NEUROLOGY 1985;35:957
© 1985 American Academy of Neurology
Alzheimer's disease in Down's syndrome
Clinicopathologic studies
K. E. Wisniewski, MD, PhD,
A. J. Dalton, PhD,
D. R. Crapper McLachlan, MD,
G. Y. Wen, PhD and
H. M. Wisniewski, MD, PhD
New York State Office of Mental Retardation and Developmental Disabilities, Institute for Basic Resrarch in Developmental Disabilities, Staten Island, NY (Drs Wisiniewski, Wen, and H.M. Wisniewski); Surrey Place Center (Drs. Dalton and Crapper McLachlan). and the University of Toronto (Dr. Crapper McLachlan). toronto. Ontario, Candada.
Clinical and neuropathologic evidence points to the development of Alzheimer's disease (AD) in seven Down's syndrome patients above age 40. Dementia was observed in these patients over periods of 2.5 to 9.2 years. The first clinical sign of AD, visual memory loss, was succeeded by impaired learning capacity and decreased occupational and social functioning, and culminated in seizures and urinary incontinence. The morphometric observations of the brains of these seven patients with AD showed that the numbers of plaques and tangles exceeded 20 per 1.5 x 106 µm2 area, in both the prefrontal and hippocampal cortices. Plaques and tangles were also evident in the basal ganglia, thalamus, hypothalamus, and midbrain. In addition, we found that four of the seven brains showed small strokes, and five of the seven amyloid angiopathy. This study also indicates that by longitudinal neuropsychological evaluations and lab tests, which exclude other causes of dementia, the diagnosis of AD can be made even in severely and profoundly retarded patients.
Address correspondence and reprint requests to Dr. K.E. Wisniewski, Institute for Basic Research in Developmental Disabilities. 1050 Forest Hill Road. Staten Island. NY 10314.
Accepted for publication October 18, 1984.
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