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Olfactory dysfunction in Guamanian ALS, parkinsonism, and dementia

From the Departments of Neurology (Drs. Ahlskog and Petersen) and Health Sciences Research (Clinical Epidemiology and Biostatistics; Drs. Waring, Petersen, O'Brien, and Kurland, and M.F. Plevak), Mayo Clinic and Mayo Foundation, Rochester, MN; and The School of Nursing & Allied Health (Drs. Esteban-Santillan and Craig), University of Guam, Mangilao, Guam. Dr. Esteban-Santillan is currently affiliated with the Department of Neurology, Case Western Reserve University, Cleveland, OH.

Address correspondence and reprint requests to Dr. J. Eric Ahlskog, Division of Movement Disorders, Department of Neurology, Mayo Clinic, 200 First St. SW, Rochester, MN 55905.

Objectives: To assess whether olfactory deficits are present in the general Guamanian Chamorro population and to evaluate olfaction in each of the four neurodegenerative disease syndromes of Guam: ALS, pure parkinsonism, pure dementia, and the combined parkinsonism-dementia complex (PDC).

Background: Olfactory dysfunction was previously reported in patients with PDC of Guam.

Methods: We developed a culturally adjusted olfactory test battery, derived from the original University of Pennsylvania Smell Identification Test (UPSIT), and administered this to Chamorro residents with ALS (n = 9), pure parkinsonism (n = 9), pure dementia (n = 11), PDC (n = 31), and 53 neurologically normal Chamorro and 25 North American control subjects.

Results: Similar, marked olfactory dysfunction was found in all four syndromes of Guamanian neurodegenerative disease. This correlated poorly with measures of parkinsonism and cognition. In the neurologically normal Chamorro control group, six subjects (11%) had very low olfactory scores; these were less than the lowest North American score, raising a question of subclinical neurodegenerative disease.

Conclusions: Marked olfactory deficits are common to all four Guamanian neurodegenerative syndromes, and suggest the possibility of similar central neuropathologic substrates. The deficit in the Guamanian ALS group contrasts with idiopathic ALS, in which olfactory function has been reported to be only slightly compromised.

Supported by grants AG-08802, AG-08031, AG-06786, and M01-RR00585 from the National Institutes of Health, Bethesda, MD, and by a grant from the government of Guam.

Received February 26, 1998. Accepted in final form August 21, 1998.

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