Abnormalities of the nucleus basalis in Down's syndrome

Authors

  • Manuel F. Casanova MD,

    1. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD
    2. Neuropathology Laboratory, The Johns Hopkins University School of Medicine, Baltimore, MD
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  • Lary C. Walker PhD,

    1. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD
    2. Neuropathology Laboratory, The Johns Hopkins University School of Medicine, Baltimore, MD
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  • Peter J. Whitehouse MD, PhD,

    1. Department of Neurology and Neuroscience, The Johns Hopkins University School of Medicine, Baltimore, MD
    2. Neuropathology Laboratory, The Johns Hopkins University School of Medicine, Baltimore, MD
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  • Dr. Donald L. Price MD

    Corresponding author
    1. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD
    2. Department of Neurology and Neuroscience, The Johns Hopkins University School of Medicine, Baltimore, MD
    3. Neuropathology Laboratory, The Johns Hopkins University School of Medicine, Baltimore, MD
    • Neuropathology Laboratory, 509 Pathology Building, The Johns Hopkins University School of Medicine, 600 North Wolfe St, Baltimore, MD 21205
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Abstract

One of the most striking manifestations of Down's syndrome is profound mental retardation. Furthermore, after 35 years of age, many patients with Down's syndrome develop clinical and pathological features of Alzheimer's disease. Since brains of patients with Alzheimer's disease show significant loss of neurons in the nucleus basalis of Meynert (nbM), we sought to establish normal standards of nbM neurons in persons with Down's syndrome and to determine whether reductions in the number of neurons occur with increasing age. The number and size of neurons in the nbM were measured in selected sagittal sections from 5 patients with Down's syndrome and 5 age-matched controls. The patients (age range, 16 to 56 years) had 29% fewer nbM neurons than controls, and the oldest patient had the lowest cell count of all subjects. The size of nbM neurons did not differ significantly between the two groups. Our results show that the nbM contains fewer neurons in young persons with Down's syndrome than in normal controls and suggest that the number of these nerve cells may be further reduced in older persons with Down's syndrome.

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