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Roland Heynkes, 5.5.2001
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Tamai,Y.; Kojima,H.; Kitajima,R.; Taguchi,F.; Ohtani,Y.; Kawaguchi,T.; Miura,S.; Sato,M.; Ishihara,Y. - Demonstration of the transmissible agent in tissue from a pregnant woman with Creutzfeldt-Jakob disease - New England Journal of Medicine 1992 Aug 27; 327(9): 649
DEMONSTRATION OF THE TRANSMISSIBLE AGENT IN TISSUE FROM A PREGNANT WOMAN WITH CREUTZFELDT-JAKOB DISEASE.
To the Editor: Only one case of Creutzfeldt-Jakob disease in pregnancy has ben reported so far.[1] We report our finding of the transmissible agent in samples from a pregnant patient with Creutzfeldt-Jakob disease.
A 38-year-old woman had visual disturbance in the 20th week of pregnancy. Speech and gait disturbances progressed rapidly until she reached a state of akinetic mutism. A cesarian section was performed in the 30th week. The newborn boy did well, but he was not breast-fed. He is developing normally at the age of six years. The patient died after a three-year course. At autopsy her brain weighed only 800 g. Neuropathological examination revealed a severe loss of nerve cells, a spongy state, and demyelination in both the cerebrum and the cerebellum. On the basis of the history, this case was considered to represent a spontaneous type of Creutzfeldt-Jakob disease. Analysis of prion proteins in the patient's tissues is in progress.
Specimens of the patient's blood, placenta, amniotic fluid, and umbilical-cord blood were obtained at delivery, and a sample of the patient's milk (colostrum) was obtained on the fourth day after delivery. The materials from the patient were injected intracerabrally into BALB/c mice, as described previously.[2] The tissue from the patient's brain was found to be infective, proving that the case was of a transmsible type (Table 1). Both the placenta and cord leukocytes were infective.
Maternal erythrocytes can be transferred to the fetus.[3] That fact plus our demonstration of the transmissible agent in placenta and cord-blood samples means that it is possible that the fetus was exposed to the transmissible agent of Creutzfeldt-Jakob disease. The mother's colostrum was also found to be infective. Histologic changes in the brains of the mice inoculated with the patient's colostrum included spongiform changes of various degrees in the cortex and white matter in both the cerebrum and the cerebellum, with reactive astrogliosis. However, no signs developed in control mice inoculated with healthy mother's milk. Neither epidemiologic studies[4-6] nor experimental studies[7,8] have demonstrated the vertical transmission of this disease to date. However, our findings indicate that we must be concerned about the risk of prenatal and postnatal transmission of the disease, and that followup studies are needed of the children at risk.
Yoichi Tamai, M.D., Hisako Kojima, Ph.D., Rie Kitajima, B.S., Fumiaki Taguchi, Ph.D., Yoshio Ohtani, M.D., Takeshi Kawaguchi, M.D.,Sadanori Miura, M.D., and Masaki Satao, M.D. - Kitasato University School of Medicine Kanagawa 228, Japan
Yoshihiro Ishihara, M.D. - Tokyo Metropolitan Institute for Neurosciences Tokyo 183, Japan
1. Bernoulli C, Siegfried J, Baumgartner G, et al. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1977; 1: 478-9
2. Tamai Y, Kojima H, Ohtani Y, et al. Subcellular distribution of the transmissible agent in Creutzfeldt-Jakob disease mouse brain. Microbiol Immunol 1989; 33: 35-42
3. Zarou DM, Lichtman HC, Hellman LM. The transmission of chromium-51 tagged maternal erythrocytes from mother to fetus. Am J Obstet Gynecol 1964; 88: 565-71
4. Prusiner SB, Gajdusek C, Alpers MP. Kuru with incubation periods exceeding two decades. Ann Neurol 1982; 12: 1-9
5. Brown P., Cathala F, Raubertas RF, Gajdusek DC, Castaigne P. The epidemiology f Creutzfeldt-Jakob disease:conclusion of a 15-year investigation in France and review of the world literature. Neurology 1987; 37; 895-904
6. Davanipour Z, Epidemiology. In: Bastian FO, ed. Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies. St Louis: Mosby-Year Book 1991: 131-52
7. Manuelidis EE, Manuelidis L. Experiments on maternal transmission of Creutzfeldt-Jakob disease in guinea pigs. Proc Soc Exp Biol Med 1979; 160: 233-6
8. Amyx HL, Gibbs CJ Jr, Gajdusek DC, Greer WE. Absence of vertical transmission of subacute spongiform viral encephalopathies in experimental primates. Proc Soc Exp Biol Med 1981; 166: 469-71
| Table 1. Distribution of the Creutzfeldt-Jakob Disease Agent in the Pregnant Patient's Blood and Tissues, According to Testing in BALB/c Mice.* | |||
| Sample | affected mice/inoculated mice | ||
|---|---|---|---|
| First Passage | Second Passage | ||
| Brain | 3/10 (235+/-10) | 6/15 (144+/-8) | |
| Blood | |||
| Erythrocytes | 0/10 | - | |
| Leukocytes | 0/10 | - | |
| Plasma + | 3/8 (243+/-26) | - | |
| Umbilical-cord blood | |||
| Erythrocytes | 0/8 | - | |
| Leukocytes | 1/10 (546) | 4/5 (246+/-4) | |
| Plasma # | 0/10 | - | |
| Amniotic fluid | 0/20 | - | |
| Placenta | 5/8 (253+/-10) | - | |
| Colostrum | 2/10 (611+/-0) | 5/10(202+/-32) # | |
| * The mice were observed for a period of at least 600 days after inoculation. The numbers in parentheses indicate the mean (+/- SD) incubation period in days. + Infective only after concentration to a volume of one third or less. # The third passage proved that the agent was totally infective (21 affected/21 inoculated); the mean incubation period was 131 days. |
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Eine 38-jährige Schwangere bekam in der 20. Schwangerschaftswoche Sehstörungen, die sich schnell zu einer Bewegungsstarre (akinetischer Mutismus) steigerten. Ihr Kind wurde in der 30. Woche per Kaiserschnitt geholt, nicht gestillt und erreichte bisher gesund das 6. Lebensjahr. Nach der Geburt wurden Proben der Plazenta, Leukozyten aus der Nabelschnur und Kolostrum vom 4. Tag nach der Geburt in die Gehirne von BALB/c-Mäusen injiziert. Alle Proben waren infektiös. Die Mutter starb erst 3 Jahre nach dem Auftreten der ersten Symptome mit einem Gehirngewicht von nur noch 800 g an der Creutzfeldt-Jakob-Krankheit. Im Blutplasma der Mutter wurde die Infektiosität erst nach 3-facher Konzentrierung festgestellt. Mit Erythrozyten und Leukozyten und Fruchtwasser der Mutter verliefen die Infektionsversuche erfolglos.
Die Ergebnisse dieses Experimentes werden seit langem verschwiegen oder angezweifelt. Der wissenschaftliche Lenkungsausschuß der EU schreibt dazu: [ANCL]
One single case has been reported on in 1992 of a 38-year-old pregnant woman with sporadic CJD whose colostrum was found to be infected when injected i/c into mice (Tamai et al, 1992). However, following further morphological examination of fixed mouse brain by immunohistochemistry for PrPsc, the Japanese authorities concluded that the published results were invalid as no spongiform change or PrPsc was found on first passage from human colostrum to mice. The brain from the second passage (mouse to mouse) did show spongiform change and PrPsc but this was not attributed to transmission from the colostrum (Prof. K Yamanouchi, personal communication to R. Bradley February 1997, in: E.C., 1997)
ANCL . Scientific Steering Committee - Safety of milk with regard to TSE: State of affairs - http://europa.eu.int/comm/food/fs/sc/ssc/out175_en.html
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