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Distribution of cerebral degeneration in Alzheimer's disease

A clinico-pathological study

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Summary

Seven cases of Alzheimer's disease were studied in detail from a clinical and neuropathological point of view. The degenerative process was mapped with regard to regional variations in the intensity, extent and consistency of focal accentuations.

The degeneration was regularly found to be most pronounced in certain areas: maximal cortical degeneration occurred in the medial temporal (limbic) area and, in the lateral hemisphere, consistently within a field expanding from the posterior inferior temporal areas to the adjoining portions of the parieto-occipital lobes. In addition, the posterior cingulate gyrus was severely involved. On the other hand certain areas were notably and consistently spared or less involved, mainly the anterior cingulate gyrus and the calcarine and central sensory motor areas (primary projection areas). The frontal lobes occupied an intermediate position, being less severely involved than is usually reported.

The clinical symptoms correlated well with this pattern of degeneration. Thus groups of symptoms such as memory dysfunction, emotional and personality alterations, and some symptoms of the Klüver-Bucy syndrome, were referable to the limbic lesions.

The cortical lesions of the temporo-parieto-occipital association cortex correlated with the symptoms of agnosia, aphasia and apraxia, which were recorded in all cases.

The relative sparing of the primary projection areas correspond well to findings of retained motility and perception even in later stages of the disease. The relative sparing of the frontal lobes and the anterior cingulate gyrus was related to the preservation of habitual personality traits.

The pattern described may be related to ontogenetic features, and the tendency to focalization to the age of disease onset. The role of genetic factors and of other diseases is discussed.

Zusammenfassung

Sieben Fälle von Morbus Alzheimer wurden klinisch und neuropathologisch gründlich untersucht. Der degenerative Prozeß wurde auf seine regionalen Unterschiede der Intensität, Ausdehnung und Konstanz seiner fokalen Akzentuierung analysiert.

Die Degeneration war regelmäßig in bestimmten Arealen besonders betont. Im limbischen System des Allocortex vorwiegend medial temporal im Ammonshorn und hinteren Gyrus cinguli, in der lateralen Hemisphäre, im hinteren temporo-parieto-occipitalen Isocortex. Andererseits waren manche Regionen konstant ausgespart oder weniger betroffen, vor allem der anteriore Gyrus cinguli, die Calcarina und zentrale Teile des sensomotorischen Cortex. Die Degeneration des meist weniger stark betroffenen Frontallappens nahm eine Mittelstellung ein.

Die Krankheitssymptome korrelieren gut mit diesem Degenerationsmuster. Die vorwiegend limbischen Läsionen sind Korrelate von Gedächtnisstörungen, emotionalen und Persönlichkeitsveränderungen und Teilsymptomen eines Klüver-Bucy-Syndroms. Die Läsionen im temporo-parieto-occipitalen Assoziationscortex entsprechen bei allen Fällen den Symptomen von Agnosie, Aphasie und Apraxie.

Die relative Aussparung der primären Projektionsareale korreliert mit den geringen oder fehlenden motorisch-sensiblen Störungen sogar in späten Krankheitsstadien. Das relativ geringe Betroffensein der Frontallappen und des vorderen Gyrus cinguli entspricht wahrscheinlich dem relativen Erhaltenbleiben von Habitus, Verhaltensweisen und Persönlichkeitszügen.

Ontogenetische Faktoren der beschriebenen regionalen Degenerationsmuster, Korrelationen mit dem Alter des Krankheitsbeginns und die Bedeutung genetischer Faktoren werden diskutiert.

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  1. Departments of Pathology and Psychiatry, University Hospital, S-221 85, Lund, Sweden

    A. Brun & L. Gustafson

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  1. A. Brun
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  2. L. Gustafson
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Supported by the Swedish Medical Research Council, projects 12-2037, 25-3950 and 61-4568.

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Brun, A., Gustafson, L. Distribution of cerebral degeneration in Alzheimer's disease. Arch. Psychiat. Nervenkr. 223, 15–33 (1976). https://doi.org/10.1007/BF00367450

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