Dr. Afaisen has nothing to disclose. Dr. Wu has nothing to disclose. Dr. Khan has nothing to disclose. Dr. Steele has nothing to disclose. Dr. Miller has received personal compensation for activities with Allon Therapeutics, Inc. and TauRx Therapeutics, Ltd. Dr. Miller has received research support from Novartis.