Summary
A 55-year-old patient with known lung cancer was admitted with dystonia of the right arm, lockjaw, horizontal gaze palsy and dysphagia. Prior to death total ophthalmoplegia and central hypoventilation developed. Postmortem examination showed brainstem encephalitis, gangioradiculoneuritis, posterior column degeneration and loss of cerebellar Purkinje cells. These changes are considered to constitute a combined paraneoplastic syndrome. The dystonic movement disorder and central hypoventilation and their possible morphologic correlates are discussed.
Zusammenfassung
Bei einem 55 Jahre alten Patienten mit bekanntem Bronchialcarcinom entwickelten sich dystone Bewegungsstörungen des rechten Armes, Schluckbeschwerden und eine progrediente Kieferklemme. Hinzu kamen horizontale Blickparese, ataktische Störungen und Verminderung der Vibrationsempfindung. Vor dem Tode bestand neben kompletter Ophthalmoplegie und nahezu vollständiger Kiefersperre eine zentrale Hypoventilation. Die neuropathologische Untersuchung zeigte das Vorliegen von Hirnstammencephalitis, Ganglioradikuloneuritis, Hinterstrangdegeneration des Rückenmarkes und geringem Purkinje-Zell-Ausfall im Kleinhirn. Die Veränderungen werden als kombiniertes paraneoplastisches Syndrom erklärt. Die klinisch imponierenden Symptome der zentralen Hypoventilation und dystonen Bewegungsstörung werden beschrieben. Mögliche morphologische Korrelate werden diskutiert.
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Dietl, H.W., Pulst, S.M., Engelhardt, P. et al. Paraneoplastic brainstem encephalitis with acute dystonia and central hypoventilation. J Neurol 227, 229–238 (1982). https://doi.org/10.1007/BF00313390
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DOI: https://doi.org/10.1007/BF00313390