title: A case of rapidly progressive multiple system degeneration: morphological findings and pathogenetic implications.
abstract: A 62-year-old woman was referred to our psychiatric hospital by the municipal health office, because she was in such a neglected condition that she was a danger to herself. Initially, it was suspected that she was suffering from dementia or psychosis. X-rays led to the suspicion of bronchial carcinoma. Consequently, the mental changes were interpreted as probable effects of metastases to the brain. There was not enough time, however, to check the patient thoroughly to find the actual cause of her altered personality. The patient developed a high fever (up to 42 degrees C). A few days later, she died of cardiorespiratory failure with severe abscess-forming obstructive pneumonia. An autopsy confirmed the bronchial carcinoma. However, metastases were only found in the hilar lymph nodes. No metastases were detectable in the CNS, either macroscopically or microscopically. The neuropathological examination of the brain revealed multiple system degeneration. The striking microscopic findings (a large number of typical apoptotic figures visible with the light microscope in ganglion cells, lack of cytoplasmic inclusion bodies in the oligodendroglia and an unusually strong monocytic reaction (so-called reactive satellitosis) indicated that the disease course had been very rapid. Perhaps these were early steps in the evolution of a multisystem atrophy. A kind of time-lapse effect, as it were, revealed simultaneously individual details of the pathogenetic course, which would have disappeared in the usual long course of the disease and thus could not have been observed, even with the most up-to-date molecular methods. It is very likely that this was a paraneoplastic syndrome.
authors.affiliation: Department of Pathology, University of Kiel, Michaelisstrasse 11, 24105 Kiel, Germany. eschlueter@path.uni-kiel.de
authors.forename: E
authors.initials: E
authors.lastname: Schlüter
authors.forename: S
authors.initials: S
authors.lastname: Domm
authors.forename: S
authors.initials: S
authors.lastname: Erdag
mesh_terms: D001921:Brain / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000150:complications / Q000235:genetics* / Q000473:pathology*; D019636:Neurodegenerative Diseases / Q000150:complications / Q000235:genetics* / Q000473:pathology*; D013997:Time Factors