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Alzheimer Degeneration in Down Syndrome: Electrophysiologic Alterations and Histopathologic Findings

Alzheimer Degeneration in Down Syndrome: Electrophysiologic Alterations and Histopathologic Findings Abstract • Progressive electroencephalographic disorganization and decreased voltage amplitude in the late components of the averaged visual evoked potentials were recorded in the last two years of life of a patient with Down disease and Alzheimer degeneration. Taken together with quantitative histopathologic findings, the electrophysiologic alterations are interpreted in terms of recent evidence from an experimental animal model of dementia. Neurons with neurofibrillary degeneration become electrically inactive and contribute to the loss of voltage generators associated with neuron death in Alzheimer disease. Loss of voltage generators may result in disfacilitation and disinhibition of surviving neurons, thus causing the loss of normal rhythms. References 1. Schochet SS, Lampert PW, McCormick WF: Neurofibrillary tangles in patients with Down's syndrome: A light and electron microscopic study . Acta Neuropathol 23:342-346, 1973.Crossref 2. Ellis WG, McCulloch JR, Corley CL: Presenile dementia in Down's syndrome: Ultrastructural identity with Alzheimer's disease . Neurology 24:101-106, 1974.Crossref 3. Malamud N: Neuropathology of organic brain syndromes associated with aging , in Gaitz CM (ed): Advances in Behavioral Biology . New York, Plenum Press Inc, 1972, vol 3, pp 63-87. 4. Owens D, Dawson JC, Losin S: Alzheimer's disease in Down's syndrome . Am J Ment Defic 75:606-612, 1971. 5. Dalton AJ, Crapper DR, Schlotterer GR: Alzheimer's disease in Down's syndrome: Visual retention deficits . Cortex 10:366-377, 1974.Crossref 6. Dalton AJ, Crapper DR, Schlotterer GR: Short-term retention deficits in Down's syndrome and Alzheimer's disease. Read before the 98th annual meeting of the American Association on Mental Deficiency, Toronto, 1974. 7. Ellingson RJ, Eisen JD, Ottersberg G: Clinical electroencephalographic observations on institutionalized mongoloids confirmed by karyotype . Electroencephalogr Clin Neurophysiol 34:193-196, 1973.Crossref 8. Coblentz JM, Mattis S, Zingesser LH, et al: Presenile dementia: Clinical aspects and evaluation of cerebrospinal fluid dynamics . Arch Neurol 29:299-308, 1973.Crossref 9. Crapper DR, Krishnan SS, Dalton AJ: Brain aluminum distribution in Alzheimer's disease and experimental neurofibrillary degeneration . Science 180:511-513, 1973.Crossref 10. Bergland GD: A guided tour of the fast Fourier transform . IEEE Spectrum 6:41-52, 1969.Crossref 11. Abercrombie M: Estimation of nuclear population from microtome sections . Anat Rec 94:239-247, 1946.Crossref 12. Wilson HR, Cowan JD: A mathematic theory of the functional dynamics of cortical and thalamic nervous tissue . Kybernetik 13:55-80, 1973.Crossref 13. Colon EJ: The cerebral cortex in presenile dementia: A quantitative analysis . Acta Neuropathol 23:281-290, 1973.Crossref 14. Konigsmark BW: Methods for counting neurons , in Nauta WJH, Ebbesson SOE (eds): Contemporary Research Methods in Neuroanatomy . New York, Springer-Verlag, 1970, pp 315-380. 15. Pakkenberg H: The number of nerve cells in the cerebral cortex of man . J Comp Neurol 128:17-20, 1966.Crossref 16. Sholl DA: A comparative study of the neuronal packing density in the cerebral cortex . J Anat 93:143-158, 1959. 17. Dayon AD: Quantitative histological studies on the aged human brain: II. Senile plaques and neurofibrillary tangles in senile dementia . Acta Neuropathol 16:95-102, 1970.Crossref 18. Siegel S: Nonparametric Statistics for the Behavioral Sciences . New York, McGraw-Hill Book Co Inc, 1956. 19. Ferguson GA: Statistical Analysis in Psychology and Education , ed 2. New York, McGraw-Hill Book Co Inc, 1959. 20. Letemendia F, Pampiglione G: Clinical and electroencephalographic observations in Alzheimer's disease . J Neurol Neurosurg Psychiatry 21:167-172, 1958.Crossref 21. Andersen P, Andersson SA: Physiological Basis of the Alpha Rhythm . New York, Appleton-Century-Crofts Inc, 1968. 22. Terry RD, Gonatas NK, Weiss M: Ultra-structural studies in Alzheimer's presenile dementia . Am J Pathol 44:269-297, 1964. 23. Crapper DR: Experimental neurofibrillary degeneration and altered electrical activity . Electroencephalogr Clin Neurophysiol 35:575-588, 1973.Crossref 24. Crapper DR, Tomko GJ: Neuronal correlates of an experimental dementia induced by aluminum neurofibrillary degeneration . Brain Res , to be published. 25. Crapper DR: Dementia: Recent observations on Alzheimer's disease and experimental aluminum encephalopathy , in Seeman P, Brown G (eds): Frontiers in Neurology and Neuroscience Research: Symposium of the Neuroscience Institute of the University of Toronto . Toronto, University of Toronto Press, 1974, pp 97-111. 26. Le Vay S: Synaptic patterns in the visual cortex of the cat and monkey: Electron microscopy of Golgi preparations . J Comp Neurol 150:53-86, 1973.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Alzheimer Degeneration in Down Syndrome: Electrophysiologic Alterations and Histopathologic Findings

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Publisher
American Medical Association
Copyright
Copyright © 1975 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1975.00490510074006
Publisher site
See Article on Publisher Site

Abstract

Abstract • Progressive electroencephalographic disorganization and decreased voltage amplitude in the late components of the averaged visual evoked potentials were recorded in the last two years of life of a patient with Down disease and Alzheimer degeneration. Taken together with quantitative histopathologic findings, the electrophysiologic alterations are interpreted in terms of recent evidence from an experimental animal model of dementia. Neurons with neurofibrillary degeneration become electrically inactive and contribute to the loss of voltage generators associated with neuron death in Alzheimer disease. Loss of voltage generators may result in disfacilitation and disinhibition of surviving neurons, thus causing the loss of normal rhythms. References 1. Schochet SS, Lampert PW, McCormick WF: Neurofibrillary tangles in patients with Down's syndrome: A light and electron microscopic study . Acta Neuropathol 23:342-346, 1973.Crossref 2. Ellis WG, McCulloch JR, Corley CL: Presenile dementia in Down's syndrome: Ultrastructural identity with Alzheimer's disease . Neurology 24:101-106, 1974.Crossref 3. Malamud N: Neuropathology of organic brain syndromes associated with aging , in Gaitz CM (ed): Advances in Behavioral Biology . New York, Plenum Press Inc, 1972, vol 3, pp 63-87. 4. Owens D, Dawson JC, Losin S: Alzheimer's disease in Down's syndrome . Am J Ment Defic 75:606-612, 1971. 5. Dalton AJ, Crapper DR, Schlotterer GR: Alzheimer's disease in Down's syndrome: Visual retention deficits . Cortex 10:366-377, 1974.Crossref 6. Dalton AJ, Crapper DR, Schlotterer GR: Short-term retention deficits in Down's syndrome and Alzheimer's disease. Read before the 98th annual meeting of the American Association on Mental Deficiency, Toronto, 1974. 7. Ellingson RJ, Eisen JD, Ottersberg G: Clinical electroencephalographic observations on institutionalized mongoloids confirmed by karyotype . Electroencephalogr Clin Neurophysiol 34:193-196, 1973.Crossref 8. Coblentz JM, Mattis S, Zingesser LH, et al: Presenile dementia: Clinical aspects and evaluation of cerebrospinal fluid dynamics . Arch Neurol 29:299-308, 1973.Crossref 9. Crapper DR, Krishnan SS, Dalton AJ: Brain aluminum distribution in Alzheimer's disease and experimental neurofibrillary degeneration . Science 180:511-513, 1973.Crossref 10. Bergland GD: A guided tour of the fast Fourier transform . IEEE Spectrum 6:41-52, 1969.Crossref 11. Abercrombie M: Estimation of nuclear population from microtome sections . Anat Rec 94:239-247, 1946.Crossref 12. Wilson HR, Cowan JD: A mathematic theory of the functional dynamics of cortical and thalamic nervous tissue . Kybernetik 13:55-80, 1973.Crossref 13. Colon EJ: The cerebral cortex in presenile dementia: A quantitative analysis . Acta Neuropathol 23:281-290, 1973.Crossref 14. Konigsmark BW: Methods for counting neurons , in Nauta WJH, Ebbesson SOE (eds): Contemporary Research Methods in Neuroanatomy . New York, Springer-Verlag, 1970, pp 315-380. 15. Pakkenberg H: The number of nerve cells in the cerebral cortex of man . J Comp Neurol 128:17-20, 1966.Crossref 16. Sholl DA: A comparative study of the neuronal packing density in the cerebral cortex . J Anat 93:143-158, 1959. 17. Dayon AD: Quantitative histological studies on the aged human brain: II. Senile plaques and neurofibrillary tangles in senile dementia . Acta Neuropathol 16:95-102, 1970.Crossref 18. Siegel S: Nonparametric Statistics for the Behavioral Sciences . New York, McGraw-Hill Book Co Inc, 1956. 19. Ferguson GA: Statistical Analysis in Psychology and Education , ed 2. New York, McGraw-Hill Book Co Inc, 1959. 20. Letemendia F, Pampiglione G: Clinical and electroencephalographic observations in Alzheimer's disease . J Neurol Neurosurg Psychiatry 21:167-172, 1958.Crossref 21. Andersen P, Andersson SA: Physiological Basis of the Alpha Rhythm . New York, Appleton-Century-Crofts Inc, 1968. 22. Terry RD, Gonatas NK, Weiss M: Ultra-structural studies in Alzheimer's presenile dementia . Am J Pathol 44:269-297, 1964. 23. Crapper DR: Experimental neurofibrillary degeneration and altered electrical activity . Electroencephalogr Clin Neurophysiol 35:575-588, 1973.Crossref 24. Crapper DR, Tomko GJ: Neuronal correlates of an experimental dementia induced by aluminum neurofibrillary degeneration . Brain Res , to be published. 25. Crapper DR: Dementia: Recent observations on Alzheimer's disease and experimental aluminum encephalopathy , in Seeman P, Brown G (eds): Frontiers in Neurology and Neuroscience Research: Symposium of the Neuroscience Institute of the University of Toronto . Toronto, University of Toronto Press, 1974, pp 97-111. 26. Le Vay S: Synaptic patterns in the visual cortex of the cat and monkey: Electron microscopy of Golgi preparations . J Comp Neurol 150:53-86, 1973.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1975

References