Zusammenfassung
Das Wernicke-Korsakoff-Syndrom (WKS) wird hervorgerufen durch einen Mangel an Thiamin (Vitamin B1) und tritt häufig bei chronischem Alkoholkonsum auf. Wir berichten über eine 64-jährige, nicht alkoholabhängige Frau, die nach 3-wöchiger parenteraler Ernährung an einem WKS erkrankte. Als ungewöhnliches Initialsymptom stellte sich eine beiderseitige Amaurose ein. Erst Tage später traten Bewusstseinsstörung und spastische Tetraparese hinzu. Das kraniale MRT zeigte symmetrische Signalalterationen (T2-, FLAIR- und Diffusionswichtung) in folgenden Lokalisationen: medialer Thalamus, periaquäduktales Mesenzephalon unter Einschluss der 4-Hügel-Platte, Corpora mamillaria und ungewöhnlicherweise beidseitig im Gyrus praecentralis. Laborchemisch wurde die Diagnose WKS durch den Nachweis des erniedrigten Thiaminspiegels bestätigt. Nach mehrwöchiger i. v. Thiamingabe bildeten sich die magnetresonanztomographischen Veränderungen fast vollständig, die neurologischen Störungen aber nur teilweise zurück.
Abstract
Wernicke-Korsakow Syndrome (WKS) is caused by thiamine (vitamin B1) deficiency and usually occurs in conjunction with chronic alcohol abuse. Our report concerns a 64-year-old, nonalcoholic, woman with no history of alcohol abuse, who became ill with WKS after 3 weeks of parenteral nourishment. As an unusual initial symptom she went blind in both eyes; this was followed a few days later by impaired consciousness and spastic tetraparesis. A cranial MRI examination showed symmetrical signal alteration (T2, FLAIR and diffusion weighting) in the medial thalamus, periaqueductal mesencephalon including the quadrigeminal plate, mamillary bodies and—most unusually—both paracentral gyri. Laboratory tests confirmed the diagnosis of WKS as significant thiamine deficiency was detected. Following several weeks of intravenous thiamine supplementation the MRI lesions were almost completely reversed but the neurological deficits regressed only partially.
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Kuhn, J., Friedel, V., Knitelius, H.O. et al. Iatrogenes Wernicke-Korsakoff-Syndrom mit ungewöhnlichen Symptomen und MRT-Veränderungen . Nervenarzt 75, 795–800 (2004). https://doi.org/10.1007/s00115-004-1694-7
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DOI: https://doi.org/10.1007/s00115-004-1694-7