CNS involvement in malignant atrophic papulosis (Kohlmeier-Degos disease): vasculopathy and coagulopathy
The clinical features in a 42 year old man with malignant atrophic papulosis with CNS involvement are described. They included mental dysfunction, paraesthesiae, weakness of left limbs, with pyramidal tract signs, bilateral ptosis, progressing to total ophthalmoplegia and obtundation. There were two...
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Published in | Journal of neurology, neurosurgery and psychiatry Vol. 44; no. 2; pp. 156 - 160 |
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Main Authors | , , |
Format | Journal Article |
Language | English |
Published |
England
BMJ Publishing Group Ltd
01.02.1981
BMJ Publishing Group LTD |
Subjects | |
Online Access | Get full text |
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Summary: | The clinical features in a 42 year old man with malignant atrophic papulosis with CNS involvement are described. They included mental dysfunction, paraesthesiae, weakness of left limbs, with pyramidal tract signs, bilateral ptosis, progressing to total ophthalmoplegia and obtundation. There were two crops of characteristic papular lesions on trunk and limbs, with terminal evidence of a coagulopathy and bruises, resulting in death in six months. Papular biopsy revealed multiple microinfarcts in the dermal collagen. The brain showed multiple small haemorrhagic infarcts of both hemispheres, the lower mid-brain, pons and a cerebellar peduncle. Microscopy showed fibrin exudation in the leptomeninges, various stages of thrombosis of small arteries without inflammatory reaction, and corresponding acute and subacute microinfarcts. The pathogenetic basis of the disease appeared to be a combined vasculopathy and coagulopathy. |
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Bibliography: | ark:/67375/NVC-TC3064WB-9 local:jnnp;44/2/156 istex:7A7F42ACE0E4E9D235115CE1F38C41E4356A2948 PMID:7217973 href:jnnp-44-156.pdf ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 0022-3050 1468-330X |
DOI: | 10.1136/jnnp.44.2.156 |