Journal of Neurology

, Volume 229, Issue 1, pp 17–32 | Cite as

Cortical changes in the parkinsonian brain: a contribution to the delineation of “diffuse Lewy body disease”

  • M. Yoshimura
Original Investigations

Summary

A group of 55 autopsy cases of paralysis agitans were studied with special attention to distribution of Lewy bodies and cortical changes. According to the frequency of Lewy bodies and senile changes (senile plaques and Alzheimer neurofibrillary tangles), three groups could be distinguished.

Group A (4 cases; 3 males, 1 female; aged 65–76 years) with progressive dementia and muscle rigidity showed numerous Lewy bodies, not only in the diencephalon and brain-stem but also in the cerebral cortex, and a high incidence of senile changes. The pattern of distribution of Lewy bodies in the brain-stem was identical to that of Parkinson's disease. Cerebral Lewy bodies were observed predominantly in small or medium-sized pyramidal neurons in the fifth and sixth layers of the temporal, frontal, insular and cingulate cortex. These cases are termed “diffuse Lewy body disease”.

Group B (15 cases; 10 males, 5 females; aged 62–79 years) with classical parkinsonism showed many Lewy bodies in the diencephalon and brain-stem, but cerebral Lewy bodies and senile changes were less frequent than in group A. In group B, the frequency of cerebral Lewy bodies and senile changes was higher in patients with dementia (7 cases, 40%) than in patients without dementia. The demented patients in this group may form a transition between groups B and A.

Group C (36 cases; 17 males, 19 females; aged 52–85 years) without dementia, showed fewer Lewy bodies in the diencephalon and brain-stem than in groups A and B; cerebral Lewy bodies were not found. The frequency of senile changes corresponded to age. Clinicopathologically, the cases in groups B and C correspond to classical paralysis agitans.

Key words

Diffuse Lewy body disease Parkinsonism Lewy bodies Senile changes 

Zusammenfassung

55 Fälle mit histopathologischer Diagnose „Paralysis agitans (PA)” wurden neuropathologisch untersucht; nach Häufigkeit und Verteilung der Lewy-Körperchen sowie seniler Veränderungen wurde 3 Gruppen unterschieden. Gruppe A: 4 Fälle von hochgradiger, progressiver Demenz mit Muskelrigor zeigen neben ausgeprägten senilen Veränderungen zahlreich Lewy-Körperchen in corticalen Nervenzellen. Die Verteilung der Lewy-Körperchen in Zwischenhirn und Hirnstamm entsprach der klassischen PA. Diese Fälle bezeichnen wir als „diffuse Lewy-Körperchen-Krankheit“. Gruppe B: 15 Fälle mit Parkinson-Syndrom und leichter bis mäßiger Demenz (in 40% der Fälle) zeigten nur einzelne corticale Lewy-Körperchen bei zahlreicheren Lewy-Körperchen in Zwischenhirn und Hirnstamm und geringeren senilen Veränderungen als bei Gruppe A. Die Fälle mit Demenz bei Gruppe B könnten als Übergangsfälle zur Gruppe A angesehen werden. Gruppe C: 36 Fälle mit Parkinson-Syndrom ohne Demenz zeigten das Vorkommen von Lewy-Körperchen mit etwas geringerer Häufigkeit als bei Gruppe A und B im Zwischenhirn und Hirnstamm, jedoch nicht in der Hirnrinde. Im klinisch-pathologischen Aspekt entsprachen die Gruppen B und C der klassischen PA.

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Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • M. Yoshimura
    • 1
    • 2
  1. 1.Neurological Institute of the University of ViennaAustria
  2. 2.Department of PathologyTokyo Metropolitan Geriatric HospitalTokyoJapan

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