[Adult neuronal ceroid lipofuscinosis (Kufs disease)--a rare cause of dementia].
Kufs' disease is a very rare type of neuronal ceroid lipofuscinosis. A case of a 52 year old man with dementia is described. The cause for patient's dementia was the adult type (Kufs' disease) of neuronal ceroid lipofuscinosis. The diagnosis based on the histopathological post mortem-examination of the brain-tissue. A brother of our patient became ill with the same symptoms and at the same age of onset. So we conclude that there is a accumulation in the family.
Kozian
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Klinik für Psychiatrie und Neurologie Hans Berger, Friedrich-Schiller-Universität Jena.
Kiszka
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Peter
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D001921:Brain / Q000473:pathology; D003704:Dementia / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009472:Neuronal Ceroid-Lipofuscinoses / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D009483:Neuropsychological Tests; D014057:Tomography, X-Ray Computed
Die adulte neuronale Zeroidlipofuszinose (M. Kufs)--eine seltene Demenzursache.