DOCUMENTS DEPARTMENT ssp 0 e 1991 . URREM UNWERSIilagé‘mume .w m EWMDGHIES EN MEDJEENE _ s‘ No. 91-5 ; “X99, ,. g Transmissible Subacute f Spongiform Encephalopathies: Transmission Between Animals and Man National Library of Medicine January 1986 through May 1991 148 Citations 3‘95"» -. 5 sfi‘ . fimfigyfik 14:345va figfikggfigggqfifiggfirlahwkfi;k% W 4 v. r' r US. DEPARTMENT OF HEALTH & HUMAN SERVICES , Public Health Service 0 National Institutes of Health CBM 91-5 Transmissible Subacute Spongiform Encephalopathies: Transmission Between Animals and Man January 1986 through May 1991 148 Citations Prepared by Fritz P. Gluckstein, D.V.M., M.L.S. Coordinator for Veterinary Affairs U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES Public Health Service National Institutes of Health National Library of Medicine Reference Section 8600 Rockville Pike Bethesda, Maryland 20894 SERIES NOTE Current Bibliographies in Medicine (CBM) is a continuation in part of the National Library of Medicine’s Literature Search Series, which ceased in 1987 with No. 87-15. In 1989 it also subsumed the Specialized Bibliography Series. Each bibliography in the new series covers a distinct subject area of biomedicine and is intended to fulfill a current awareness function. Citations are usually derived from searching a variety of online databases. NLM databases utilized include MEDLINE", AVLINE', BIOETHICSLINE', CANCERLIT’, CATLINE", HEALTH, POPLINE" and TOXLINE'. The only criterion for the inclusion of a particular published work is its relevance to the topic being presented; the format, ownership, or location of the material is not considered. Comments and suggestions on this series may be addressed to: [1 Karen Patrias, Editor Current Bibliographies in Medicine ‘ Reference Section 1 National Library of Medicine ‘ Bethesda. MD 20894 301/496-6097 Ordering Information: Current Bibliographies in Medicine is sold by the Superintendent of Documents, US. Government Printing Office, ashington, DC. 20402. To order the entire CBM series for the calendar year 1991 (20 bibliographic?) send $52.00 ($65.00 foreign) to the Superintendent of Documents citing GPO List ID: CBM91. For your convenience an order blank is given inside the back cover. Orders for individual bibliographies in the series ($3.00, $3.75 foreign) should be sent to the Superintendent of Documents citing Lhe title, CBM number, Ed the GPO Li_st ID given above. Use of funds for printing this periodical has been approved by the Director of the Ofi‘ice of Management and Budget through September 30, 1991. ii ECHug \(L It i '23.. G (a TRANSMISSIBLE SUBACUTE SPONGIFORM ENCEPHALOPATHIES: TRANSMISSION BETWEEN ANIMALS AND MAN Transmissible subacute spongiform encephalopathies are diseases of the brain in animals and man caused by unconventional viruses. The recent outbreak of bovine spongiform encephalopathy ("Mad Cow" disease) in the United Kingdom and concerns that it might be passed to man through infected meat has heightened the interest in these diseases. This bibliography deals with the 'zoonotic aspects of the transmissible subacute spongiform encephalopathies, i.e., issues relating to the transmission of these encephalopathies between animals and man. These are the transmission to man of bovine spongiform encephalopathy, scrapie, transmissible mink encephalopathy, and chronic wasting disease of deer and elk as well as the transmission to animals of Creutzfeldt-Jakob disease, Gerstmann-Straussler syndrome, and kuru to. It includes journal articles, meeting abstracts, editorials, letters to the editor, news items, and chapters of books. The contents are arranged in six subject categories; citations may appear in more than one category. iii iii! FUEL SEARCH STRATEGY A variety of online databases are usually searched in preparing bibliographies in the CBM series. To assist you in updating or otherwise manipulating the material in this search, the strategy used for the NLM’s MEDLINE database is given below. Please note that the search strategies presented here differ from individual demand searches in that they are generally broadly formulated and irrelevant citations edited out prior to printing. SS 1 = (TW) SPONGIFORM AND ALL ENCEPHALOPATH: OR BSE SSZ=(TW) MAD AND COW SS 3 = (TW) MINK AND ALL ENCEPHALOPATH: SS 4: (TW) WASTING AND ALL ELK# OR WASTING AND ALL DEER# SS 5 = SCRAPIE OR SCRAPIE AGENT SS 6 = CREUTZFELDT-JAKOB SYNDROME OR KURU OR ALL GERSTMANN: (TW) SS7=lOR 2 OR 3 OR 4 OR 5 OR 6 SS 8 = 7 AND ZOONOSES SS9=l AND HUMAN AND ANIMAL SSlO=2 OR 3 OR 4 OR 5 8811 = 10 AND HUMAN SS12=6 AND ANIMAL SSl3=8 OR 9 OR 11 OR 12 GRATEFUL MED“D To make online searching easier and more efficient, the Library offers GRATEFUL MED, microcomputer-based software that provides a user-friendly interface to most NLM databases. This software was specifically developed for health professionals and features multiple choice menus and "fill in the blank" screens for easy search preparation. GRATEFUL MED runs on an IBM PC (or IBM- compatible) with DOS 2.0 or a Macintosh, and requires a Hayes (or Hayes-compatible) modem. It may be purchased from the National Technical Information Service in Springfield, Virginia, for $29.95 (plus $3.00 per order for shipping). For your convenience, an order blank has been enclosed at the back of this bibliography. iv SAMPLE CITATIONS Citations in this bibliographic series are formatted according to the rules established for Index Medicus'. Sample journal and monograph citations appear below. For journal articles written in a foreign language, the English translation of the title is placed in brackets; for monographs, the title is given in the original language. In both cases the language of publication is shown by a three letter abbreviation appearing at the end of the citation. Note also that a colon (z) may appear within an author’s name or article title. The NLM computer system automatically inserts this symbol in the place of a diacritical mark. Journal Article: Authors Article Title / \ \ Holt TA, Phillips J. Bovine spongiform encephalopathy. Br Med J (Clin Res) 1988 Jun 4;296(6636):1581-2. / l I Abbreviated Journal Date Volume Issue Pages Title Monograph: Author Title Southwood R. Report of the working party on bovine spongiform encephalopathy. Stanmore (Middlesex): UK Dept. of Health, Leaflets Unit; 1988. 35 p. / / l Place of Publisher Date Total No. Publication of Pages TABLE OF CONTENTS General Bovine Spongiform Encephalopathy Scrapie Creutzfeldt-Jakob Syndrome Gerstmann-Straussler Syndrome Books and Book Chapters vfi page . » ‘ val , Q. . . . “M y ) _ ... 4 a, L _ f V. ; M. . A A I w. . \ n . a m_ _ , J n, a ,, . , 2 .1 La: \ General Bocharov AL. Romanova LG. [Autocatalytic nature of "slow virus infections"]. Mol Biol (Mosk) 1986 Nov-Dec;20(6):1665-71. (Rus). Brahic M. [Slow viruses]. Rev Prat 1987 Oct 22; 37(42):2535-9. (Fre). Brown F. Unconventional viruses and the central nervous system. BMJ l987;295(6594):347-8. Carp RI. Kascsak R]. Wisniewski HM, Merz PA. Rubenstein R. Bendheim P. Bolton D. The nature of the unconventional slow infection agents remains a puzzle. Alzheimer Dis Assoc Disord 1989 Spring- Summer;3(1-2):79-99. Dreyfus J-C. [Prions or no prions: transgenic mice and mad cows]. M S (Med Sci) l990;6(3):311. (Fre). Edlin G]. The senile dementias: a new model. Med Hypotheses 1988 Sep;27(1):29-3l. Frzydl V. [Relation between slow virus infection of animals and man. their pathogenicity and resistance to therapy]. Fortschr Neurol Psychiatr 1988 Jun; 56(6):186-92. (Ger). Hsiao K. Prusiner SB. Inherited human prion diseases. Neurology 1990 Dec;40(12):1820-7. Israil A. [Prions]. Rev Ig (Bacteriol) 1988 Apr- Jun;33(2):117-32. (Rum). Israil A. [Update on prions and prion diseases]. Rev Ig (Bacteriol) 1990;35(1):l7-40. (Rum). Last PM. Cell therapy a cruel and dangerous deception a drama in three acts. J Paediatr Child Health 1990; 26(4): 197-9. Liberski PP. [Transmissible spongiform encephalopathy. Natural diseases and experimental studies]. Neuropatol Pol l986;24(4):43l-41. (Pol). McKerrell RE. Farmer H. Spongifonn encephalopathies: implications of recent developments. J R Soc Med 1990;83(5):334-6. Morse SS. Looking for a link [letter]. Nature 1990 Mar 22;344(6264):297. Pastoret PP. [Spongiform encephalopathies in humans and animals]. Ann Med Vet 1991 Feb;135(2):13l. (Fre). Picavet T. [Spongiform encephalopathy in humans and animals. Meeting report 1990 Dec 17; Brussels]. Vlaams Diergeneeskd Tijdschr 1991 Mar-Apr; 60(2):71. (Dut). [Preparations from animal organs or organ extracts. Slow virus infections transmissible through medicaments?] Dtsch Apoth Ztg 1990;130(38): 2076. (Ger). Prion disease--spongiform encephalopathies unveiled. Lancet 1990 Jul 7;336(8706):21-2. Tateishi J. [Slow virus, prion and nervous system]. Tanpakushitsu Kakusan Koso 1990 May;35 (7 Suppl):1327—31. (Jpn). Zschiesche W. Jakob W. Pathology of animal amyloidoses. Pharmacol Ther 1989;41(1-2):49-83. 2 Bovine Spongiform Encephalopathies Adams DH. Bovine spongiform encephalopathy--a new disease u'ansmissable to humans? Med Hypotheses 1990 Aug;32(4):313-7. Aldhous P. BSE. No madness in UK policy [news]. Nature 1990 Jul 19;346(6281):211. Aldhous P. Epidemiology: BSE causing public alarm [news]. Nature 1990 Jan l8;343(6255):196. Barlow RM. Bovine spongiform encephalopathy (BSE): menace or mirage? Br Vet J 1990 Nov- Dec;l46(6): 485- 6. Barr M. Bovine spongiform encephalopathy/scrapie [letter]. Vet Rec 1990 Jul 7;127(1):23. Beardsley T. Oravske kuru. A human dementia raises the stakes in mad cow disease [news]. Sci Am 1990 Aug;263(2):24-6. Blakemore WF. Bovine spongiform encephalopathy and scrapie: potential human hazards. Outlook Agric 1989;18(4):165-8. Boothby CB. Bovine spongiform encephalopathy: possible toxicity link? [letter]. Vet Rec 1988 Jan 23;122(4):95. Bovine spongiform encephalopathy (BSE) - The story so far. State Vet J 1990;44(124):3-18. Bovine spongiform encephalopathy [letter]. BMJ 1990 Apr 7;300(6729):943-4. Bovine spongiform encephalopathy [letter]. BMJ 1988 Jul 9;297(6641):133-4. Bovine spongiform encephalopathy [letter]. Vet Rec 1988 Jul 23:123(4):114. [Bovine spongiform encephalopathy: old disease in new dress]. Dtsch Apoth Ztg l990;130(38):2074-5. (Ger). Bradley R. Bovine spongiform encephalopathy: the need for knowledge. balance. patience. and action. J Pathol 1990 Apr;160(4):283-5. BSE and scrapie: agents for change [editorial]. Lancet 1988 Sep 10;2(8611):607-8. BSE in perspective [editorial]. Lancet 1990 May 26; 335(8700):1252-3. Burke E. BSE: conflicting advice on ‘mad cow disease.‘ Health Visit 1990 Ju1;63(7):218. Burns KN. Current animal events concerning human health. I R Agric Soc Eng] 1989;150:90-100. Cherfas J. Mad cow disease: uncertainty rules. Science 1990 Sep 28;249(4976):1492-3. Collee JG. Bovine spongiform encephalopathy. Lancet 1990 Nov 24;336(8726):1300-3. Collinge J. Owen F. Poulter M. Leach M. Crow TJ. Rossor MN. Hardy J, Mullan MJ. Janota I. Lantos PL. Prion dementia without characteristic pathology. Lancet 1990 Jul 7;336(8706):7-9. Dealler S, Lacey RW. Bovine spongiform encephalopathy and man [letter]. Lancet 1991 Jan 19;337(8734):l73—4. Dealler SF. Lacey RW. 'I‘ransmissible spongiform encephalopathies: the threat of bse to man. Food Microbiol l990;7(4):253-80. Done JT. Bovine Spongiform encephalopathy [letter]. Vet Rec 1990 Jun 23:126(25):628-9. Duchen L. Spongiform encephalopathy. Practitioner 1989 Oct 22;233(1477):1407. 1409-10. Egert J. Suess J. [The bovine spongiform encephalopathy]. Z Klin Med 1990;45(23):2065-7. (Ger). Fear C. Devakumar M. Bovine spongiform encephalopathy [letter]. BMJ 1990 Mar 24; 300(6727):817. Frankness needed [editorial]. Nature 1990 Jun 21; 345(6277):648. Gibbs CJ. RecoMendations for initiation of research studies on bovine spongiform encephalopathy. J Am Vet Med Assoc 1990 May 15 .:196(10) 1687- 8. Hodgson E. BSE. An unlikely zoonosis. Occup Health (Lond) 1990 Sep;42(9):265-6. Holt TA. Phillips J. Bovine spongiform encephalopathy. Br Med J (Clin Res) 1988 Jun 4; 296(6636):1581-2. Hope J. Bovine spongiform encephalopathy. J Biol Educ 1990 Winter;24(4):225-8. How the French see BSE. Vet Rec 1990 Nov 3; 127(18):440-1. Howard R, Castle D. Concern about bovine spongiform encephalopathy [letter]. Lancet 1990 Aug 4;336(8710):316. Human spongiform encephalopathy. State Vet J 1990; 44(124):19-30. Karlsson Y. [Can the mad cow disease hit Sweden? Can it be transmitted to humans by meat?] Lakartidningen 1990 Jul ll;87(28-29):2361-4. (Swe). Kimberlin RH. Bovine spongiform encephalopathy: taking stock of the issues. Nature 1990;345(6278): 736-4. Kirnberlin RH. Detection of bovine spongiform encephalopathy in the United Kingdom. J Am Vet Med Assoc 1990 May 15;196(10):1675-6. Kosenow W. [Slow virus diseases--apropos the danger by infectious bovine encephalopathy (BSE)]. Kinderkrankenschwester 1990 Jul;9(7):213. (Ger). Ladds AD. Policy on BSE [letter]. Vet Rec 1989 May 27;124(21):571. Lovestone S. Delusional bovine spongiform encephalopathy [letter]. Lancet 1990 Sep 1; 336(8714):565. Mad cows and the minister [editorial]. Nature 1990 May 24;345(6273):277-8. Madeiros CA. BSE safety precautions [letter]. Vet Rec 1989 Jul 15:125(3):73-4. Matthews D. Bovine spongiform encephalopathy. J R Soc Health 1991 Feb;111(1):3-5. Matthews WB. Bovine spongiform encephalopathy. BMJ 1990 Feb 17;300(6722):412-3. Mayr A. Ring C. [Bovine spongiform encephalopathy: a challenge to research. administration. and the meat industry]. Fleischwirtsch 1991 Mar;7l(3):269-71. (Ger). McKerrell RE. Farmer H. Spongiform encephalopathies: implications of recent developments. J R Soc Med 1990;83(5):334-6. Mields W. [British cattle plague--also a danger for man? Bovine spongiform encephalopathy (BSE)-- scrapie disease]. Fortschr Med 1990 Aug 10; 108(23):448-9. (Ger). Mields W. [Is bovine spongiform encephalopathy. a hitherto unknown zoonosis. a potential menace?] Arztl Lab 1990;36(9):240-5. (Ger). Minor P. Bovine spongiform encephalopathy and biological products for human use. Report of an informal meeting held at NIBSC on 16 May 1988. Biologicals 1990 Jan;l8(1):77-80. Molgaard CA. Golbeck AL. Mad cows and Englishmen: Bovine spongiform encephalopathy. Neuroepidemiology 1990;9(6):285-6. MPs to look at government handling of BSE [news]. Vet Rec 1990 May 26;126(21):516. Multidisciplinary team dismisses BSE ‘threat' [news]. Vet Rec 1990 Jun 23;126(25):612-3. Narang HK. Perry RH. High incidence of Creutzfeldt- Jakob disease in Northern England [abstract]. Neuropathol Appl Neurobiol l990;16(3):259. Parodi AL. Brugere-Picoux J . Chatelain J, Laplanche JL. [Bovine spongiform encephalopathy: a new entity caused by a non-conventional transmissible agent]. Bull Acad Natl Med 1990 Jun-Jul;174(6):731-9; discussion 740-1. (Fre). Phillpotts R J. Bovine spongiform encephalopathy a hazard to cell culture and biologically based medicines. Cytotechnology 1989;2(3):161-2. Rasmussen SB. [Bovine spongiform encephalopathy (BSE)]. Dan Veterinaenidsslcr 1989;72(11):637-40. (Dan). Roberts GW, Collinge J. Bovine spongiform encephalopathy. Br Med J 1990;300(6729):943-4. Samaille JP. [Animal production and public health]. Action Vet l990;3-4:1121-2. (Fre). Schreuder BE, Osterhaus AD. [Bovine spongiform encephalopathy (ESE): a review]. Tijdschr Diergeneeskd 1990;115(11):507-l7. (Dut). Seale J. Crossing the species barrier-viruses and the origins of AIDS in perspective. J R Soc Med 1989; 82(9):519-23. Southwood calls for monitoring of BSE risk in veterinarians [news]. Vet Rec 1989 Mar 4; 124(9):207-10. Stenklev NC. [Mad cow disease]. Tidsskr Nor Laegeforen 1990 Sep 20;110(22):2914. (Nor). Stober M. [Bovine spongiform encephalopathy (BSE): state of knowledge and measures]. DTW Dtsch Tierarztl Wochenschr 1990 Dec;97(12):540-3. (Ger). Survey of encephalopathy incidence set up by Health ministry [news]. Vet Rec 1990 May 5:126(18): 447-8. Taylor DM. Bovine spongiform encephalopathy and human health. Vet Rec 1989 Oct l4;125(l6):413-5. Taylor DM. No known human health risk from scrapie or BSE [abstract]. Large Anirn Vet Rep 1990; 1(3):20. 4 'I‘yrrell report spells out priorities for BSE research [news]. Vet Rec 1990 Jan 20;126(3):48. Wacker K. Liebermann H. Meyer A. [Bovine spongiform encephalopathy a new slow infection of cattle]. Monatsh Veterinaermed 1990;45(21): 739-41. (Ger). Whitwam L. Searching for a link. Nurs Times 1990 Feb 14-20;86(7):23. Scrapie Arya SC. Prion disease [letter]. Lancet 1990 Aug ll;336(8711):370. Arya SC. Spread of "unconventional viruses" through sheep-brain rabies vaccines. Vaccine 1991 Jan; 9(1):70. Baron H, Baron-Van Evercooren A. Brucher JM. Antiserum to scrapie-associated fibril protein reacts with amyloid plaques in familial transmissible dementia. J Neuropathol Exp Neurol 1988;47(2): 158-65. Barr M. Bovine spongiform encephalopathy/scrapie [letter]. Vet Rec 1990 Jul 7;127(l):23. Blakemore WF. Bovine spongiform encephalopathy and scrapie: potential human hazards. Outlook Agric 1989;18(4):165-8. Bockman IM, Kingsbury DT. Immunological analysis of host and agent effects on Creutzfeldt-Jakob disease and scrapie prion proteins. J Virol 1988 Sep;62(9):3120-7. Braun MJ. Gonda MA. Is scrapie Prp 27-30 related to AIDS virus? Nature 1987;325(6100):113-4. Brown P, Gajdusek DC. Survival of scrapie virus after 3 years' interment. Lancet 1991;337(8736):269-70. BSE and scrapie: agents for change [editorial]. Lancet 1988 Sep 10;2(8611):607-8. Cathala F. Scrapie in France. J Am Vet Med Assoc 1990 May 15;196(10):1680. Chatelain J. Dautheville-Guibal C. Ovine scrapie: Follow-up of sheep belonging to an endemic scrapie-infected flock. Eur J Epidemiol 1989; 5(1): 1 13-6. Chatelain JC. Dautheville—Guibal C. [Has scrapie of sheep and goats a role in the transmission of Creutzfeldt-Jakob disease? (letter)]. Rev Epidemiol Sante Publique l988;36(6):488. (Fre). Goldfarb LG. Mitrova E. Brown P. Toh BH. Gajdusek DC. Mutation of codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia. Lancet 1990;336(8713):514-5. Goudsmit J, Van der Waals FW. Scrapie and its association with ‘amyloid-like' fibrils and glycoproteins encoded by cellular genes: an animal model for human dementia. Prog Brain Res 1986; 70:391-8. Hsiao K. Meiner Z, Kahana E. Cass C. Kahana I. Avrahami BA. Scarlato G. Abramsky O, Prusiner SB. Gabizon R. Mutation of the prion protein in Lybian jews with Creutzfeldt-Jakob disease. N Engl J Med 1991 Apr 18;324(l6):1091-7. Lewin PK. Scrapie and human neurodegenerative diseases [letter]. Can Med Assoc J 1990 May 1; l42(9):928. Liberski PP. [Scrapie agent (1etter)]. Pol T‘yg Lek 1987 Aug 31;42(35):1095-6. (Pol). Lukiw WJ. Cho HJ. Kaufmann JC. Crapper McLachlan DR. The molecular mechanisms of scrapie encephalopathy and relevance to human neurodegenerative disease. Can J Vet Res 1990 Jan;54(l):49-57. Mititelu G, Bourceanu I. The ovine risk presence in prion diseases a thematic follow-up [abstract]. Acta Neurol Scand 1990;81(3):26l. Taylor DM. No known human health risk from scrapie or BSE [abstract]. Large Anirn Vet Rep 1990; 1(3):20. Creutzfeldt-Jakob Syndrome Arya SC. Prion disease [letter]. Lancet 1990 Aug ll;336(8711):370. Arya SC. Spread of "unconventional viruses" through sheep-brain rabies vaccines. Vaccine 1991 Jan; 9(1):70. Baker HF. Duchen LW, Jacobs JM. Ridley RM. Spongiform encephalopathy transmitted experimentally from Creutzfeldt—Jakob and familial Gerstmann-Straussler—Scheinker diseases. Brain 1990 Dec;113(Pt 6):l891-909. Baron H. Baron-Van Evercooren A. Brucher JM. Antiserum to scrapie-associated fibril protein reacts with amyloid plaques in familial transmissible dementia. J Neuropathol Exp Neurol 1988;47(2): 158-65. Bockrnan JM. Kingsbury DT. Immunological analysis of host and agent effects on Creutzfeldt-Jakob disease and scrapie prion proteins. J Virol 1988 Sep;62(9):3120-7. Boellaard JW. Schlote W. Tateishi J. Neuronal autophagy in experimental Creutzfeldt—Jakob’s disease. Acta Neuropathol (Berl) l989;78(4):410-8. Brown P. Cathala F, Raubertas RF. Gajdusek DC. Castaigne P. The epidemiology of Creutzfeldt— Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. Neurology 1987 Jun;37(6):895-904. Cathala F. Scrapie in France. J Am Vet Med Assoc 1990 May 15:196(10):1680. Cathala F. Brown P. Chatelain J . Castaigne P. Gajdusek C. [Creutzfeldt-Jakob disease in France. Value of familial forms. Is there a gene controlling the length of the incubation period?] Presse Med 1986 Feb 22; 15(8):379—82. (Fre). Chatelain J. Dautheville—Guibal C. Ovine scrapie: Follow-up of sheep belonging to an endemic scrapie-infected flock. Eur J Epidemiol 1989; 5(1): 1 13-6. Chatelain JC, Dautheville—Guibal C. [Has scrapie of sheep and goats a role in the transmission of Creutzfeldt-Jakob disease? (1etter)]. Rev Epidemiol Sante Publique 1988;36(6):488. (Fre). Chippaux A. [Non-conventional central nervous system agents]. Bull Soc Pathol Exot Filiales 1987; 80(5):727-36. (Fre). Davanipour Z, Alter M, Sobel E. Creutzfeldt-Jakob disease. Neurol Clin 1986 May;4(2):415-26. 6 Davanipour Z. Alter M. Sobel E. Asher DM. Gajdusek DC. Transmissible virus dementia: evaluation of a zoonotic hypothesis. Neuroepidemiology 1986; 5(4):]94-206. Draganescu N. [Presenile dementia (Creutzfeldt—Jakob disease). Clinical and experimental data on a newly reported case]. Virologie 1988 Oct-Dec;39(4): 257-60. (Fre). Goldfarb LG. Mitrova E. Brown P. Toh BH. Gajdusek DC. Mutation of codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia. Lancet 1990;336(8713):514-5. Goudsmit J. Van der Waals FW. Scrapie and its association with ‘amyloid-like' fibrils and glycoproteins encoded by cellular genes: an animal model for human dementia. Prog Brain Res 1986; 70:391-8. Gourmelon P. Amyx HL, Baron H. Lemercier G. Court L. Gibbs CJ Jr. Sleep abnormalities with REM disorder in experimental Creutzfeldt-Jakob disease in cats: at new pathological feature. Brain Res 1987 May 19:41 l(2):39l-6. Hsiao K. Meiner Z, Kahana E. Cass C. Kahana I. Avrahami BA, Scarlato G. Abramsky O. Prusiner SB. Gabizon R. Mutation of the prion protein in Lybian jews with Creutzfeldt-Jakob disease. N Engl J Med 1991 Apr 18;324(16):1091-7. Kim JH. Lach B. Manuelidis EE. Creutzfeldt-Jakob disease with intranuclear vacuolar inclusions: a biopsy case of negative light microscopic findings and successful animal transmission. Acta Neuropathol (Berl) l988;76(4):422-6. Kim JH. Manuelidis EE. Neuronal alterations in experimental Creutzfeldt-Jakob disease: a Golgi study. J Neurol Sci 1989 Jan;89(1):93-101. Kim JH. Manuelidis EE. Serial ultrastructural study of experimental Creutzfeldt-Jacob disease in guinea pigs. Acta Neuropathol (Berl) 1986;69(1-2):81-90. Kim JH. Manuelidis L. Manuelidis EE. Cerebral glycosidases in experimental Creutzfeldt-Jakob disease. J Neurol Sci 1988 Mar;84(1):95-100. Kim JH. Manuelidis L. Manuelidis EE. Lysosomal glycosidase activities in experimental creutzfeldt- jakob disease [abstract]. J Neuropathol Exp Neurol l986;45(3):336. Kitamoto T. Mohri S. Tateishi J. Organ distribution of proteinase-resistant prion protein in humans and mice with Creutzfeldt-Jakob disease. J Gen Virol 1989 Dec;70(Pt 12):3371-9. Kitamoto T. Tateishi J, Sawa H. Doh-Ura K. Positive transmission of Creutzfeldt-Jakob disease verified by murine kuru plaques. Lab Invest 1989 Apr:60(4): 507-12. Kitamoto T. Yi R, Mohri S. Tateishi J. Cerebral amyloid in mice with Creutzfeldt—Jakob disease is influenced by the strain of the infectious agent. Brain Res 1990 Jan 29;508(1):165-7. Liberski PP. Yanagihara R. Asher DM. Gibbs CJ Jr. Gajdusek DC. Reevaluation of the ultrastructural pathology of experimental Creutzfeldt—Jakob disease. Serial studies of the Fujisaki strain of Creutzfeldt- Jakob disease virus in mice. Brain 1990 Feb;113 (Pt 1):121-37. Liberski PP. Yanagihara R. Gibbs CJ Jr. Gajdusek DC. Appearance of tubulovesicular structures in experimental Creutzfeldt-Jakob disease and scrapie precedes the onset of clinical disease. Acta Neuropathol (Berl) l990;79(4):349-54. Liberski PP, Yanagihara R. Gibbs CJ Jr. Gajdusek DC. Spread of Creutzfeldt-Jakob disease virus along visual pathways after intraocular inoculation. Arch Virol 1990;111(1-2):141-7. Liberski PP. Yanagihara R. Gibbs CJ Jr. Gajdusek DC. Tubulovesicular structures in experimental Creutzfeldt-Jakob disease and scrapie. Intervirology 1988;29(2):115-9. Liberski PP. Yanagihara R. Gibbs CJ Jr. Gajdusek DC. White matter ultrasu'uctural pathology in experimental creutzfeldt-jakob disease in mice [abstract]. Neurology l988;38(3 Suppl 1):288. Liberski PP, Yanagihara R. Gibbs CJ Jr, Gajdusek DC. White matter ultrastructural pathology of experimental Creutzfeldt-Jakob disease in mice. Acta Neuropathol (Berl) l989;79(1):l-9. Manuelidis L. Kim JH. Manuelidis EE. Experimental Creutzfeldt-Jakob diease with destructive lesions in the white matter [abstract]. J Neuropathol Exp Neurol l986;45(3):330. Marx J. Human brain disease recreated in mice [news]. Science 1990 Dec l4;250(4987)11509-10. Meiner Z, Gabizon R. Kahana E. [Creutzfeldt—Jakob diseases: etiology and epidemiology]. Harefuah 1991 Jan l;120(1):19—22. (Heb). Mititelu G. Bourceanu I. The ovine risk presence in prion diseases a thematic follow-up [abstract]. Acta Neurol Scand 1990;81(3):261. Mohri S. Tateishi J. Host genetic control of incubation periods of Creutzfeldt-Jakob disease in mice. J Gen Virol 1989 Jun;70(Pt 6)11391-400. Mori S. Hamada C. Kumanishi T, Fukuhara N, Ichihashi Y. Ikuta F. Miyatake T. Tsubaki T. A Creutzfeldt- Jakob disease agent (Echigo-l strain) recovered from brain tissue showing the ‘panencephalopathic type' disease. Neurology 1989 0ct;39(10)21337-42. Narang HK, Perry RH. High incidence of Creutzfeldt- Jakob disease in Northern England [abstract]. Neuropathol Appl Neurobiol l990;16(3):259. Ohtani Y, Tamai Y. [A study of ganglioside alterations in the nervous system of Creutzfeldt—Jakob disease-- analysis in two human cases and experimentally affected rodents]. Seishin Shinkeigaku Zasshi 1988; 90(1):l-l9. (Jpn). Ortega Calvo M. Martinez Manzanares C. [Is creutzfeldt—jakob disease the first sign of a biological heterodoxy?] Med Clin 1989:9203): 497-500. (Spa). Tateishi 1. [Review: experimental studies on Creutzfeldt-Jakob disease]. Jikken Dobutsu 1989 Oct;38(4):289-92. (Jpn). Whitwam L. Searching for a link. Nurs Times 1990 Feb l4-20;86(7):23. Gerstmann-Straussler Syndrome Baker H. Autosomal dominant spongiform encephalopathy. Biomed Pharmacother 1986; 40(2):77. Baker HF. Duchen LW, Jacobs IM. Ridley RM. Spongiform encephalopathy transmitted experimentally from Creutzfeldt-Jakob and familial Gersu'nann-Su’aussler-Scheinker diseases. Brain 1990 Dec;ll3(Pt 6):1891-909. Baron H. Baron-Van Evercooren A. Brucher IM. Antiserum to scrapie-associated fibril protein reacts with amyloid plaques in familial transmissible dementia. J Neuropathol Exp Neurol l988;47(2): 158-65. Hsiao K. Baker HF, Crow TJ. Poulter M. Owen F. Terwilliger JD. Westaway D. Ott J. Prusiner SB. Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature 1989; 338(6213):342-5. Marx J. Human brain disease recreated in mice [news]. Science 1990 Dec l4;250(4987):1509-10. Tateishi J. Kitamoto T. Doh-ura K. Sakaki Y. Steinmetz G. Tranchant C. Warter IM. Heldt N. Immunochemical. molecular genetic. and transmission studies on a case of Gerstmann- Strzaussler-Scheinker syndrome. Neurology 1990 Oct;40(10):1578-81. Tateishi J. Kitamoto T. Hashiguchi H. Shii H. Gerstmann-Strzaussler-Scheinker disease: immunohistological and experimental studies. Ann Neurol 1988 Jul;24(l):35-40. 8 Books and Book Chapters Asher DM. Gibbs CJ Jr. Gajdusek DC. Slow viral infections: safe handling of the agents of subacute spongiform encephalopathies. In: Miller BM. et al., editors. Laboratory safety: principles and practices. Washington: American Society for Microbiology; 1987. p. 59-71. Beck E. Daniel PM. Neuropathology of transmissible spongiforrn encephalopathies. In: Prusiner SB. McKinley MP. editors. Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. San Diego: Academic Press; 1987. p. 331-86. Davanipour Z. Epidemiology. In: Bastian F0, editor. Creutzfeldt-Jakob disease and other transmissible spongiform encephalopathies. St Louis: Mosby- Yearbook: 1991. p. 131-52. Gajdusek CD. Subacute spongiforrn encephalopathies: transmissible cerebral arnyloidoses caused by unconventional viruses. In: Fields BN. Knipe DM. editors. Fields virology. 2nd ed. Vol. 2. New York: Raven Press; 1990. p. 2289-324. Masters CL. Epidemiology of Creutzfeldt-Jakob Disease: studies on the natural mechanisms of transmission. In: Prusiner SB. McKinley MP, editors. Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. San Diego: Academic Press; 1987. p. 511-34. Prusiner SB. An introduction to scrapie and creutzfeldt-jakob disease research. In: Prusiner SB. McKinley MP. editors. Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. San Diego: Academic Press; 1987. p. 1-16. Southwood R. Report of the working party on bovine spongiform encephalopathy. Stanmore (Middlesex): UK Dept. of Health, Leaflets Unit; 1989. 35 p. Tateishi J. I-Iikita K. Kitarnoto T. Nagara H. Experimental Creutzfeldt—Jakob disease induction of arnyloid plaques in rodents. In: Prusiner SB, McKinley MP. editors. Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. 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