NR AMHQ
AU Weis,J.; Kretzschmar,H.A.; Windl,O.; Podoll,K.; Schwarz,M.
TI Fatal spongiform encephalopathy in a patient who had handled animal feed
QU Lancet 1996 Nov 2; 348(9036): 1240
PT letter
VT
Sir - Several cases of Creutzfeldt-Jakob disease (CJD) have been reported in dairy farmers exposed to bovine spongiform encephalopathy (BSE).[1] We report CJD in a patient who had handled animal feed.
A 66-year-old man presented with marked cognitive impairment including disturbances of memory, orientation, and concentration. First signs of intellectual deterioration had been noted 5 months before admission. There were visual disturbances including visual hallucinations as well as myoclonus and increased muscle tone of the limbs. Transplantations, previous neurosurgical procedures, or a family history of dementia were not known to the relatives. The patient had been trading professionally for many years in foodstuffs mainly for birds, but also for other animals such as pigs, until he fell ill. According to family members, he had occasionally tasted samples of the feed to check the quality of the ingredients. The components used to mix the feed are not known. In addition, family members reported that the patient used to prepare for personal consumption a gelatinous material from bovine bones and bone marrow almost daily for several years.
At later stages of the disease, there were pathological grasping reflexes, oral automatism, and progressive mental deterioration leading to akinetic mutism. The patient died from pulmonary embolism. Necropsy revealed severe typical spongiform changes of the cerebral cortex associated with gliosis and neuronal loss. Immunohistochemistry with a polyclonal prion protein (PrP) antiserum (MP-WB 138) showed diffuse neocortical deposits of PrP, but no plaques. No disease-associated mutation was detected by single-strand conformational polymorphism (SSCP) analysis and direct sequencing of the coding region of the PrP gene (PrNP). On one allele of PrNP, a variation at codon 124 (GGC*GGG) was found that is silent in the aminoacid. This variant allele has been described only in conjunction with the disease-associated Q217R mutation in a Swedish family with Gerstmann-Sträussler-Scheinker disease,[2] but it is most likely a rare and biologically silent polymorphism. At codon 129 the patient was homozygous for methionine. Homozygosity for methionine at this position is more frequent in CJD patients than in the normal population and was found in all patients analysed showing the new CJD variant (nvCJD) in the UK.[3]
The present case is by no means proof of an occupational risk of CJD for people who handle animal feed. No differential increase in the risk of CJD to farmers in the UK was found by the European Community surveillance project group.[4]
1 Smith PEM, Zeidler M, Ironside JW, et al. Creutzfeldt-Jakob disease in a dairy farmer. Lancet 1995; 346: 898.
2 Hsiao K, Dlouhy SR, Farlow MR, et al. Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Nature Genetics 1992; 1: 68-71.
3 Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: 921-25.
4 Delasnerie-Laupretre N, Poser S, Pocchiari M, et al. Creutzfeldt-Jakob disease in Europe. Lancet 1995; 346: 898.
Copyright (c) 1996, Elsevier Science Ltd. All rights reserved.
IN Ein 66 Jahre alter Tierfutterhändler, der gelegentlich Tierfutter kostete und sich täglich eine Suppe aus Rinderknochen kochte, erkrankte an der Creutzfeldt-Jakob-Krankheit. Über neurologische Operationen oder einen familiären Hintergrund war den Angehörigen nichts bekannt. Die Sequenzierung der kodierenden Regionen seiner Prionproteingene zeigte keine bekannte CJK-Mutation. Der Patient war homozygot Met/Met am Codon 129 und hatte lediglich eine stille Mutation GGC*GGG im Codon 124. Schon 5 Monate vor seiner Einweisung ins Klinikum Aachen hatte intellektuell nachgelassen. Er litt unter Sehstörungen, Halluzinationen, Myoklonien und erhöhter Muskelspannung in den Gliedern. Im Spätstadium wurden pathologische Greifreflexe und Mundbewegungen, zunehmender geistiger Verfall und schließlich Bewegungslosigkeit sowie Stummheit festgestellt. Der Patient starb an einer Lungenembolie. Neuropathologisch waren in der Hirnrinde die typische schwammförmige Degeneration aufgrund des Neuronenverlustes sowie die üblihe Gliosis zu beobachten. Immunologisch wurden diffuse Prionprotein-Ablagerungen, aber keine Plaques markiert.
ZR 4
MH Aged; Animal; *Animal Feed; Case Report; Cattle; Fatal Outcome; Human; Male; Prion Diseases/*etiology/physiopathology
AD *J Weis, H A Kretzschmar, O Windl, K Podoll, M Schwarz, *Institut für Neuropathologie and Neurologische Klinik, RWTH, D-52074 Aachen, Germany; and Institut für Neuropathologie, Universität Göttingen, Göttingen
SP englisch
PO England
OR Prion-Krankheiten 8