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NEUROLOGY 1988;38:938
© 1988 American Academy of Neurology

Dementia in Down's syndrome

Cerebral glucose utilization, neuropsychological assessment, and neuropathology

M. B. Schapiro, MD, M. J. Ball, MD, FRCP(C), C. L. Grady, PhD, J. V. Haxby, PhD, J. A. Kaye, MD and S. I. Rapoport, MD

From the Laboratory of Neurosciences, Section on Brain Aging and Dementia (Drs. Schapiro, Grady, Haxby, Kaye, and Rapoport), National Institute on Aging, Clinical Center, Bethesda, MD; and the Department of Pathology, Clinical Neurological Sciences and Psychiatry (Dr. Ball), University of Western Ontario, London, ON, Canada.

We measured the cerebral metabolic rate for glucose (CMRglc) with positron emission tomography and [18F]2-fluoro-2-deoxy-D-glucose in a 47-year-old man with trisomy 21 Down's syndrome (DS) and autopsy-confirmed Alzheimer's disease. Dementia was evident from a confirmed history of cognitive decline, memory loss, and personality change. CMRglc in the subject was compared with the mean obtained in 13 healthy younger DS subjects, aged 19 to 33 years. Test scores of general intelligence, visuospatial ability, language, and memory function showed poorer performance in the older subject compared with the younger group. Mean hemispheric CMRglc in the older DS subject was 28% less than in the young DS group, and marked hypometabolism was evident in parietal and temporal lobe association cortices. At autopsy, extensive neuropathology was noted, especially in the parietal and temporal cortical regions, more so than reported in DS subjects without documented dementia. This study is the first complete assessment of cerebral metabolism, neuropsychological competence, and neuropathology in a DS subject with a documented course of dementia, and demonstrates the superimposition of Alzheimer type dementia on previous mental retardation.

Address correspondence and reprint requests to Dr. Schapiro, Laboratory of Neurosciences, National Institute on Aging, Bldg. 10, Rm. 12S207, Bethesda, MD 20892.

Supported in part by grants from the National Institute on Aging (2R01AGNS03047 04A1), and the Medical Research Council of Canada (PG21).

Received October 20, 1986. Accepted for publication in final form October 9, 1987.




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S. J. Teipel, G. E. Alexander, M. B. Schapiro, H.-J. Moller, S. I. Rapoport, and H. Hampel
Age-related cortical grey matter reductions in non-demented Down's syndrome adults determined by MRI with voxel-based morphometry
Brain, April 1, 2004; 127(4): 811 - 824.
[Abstract] [Full Text] [PDF]


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