Summary
A family is described in which for three subsequent generations numerous individuals were affected with a progressive neuropsychiatric disease with pyramidal, bulbar and cerebellar symptoms, relapsing course and gradually evolving severe dementia.
Post-mortem studies performed on three siblings afflicted with the disease suggest that the remarkably uniform macroscopic picture of the cerebral changes consisting in multiple small cystic infarctions, particularly localized to the central grey and white matter and pons as well as the cortical and central brain atrophy, is caused by an occlusive disease of small intracerebral and leptomeningeal arteries and arterioles. Collected pertinent information concerning the affected family members shows that the illness begins in early adulthood (at 29–38 years of age), affects both sexes and generally lasts for 10–15 years. The only exception so far noticed was a second generation descendant of one of the siblings. This patient died about 5 months after clinical onset of the disease in masslve cerebral haemorrhage and showed similar vascular changes as the older members of the family. The disease is considered to be genetically caused and transmitted as a dominant autosomal character. For this apparently new nosological entity the eponym “hereditary multi-infarct dementia” is suggested.
Similar content being viewed by others
References
Adams, C. W. M.: Neurohistochemistry. Elsevier: Amsterdam 1965
Åkesson, H. O.: A population study of senile and arteriosclerotic psychoses. Human Heredity19, 546–566 (1969)
Arnason, A.: Apoplexie und ihre Vererbung. Acta Psychiat. neurol. Suppl.VII (1935)
Essen-Möller, E.: A family with Alzheimer's disease. Acta Psychiat. Neurol. Scand.21, 233–244 (1946)
Gardner, D. L.: Pathology of Connective Tissue Disease. Edward Arnold London (1965)
Gudmundsson, G., Hallgrimsson, J., Jonasson, T. A., Bjarnason, O.: Hereditary cerebral haemorrhage with amyloidosis. Brain95, 387–404 (1972)
Haschinski, V. C., Lassen, N. A., Marshall, J.: Multi-infarct dementia. A cause of mental deterioration in the elderly. Lancet, July 27, 207–210 (1974)
Heston, L. L., Lowther, D. L. W., Leventhal, C. M.: Alzheimer's disease. Arch. Neurol.15, 225–233 (1966)
Jellinek, H., ed.: Arterial Lesions and Arteriosclerosis. London and New York: Plenum Press 1974
Jellinger, K.: Cerebrovascular amyloidosis with cerebral haemorrhage. J. Neurol.214, 195–206 (1977)
Jörgensen, L., Torvik, A.: Ischaemic Cerebrovascular Diseases in an Autopsy Series. Part 1. Prevalence, Location and Predisposing Factors in Verified Thrombo-embolic Occlusions, and their Significance in the Pathogenesis of Cerebral Infarction. J. Neurol. Sci.3, 490–509 (1966)
Maeda, S., Nakayama, H., Isaka, K., Aihara, Y., Nemoto, S.: Familial unusual encephalopathy of Binswanger's type without hypertension. Folia Psychiat. Neurol. Jap.30, 164–177 (1976)
Mayer-Gross, W., Slater, E., Roth, M.: Clinical Psychiatry (Ed. E. Slater and M. Roth), London: Baillière, Tindall and Cassell 1969
McMenemey, W. H.: The dementias and progressive diseases of the basal ganglia. In: Greenfield's Neuropathology, pp. 520–576 (Eds. W. Blackwood, W. H. McMenemey, A. Meyer, R. M. Norman and D. S. Russell). London: Edward Arnold 1963
Paulsson, G. V., Perrine, G., Jr.: Cerebral vascular disease in mental hospitals. In: Cerebral Vascular Diseases. Sixth Conference. Eds. J. F. Toole, R. G. Siekert and J. P. Whisnant. New York: Grune and Stratton 1968
Pratt, R. T. C.: The genetics of Neurological Disorders. London: Oxford University Press 1967
Torvik, A., Endresen, G. K. M., Abrahamsen, A. F., Godal, H. C.: Progressive dementia caused by an unusual type of generalized small vessel thrombosis. Acta Neurol. Scandinav.47, 137–150 (1971)
Worm-Petersen, J., Pakkenberg, H.: Atherosclerosis of cerebral arteries, pathological and clinical correlation. J. Gerontol.23, 445 (1968)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Sourander, P., Walinder, J. Hereditary multi-infarct dementia. Acta Neuropathol 39, 247–254 (1977). https://doi.org/10.1007/BF00691704
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00691704