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Beitrag zur „progressiven multifokalen Leukoencephalopathie“

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Zusammenfassung

Bei einer 56jährigen Frau, bei der 2 Jahre vor dem Tode nach Auftreten mediastinaler und supraclaviculärer Drüsentumoren ein malignes Lymphogranulom festgestellt und eine Röntgenbehandlung durchgeführt wurde, setzte nach passagerer Beschwerdefreiheit 2 Monate ante exitum ein rasch progredientes zentralnervöses Krankheitsbild mit schweren psychischen Veränderungen bis Somnolenz, Frontalhirnsyndrom, rechtsseitiger Hemianopsie sowie schlaffer Quadruparese ein. Im Liquor bestand geringe Zell- und Eiweißvermehrung. Das stark diffus abnorme EEG bot generalisierte, frontal akzentuierte ϑ-δ-Tätigkeit. Autoptisch wurde eine typische Lymphogranulomatose verifiziert. Der neuropathologische Befund ergab multiple, unscharf begrenzte Entmarkungsherde im Großhirn sowie gehäuft in Hirnstamm und Oblongata, meist im Stadium des mobilen Abbaues mit gut erhaltenen Achsencylindern, intaktem Zellparenchym und fehlender Fasergliawucherung. In den als partielle Marknekrosen imponierenden Herdformationen besteht eine auffallende Gliazellreaktion mit polymorphkernigen, hyperplastischen Astrocyten, großen, chromatinreichen Kernen und einzelnen multinucleären Riesenzellen, doch tritt eine Astrogliawucherung auch in gesunden Hirnpartien auf. Als früheste Läsion erscheinen die in der Herdperipherie und deren Umgebung hervortretende starke Oligodendrogliaschwellung mit vereinzelten Kerneinschlüssen sowie eine spindelige Adventitiazellwucherung mit perivasaler Mikrogliaproliferation. Granulomatöse Veränderungen fehlen. Das Läsionsbild entspricht der von Aström, Mancall u. Richardson (1958) beschriebenen „progressiven multifokalen Leukoencephalopathie“, deren differentialdiagnostische Abgrenzung sowie Pathogenese und Ätiologie kurz diskutiert werden. Die Herdbildung wird formalgenetisch als sekundäres Entmarkungsgeschehen nach Art einer fokalen glio-vasalen Markdystrophie vermutlich infolge primärer Oligodendrogliaaffektion sowie mit atypischer Makrogliareaktion unter einem pathologischen formativen Reiz aufgefaßt, dessen Wesen bisher ungeklärt ist.

Summary

In a woman 56 years of age a malignant lymphoma was found as the cause of mediastinal and supraclavicular swellings 2 years before death, and X-ray treatment was carried out. After a short period, during which she had no complaints, a rapidly progressive disorder of the central nervous system commenced, with severe mental disturbances, somnolence, a frontal lobe syndrome, hemianopsia of the right side and flaccid quadruparesis. There was a slight increase of cells and protein in the cerebrospinal fluid, in the EEG generalized ϑ-δ-wave activity with frontal accentuation was found. Autopsy revealed typical lymphogranulomatosis. Neuropathologically, multiple, not sharply defined foci of demyelination weare seen in the cerebrum, more numerous in the brainstem and oblongata, and mostly in the stage of mobile breakdown with well preserved axon cylinders, intact cell parenchyma and without proliferation of fibrous glia. In the foci with the appearance of partial myelin necrosis there was a conspicuous glia cell reaction with polymorphonuclear, hyperplastic astrocytes, large chromatin-rich nuclei and single multinuclear giant cells. Astroglia proliferation, however, was also seen in the unaltered areas of the brain. The earliest lesions appearing are marked swellings of oligodendroglia with occasional nuclear inclusions, spindle-shaped proliferation of adventitial cells and perivascular microgliaproliferation in the periphery of foci and the surrounding areas. Granulomatous changes are absent. The lesions correspond to those described by Aström, Mancall and Richardson (1958) as „progressive multifocal leucoencephalopathy“. The differential diagnosis, pathogenesis and etiology of this disease are briefly discussed. The production of foci is morphogenetically explained as a secondary demyelination phenomenon of the character of a focal glio-vasal myelin dystrophy probably caused by primary affection of the oligodendroglia with atypical microglia reaction under the influence of a pathological formative stimulus of unclear nature.

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  1. Aus dem Neurologischen Institut der Universität Wien, Austria

    K. Jellinger & F. Seitelberger

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Jellinger, K., Seitelberger, F. Beitrag zur „progressiven multifokalen Leukoencephalopathie“. Deutsche Zeitschrift f. Nervenheilkunde 187, 749–769 (1965). https://doi.org/10.1007/BF00242492

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