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The Lewy-Body Variant of Alzheimer's Disease

Clinical and Pathological Findings

Published online by Cambridge University Press:  02 January 2018

Hans Förstl*
Affiliation:
Section of Old Age Psychiatry, Institute of Psychiatry, London SE5 8AF
Alistair Burns
Affiliation:
Section of Old Age Psychiatry, Institute of Psychiatry, London SE5 8AF
Phil Luthert
Affiliation:
Section of Old Age Psychiatry, Institute of Psychiatry, London SE5 8AF
Nigel Cairns
Affiliation:
Section of Old Age Psychiatry, Institute of Psychiatry, London SE5 8AF
Raymond Levy
Affiliation:
Department of Neuropathology, Institute of Psychiatry
*
Central Institute of Mental Health, PO Box 122120, JS, W6800 Mannheim, Germany

Abstract

At post-mortem, Lewy bodies (LBs) were found in the brainstem and neocortex of eight out of 65 patients who had been collected during a prospective long-term study on clinically diagnosed Alzheimers disease. All eight patients had accompanying Alzheimer pathology which was less severe than in a sample of eight age and sex-matched patients from the same study with neuropathologically verified Alzheimer's disease. Parkinsonian features were more common in patients with LBs. There were no particular differences in duration of illness, severity of cognitive impairment, presence of hallucinations, or fluctuations in the course of illness. Frontal cerebral atrophy was more marked in patients with LBs, as was the loss of neurons in the basal nucleus of Meynert and the substantia nigra. Cognitive performance correlated with the number of pigmented neurons in the substantia nigra. We conclude that the differential diagnosis of LB dementia should be considered in patients satisfying NINCDS-ADRDA criteria for Alzheimer-type dementia who show marked Parkinsonian features and a frontal accentuation of cerebral atrophy.

Type
Research Article
Copyright
Copyright © The Royal College of Psychiatrists 

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References

Aherne, W. A. & Dunnill, M. S. (1988) Morphometry. London: Edward Arnold.Google Scholar
Bergeron, C. & Pollanen, M. (1989) Lewy bodies in Alzheimer disease - one or two diseases? Alzheimer Disease and Associated Disorders, 3, 197204.CrossRefGoogle ScholarPubMed
Burkhardt, C. R., Filley, C. M., Kleinschmidt-DeMasters, B. K., et al (1988) Diffuse Lewy body disease and progressive dementia. Neurology, 38, 15201528.Google Scholar
Burns, A., Jacoby, R. & Levy, R. (1990a) Psychiatric phenomena in Alzheimer's disease. British Journal of Psychiatry, 157, 7294.CrossRefGoogle ScholarPubMed
Burns, A., Luthert, P., Levy, R., et al (1990b) Accuracy of clinical diagnosis of Alzheimers disease. British Medical Journal, 301, 1026.Google Scholar
Byrne, E. J., Lennox, G., Lowe, J., et al (1989) Diffuse Lewy body disease: clinical features in 15 cases. Journal of Neurology, Neurosurgery and Psychiatry, 52, 709717.Google Scholar
Clark, A. W., White, C. L. III, Manz, H. J., et al (1986) Primary degenerative dementia without Alzheimer pathology. Canadian Journal of the Neurological Sciences, 13, 462470.Google Scholar
Crystal, H. A., Dickson, D. W., Lizardi, J. E., et aI (1990) Antemortem diagnosis of diffuse Lewy body dementia. Neurology, 40, 15231528.Google Scholar
Dickson, D. W., Davies, P., Mayeux, R., et al (1987) Diffuse Lewy body disease: neuropathological and biochemical studies of six patients. Acta Neuropathologica, 75, 815.CrossRefGoogle ScholarPubMed
Dickson, D. W., Crystal, H., Mattiace, L. H., et al (1989) Diffuse Lewy body disease: light and electron microscopic immunocytochemistry of senile plaques. Acta Neuropathologica, 78, 572584.CrossRefGoogle ScholarPubMed
Egoertson, D. E. & Sima, A. A. F. (1986) Dementia with cerebral Lewy bodies: a mesocortical dopaminergic deficit? Archives of Neurology, 43, 524527.CrossRefGoogle Scholar
Folstein, M. F., Folstein, S. E. & McHugh, P. R. (1975) MiniMental State: a practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research, 12, 189198.Google Scholar
Forno, L. S. (1969) Concentric hyalin intraneuronal inclusions of Lewy type in the brains of elderly persons (50 incidental cases): relationship to parkinsonism. Journal of the American Geriatric Association, 17, 557575.CrossRefGoogle ScholarPubMed
Förstl, H., Burns, A., Jacoby, R., et al (1991) Quantitative CT scan analysis in senile dementia of the Alzheimer type: I. Computerized planimetry of cerebrospinal fluid areas. International Journal of Geriatric Psychiatry, 6, 709713.Google Scholar
Förstl, H., & Levy, R. (1992) F. H. Lewy on Lewy bodies, parkinsonism and dementia. International Journal of Geriatric Psychiatry, 6, 757766.Google Scholar
Förstl, H., Burns, A., Levy, R., et al (1992) Neurologic signs in Alzheimer's disease: results of a prospective and neuropathologic study. Archives of Neurology, 49, 10381042.Google Scholar
Gibb, W. R. G., Esiri, M. M. & Lees, A. J. (1985) Clinical and pathological features of diffuse cortical Lewy body disease (Lewy body dementia). Brain, 110, 11311153.CrossRefGoogle Scholar
Gibb, W. R. G., Luthert, P. J., Janota, I., et al (1989a) Cortical Lewy body dementia: clinical features and classification. Journal of Neurology, Neurosurgery and Psychiatry, 52, 185192.CrossRefGoogle ScholarPubMed
Gibb, W. R. G., Mountjoy, C. Q., Mann, D. M. A., et al (1989b) A pathological study of the association between Lewy body disease and Alzheimer's disease. Journal of Neurology, Neurosurgery and Psychiatry, 52, 701708.Google Scholar
Hansen, L., Salmon, D., Galasko, D., et al (1990) The Lewy body variant of Alzheimer's disease: a clinical and pathologic entity. Neurology, 40, 18.Google Scholar
Heilig, C. W., Knopman, D. S., Mastri, A. R., et al (1985) Dementia without Alzheimer pathology. Neurology, 35, 762765.CrossRefGoogle ScholarPubMed
Joachim, C. L., Morris, J. H. & Selkoe, D. J. (1988) Clinically diagnosed Alzheimers disease: autopsy results in 150 cases. Annals of Neurology, 24, 5056.CrossRefGoogle ScholarPubMed
Kosaka, K., Tsuchiya, K. & Yoshimura, M. (1988) Lewy-body disease with and without dementia: a clinicopathological study of 35 cases. Clinical Neuropathology, 7, 299305.Google Scholar
Lennox, G., Lowe, R., Landon, M., et al (1989) Anti-Ubiquitin immunocytochemistry is more sensitive than conventional techniques in the detection of diffuse Lewy body disease. Journal of Neurology, Neurosurgery and Psychiatry, 52, 12361247.CrossRefGoogle ScholarPubMed
Lewy, F. H. (1912) Paralysis agitans. I. Pathologische Anatomie. In Handbuch der Neurologie (ed. Lewandowsky, M.), Vol. 3/II, pp. 920933. Berlin: Julius Springer.Google Scholar
McKhann, G., Drachman, D., Folstein, M., et al (1984) Clinical diagnosis of Alzheimers disease: report of the NINCDS-ADRDA work group under the auspices of the Department of Health and Human Services Task Force on Alzheimer's disease. Neurology, 34, 939944.Google Scholar
Okazaki, H., Lipkin, L. E. & Aronson, S. M. (1961) Diffuse intracytoplasmatic ganglionic inclusions (Lewy type) associated with progressive dementia and quadriparesis in flexion. Journal of Neuropathology and Experimental Neurology, 20, 237244.CrossRefGoogle Scholar
Perry, R. H., Irving, D., Blessed, G., et al (1990) Senile dementia of the Lewy body type: a clinically and neuropathologically distinct form of Lewy body dementia in the elderly. Journal of the Neurological Sciences, 95, 119139.Google Scholar
Roth, M., Tym, E., Mountjoy, C., et al (1986) CAMDEX: a standardised instrument for the diagnosis of mental disorder in the elderly with special reference to the early detection of dementia. British Journal of Psychiatry, 149, 698709.Google Scholar
Sima, A. A. F., Clark, A. W., Sternberger, N. A., et al (1986) Lewy body dementia without Alzheimer changes. Canadian Journal of Neurological Sciences, 13, 490497.CrossRefGoogle ScholarPubMed
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