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Alzheimer's Disease: A Family Study

Alzheimer's Disease: A Family Study Abstract ALZHEIMER'S disease remains incompletely defined despite the case reports and clinicopathological surveys of several decades. The etiology of the disorder is unknown. Although several families with a high prevalence of the disease have been reported,1 many other cases appear to be sporadic. Clinically, the most consistent manifestation is progressive dementia beginning during the presenile years. However, dementia is commonly seen in persons with other brain disorders, including nonspecific loss of mental powers associated with aging.2 The diagnosis depends upon demonstration of senile plaques and neurofibrillary changes in brain tissue; but even these alterations, although relatively specific, have been reported with other disease states.3-5 In any case, extensive biopsy or even autopsy series are impractical for large scale clinical or genetic studies of this uncommon disease which affects a relatively inaccessible tissue. This report describes our study of a family, the largest yet reported, in which 19 persons References 1. Feldman, R.G., et al: Familial Alzheimer's Disease , Neurology 13:811-824, 1963.Crossref 2. Allison, R.S.: The Senile Brain: A Clinical Study , London: Edward Arnold, Ltd., 1962. 3. von Braunmühl, A.: " Alterserkrankungen des Zentralnervensystems ," in Lubarsch, O.; Henke, F.; and Rossle, R. (eds.): Handbuch der Speziellen Pathologischen Anatomie und Histologie , Berlin: Springer-Verlag, 1957, vol 13, part 1A, pp 337-539. 4. Hirano, A., and Zimmerman. H.M.: Alzheimer's Neurofibrillary Changes: A Topographic Study , Arch Neurol 7:227-242, 1962.Crossref 5. McMenemey, W.H.: " The Dementias and Progressive Diseases of the Basal Ganglia ," in Blackwood, W., et al (eds.) Greenfield's Neuropathology , ed 2, London: Edward Arnold, Ltd., 1963, pp 520-546. 6. Vassar, P.S., and Culling, C.F.A.: Fluorescent Stains: With Special Reference to Amyloid and Connective Tissues , Arch Path 68:487-498, 1959. 7. van Bogaert, L.; Maere, M.; and de Smedt, E.: Sur les formes familiales précoces de la maladie d'Alzheimer , Mschr Psychiat Neurol 102:249-301, 1940.Crossref 8. Lüers, T.: Űber die familiäre juvenile Form der Alzheimerschen Krankheit mit neurologischen Herderscheinungen , Arch Psychiat Nervenkr 179:132-145, 1947. 9. Jelgersma, H.C.: Ein Fall von juveniler hereditärer Demenz vom Alzheimer Typ mit Parkinsonismus und Klüver-Bucy-Syndrom , Arch Psychiat Nervenkr 205:262-266, 1964. 10. Green, M.A., et al: Cerebral Biopsy in Patients With Presenile Dementia , Dis Nerv Syst 13:303-307, 1952. 11. Sim, M., and Sussman, I.: Alzheimer's Disease: Its Natural History and Differential Diagnosis , J Nerv Ment Dis 135:489-499, 1962.Crossref 12. Margolis, G.: Senile Cerebral Disease: A Critical Survey of Traditional Concepts Based Upon Observations With Newer Techniques , Lab Invest 8:335-370, 1959. 13. Schwartz, P.; Kurucz, J.; and Kurucz, A.: Recent Observations on Senile Cerebral Changes and Their Pathogenesis , J Amer Geriat Soc 12:908-922, 1964. 14. Walford, R.L., and Sjaarda, J.R.: Increase of Thioflavine-T-Staining Material (Amyloid) in Human Tissue With Age , J Geront 19:57-61, 1964.Crossref 15. Haberland, C.: Primary Systemic Amyloidosis: Cerebral Involvement and Senile Plaque Formation , J Neuropath Exp Neurol 23:135-150, 1964.Crossref 16. Krigman, M.R.; Feldman, R.G.; and Bensch, K.: Alzheimer's Presenile Dementia: A Histochemical and Electron Microscopic Study , Lab Invest 14:381-396, 1965. 17. Hirano, A., et al: Parkinsonism-Dementia Complex, an Endemic Disease on the Island of Guam: I. Clinical Features , Brain 84:642-661, 1961.Crossref 18. Hirano, A., et al: Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex on Guam: Further Pathologic Studies , Arch Neurol 15:35-51, 1966.Crossref 19. Goodman, L.: Alzheimer's Disease: A Clinico-Pathologic Analysis of 23 Cases With a Theory on Pathogenesis , J Nerv Ment Dis 117(sic, actually (118) ):97-130, 1953.Crossref 20. Myrianthopoulos, N.C., and Smith, J.K.: Amyotrophic Lateral Sclerosis With Progressive Dementia: And With Pathologic Findings of the Creutzfeld-Jakob Syndrome , Neurology 12:603-610, 1962.Crossref 21. Elizan, T.S., et al: Amyotrophic Lateral Scalerosis and Parkinsonism-Dementia Complex on Guam: Neurological Reevaluation , Arch Neurol 14:356-369, 1966.Crossref 22. Maynard-Smith, S.; Penrose, L.S.; and Smith, C.A.B.: Mathematical Tables for Research Workers in Human Genetics , Boston: Little, Brown and Co., no date (1962?). 23. Sjögren, T.; Sjögren, H.; and Lindgren, A.G.H.: Morbus Alzheimer and Morbus Pick: A Genetic, Clinical and Patho-Anatomical Study , Acta Psychiat Scand , (Suppl 82) , pp 1-152, 1952. 24. Wheelan, L.: Familial Alzheimer's Disease , Ann Hum Genet 23:300-310, 1959.Crossref 25. Constantinidis, J.; Garrone, G.; and de Ajuriaguerra, J.: L'hérédité des démences de l'age avancé Encéphale 51:301-344, 1962. 26. Nahman, S., and Rabinowicz, T.: Considérations sur un cas familial de maladie d'Alzheimer , Encéphale 52:366-381, 1963. 27. Lowenberg, K., and Waggoner, R.W.: Familial Organic Psychosis (Alzheimer's Type) , Arch Neurol Psychiat 31:737-754, 1934.Crossref 28. McMenemy, W.H., et al: Familial Presenile Dementia: Report of Case With Clinical and Pathological Features of Alzheimer's Disease , J Neurol Psychiat 2:293-302, 1939.Crossref 29. Worster-Drought, C.; Greenfield, J.G.; and McMenemy, W.H.: A Form of Familial Presenile Dementia With Spastic Paralysis , Brain 67:38-43, 1944.Crossref 30. Lauter, H.: Genealogische Erhebungen in einer Familie mit Alzheimerscher Krankheit , Arch Psychiat Nervenkr 202:126-139, 1961. 31. Zawuski, G.: Zur Erblichkeit der Alzheimerschen Krankheit , Arch Psychiat Nervenkr 201:123-132, 1960. 32. Davidson, E.A., and Robertson, E.E.: Alzheimer's Disease With Acne Rosacea in One of Identical Twins , J Neurol Neurosurg Psychiat 18:72-77, 1955.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1966 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1966.00470150003001
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Abstract

Abstract ALZHEIMER'S disease remains incompletely defined despite the case reports and clinicopathological surveys of several decades. The etiology of the disorder is unknown. Although several families with a high prevalence of the disease have been reported,1 many other cases appear to be sporadic. Clinically, the most consistent manifestation is progressive dementia beginning during the presenile years. However, dementia is commonly seen in persons with other brain disorders, including nonspecific loss of mental powers associated with aging.2 The diagnosis depends upon demonstration of senile plaques and neurofibrillary changes in brain tissue; but even these alterations, although relatively specific, have been reported with other disease states.3-5 In any case, extensive biopsy or even autopsy series are impractical for large scale clinical or genetic studies of this uncommon disease which affects a relatively inaccessible tissue. This report describes our study of a family, the largest yet reported, in which 19 persons References 1. Feldman, R.G., et al: Familial Alzheimer's Disease , Neurology 13:811-824, 1963.Crossref 2. Allison, R.S.: The Senile Brain: A Clinical Study , London: Edward Arnold, Ltd., 1962. 3. von Braunmühl, A.: " Alterserkrankungen des Zentralnervensystems ," in Lubarsch, O.; Henke, F.; and Rossle, R. (eds.): Handbuch der Speziellen Pathologischen Anatomie und Histologie , Berlin: Springer-Verlag, 1957, vol 13, part 1A, pp 337-539. 4. Hirano, A., and Zimmerman. H.M.: Alzheimer's Neurofibrillary Changes: A Topographic Study , Arch Neurol 7:227-242, 1962.Crossref 5. McMenemey, W.H.: " The Dementias and Progressive Diseases of the Basal Ganglia ," in Blackwood, W., et al (eds.) Greenfield's Neuropathology , ed 2, London: Edward Arnold, Ltd., 1963, pp 520-546. 6. Vassar, P.S., and Culling, C.F.A.: Fluorescent Stains: With Special Reference to Amyloid and Connective Tissues , Arch Path 68:487-498, 1959. 7. van Bogaert, L.; Maere, M.; and de Smedt, E.: Sur les formes familiales précoces de la maladie d'Alzheimer , Mschr Psychiat Neurol 102:249-301, 1940.Crossref 8. Lüers, T.: Űber die familiäre juvenile Form der Alzheimerschen Krankheit mit neurologischen Herderscheinungen , Arch Psychiat Nervenkr 179:132-145, 1947. 9. Jelgersma, H.C.: Ein Fall von juveniler hereditärer Demenz vom Alzheimer Typ mit Parkinsonismus und Klüver-Bucy-Syndrom , Arch Psychiat Nervenkr 205:262-266, 1964. 10. Green, M.A., et al: Cerebral Biopsy in Patients With Presenile Dementia , Dis Nerv Syst 13:303-307, 1952. 11. Sim, M., and Sussman, I.: Alzheimer's Disease: Its Natural History and Differential Diagnosis , J Nerv Ment Dis 135:489-499, 1962.Crossref 12. Margolis, G.: Senile Cerebral Disease: A Critical Survey of Traditional Concepts Based Upon Observations With Newer Techniques , Lab Invest 8:335-370, 1959. 13. Schwartz, P.; Kurucz, J.; and Kurucz, A.: Recent Observations on Senile Cerebral Changes and Their Pathogenesis , J Amer Geriat Soc 12:908-922, 1964. 14. Walford, R.L., and Sjaarda, J.R.: Increase of Thioflavine-T-Staining Material (Amyloid) in Human Tissue With Age , J Geront 19:57-61, 1964.Crossref 15. Haberland, C.: Primary Systemic Amyloidosis: Cerebral Involvement and Senile Plaque Formation , J Neuropath Exp Neurol 23:135-150, 1964.Crossref 16. Krigman, M.R.; Feldman, R.G.; and Bensch, K.: Alzheimer's Presenile Dementia: A Histochemical and Electron Microscopic Study , Lab Invest 14:381-396, 1965. 17. Hirano, A., et al: Parkinsonism-Dementia Complex, an Endemic Disease on the Island of Guam: I. Clinical Features , Brain 84:642-661, 1961.Crossref 18. Hirano, A., et al: Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex on Guam: Further Pathologic Studies , Arch Neurol 15:35-51, 1966.Crossref 19. Goodman, L.: Alzheimer's Disease: A Clinico-Pathologic Analysis of 23 Cases With a Theory on Pathogenesis , J Nerv Ment Dis 117(sic, actually (118) ):97-130, 1953.Crossref 20. Myrianthopoulos, N.C., and Smith, J.K.: Amyotrophic Lateral Sclerosis With Progressive Dementia: And With Pathologic Findings of the Creutzfeld-Jakob Syndrome , Neurology 12:603-610, 1962.Crossref 21. Elizan, T.S., et al: Amyotrophic Lateral Scalerosis and Parkinsonism-Dementia Complex on Guam: Neurological Reevaluation , Arch Neurol 14:356-369, 1966.Crossref 22. Maynard-Smith, S.; Penrose, L.S.; and Smith, C.A.B.: Mathematical Tables for Research Workers in Human Genetics , Boston: Little, Brown and Co., no date (1962?). 23. Sjögren, T.; Sjögren, H.; and Lindgren, A.G.H.: Morbus Alzheimer and Morbus Pick: A Genetic, Clinical and Patho-Anatomical Study , Acta Psychiat Scand , (Suppl 82) , pp 1-152, 1952. 24. Wheelan, L.: Familial Alzheimer's Disease , Ann Hum Genet 23:300-310, 1959.Crossref 25. Constantinidis, J.; Garrone, G.; and de Ajuriaguerra, J.: L'hérédité des démences de l'age avancé Encéphale 51:301-344, 1962. 26. Nahman, S., and Rabinowicz, T.: Considérations sur un cas familial de maladie d'Alzheimer , Encéphale 52:366-381, 1963. 27. Lowenberg, K., and Waggoner, R.W.: Familial Organic Psychosis (Alzheimer's Type) , Arch Neurol Psychiat 31:737-754, 1934.Crossref 28. McMenemy, W.H., et al: Familial Presenile Dementia: Report of Case With Clinical and Pathological Features of Alzheimer's Disease , J Neurol Psychiat 2:293-302, 1939.Crossref 29. Worster-Drought, C.; Greenfield, J.G.; and McMenemy, W.H.: A Form of Familial Presenile Dementia With Spastic Paralysis , Brain 67:38-43, 1944.Crossref 30. Lauter, H.: Genealogische Erhebungen in einer Familie mit Alzheimerscher Krankheit , Arch Psychiat Nervenkr 202:126-139, 1961. 31. Zawuski, G.: Zur Erblichkeit der Alzheimerschen Krankheit , Arch Psychiat Nervenkr 201:123-132, 1960. 32. Davidson, E.A., and Robertson, E.E.: Alzheimer's Disease With Acne Rosacea in One of Identical Twins , J Neurol Neurosurg Psychiat 18:72-77, 1955.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1966

References