Source: http://www.asmscience.org/content/book/10.1128/9781555817831.chap14
Timestamp: 2019-04-19 10:44:46+00:00

Document:
This chapter considers viruses that cause subacute and chronic encephalitis in immunocompetent persons. Subacute sclerosing panencephalitis (SSPE) is a rare late manifestation of measles virus infection, developing years after natural measles infection. During the first half of the 20th century several papers were written describing the clinical and pathological features of SSPE, but different pathological variants were described as if they were different diseases. The absence of recent reports suggests that progressive rubella panencephalitis (PRP) may have essentially disappeared. The reasons for its appearance and disappearance are mysterious. It shares certain features with SSPE. The great majority of cases of HAM/TSP are now known to be caused by human T-cell leukemia virus type 1 (HTLV-1), with occasional cases caused by a related retrovirus, HTLV- 2. Rasmussen&apos;s encephalitis is a condition of chronic intractable epilepsy in children that is associated with signs of progressive hemispheral dysfunction and unilateral brain atrophy. Resected brain tissue can demonstrate perivascular infiltrates and glial nodules, usually in the absence of clinical features of encephalitis. Viral studies are performed to exclude other causes of encephalitis. In contrast to encephalitis lethargica, however, Vilyuisk encephalitis appears to be an endemic disease, and the chronic phase has more widespread central nervous system (CNS) destruction. Experimental models have been useful to examine the pathogenesis of neurodevelopmental abnormalities and also behavioral defects, the role of immune-mediated damage, and the development of chronic Borna disease.
Close-up of fixed, sliced brain from a case of longstanding SSPE. The granular, rubbery consistency and discoloration of the white matter are evident.
Sliced cerebrum from the case in Fig. 14.1. There is loss of white and gray matter, ventricular dilatation, and narrowed corpus callosum.
Cortical inflammation consisting predominantly of lymphocytes and macrophages in a case of SSPE of 3 months&apos; duration. Hematoxylin and eosin stain.
Immunoperoxidase staining using antimeasles antibody to demonstrate measles virus antigen in (a) neuron cytoplasm, processes, and nucleus and (b) oligodendrocyte nuclei in a case of SSPE of 9 months&apos; duration. Counterstained with hematoxylin.
White matter from a case of long-standing SSPE. There is an increase in glial nuclei and widened perivascular spaces containing reticulin fibers, but little inflammation. Hematoxylin and eosin stain.
Electron micrograph of intranuclear paramyxovirus tubules from the temporal lobe at autopsy from a case of SSPE. × 125,000. Courtesy of J. E. Richmond.
Low-power view of myelin-stained section of the spinal cord in HAM/TSP. Note pallor of staining in the lateral corticospinal tracts and posterior columns.
Inflammation in the spinal cord in HAM/TSP with small veins and cord parenchyma infiltrated with mononuclear inflammatory cells.
Meningeal thickening and inflammation and slight perivascular and meningeal inflammation in HAM/TSP.
MRI of Rasmussen&apos;s syndrome. Coronal FLAIR image demonstrates hyperintensity in the cortical and subcortical regions of the medial superior frontal lobe. Courtesy of Dennis Spencer, Yale University School of Medicine.
1. Abe, T.,, T. Nukada,, H. Hatanaka,, M. Tajima,, M. Hiraiwa,, and H. Ushijima. 1983. Myoclonus in a case of suspected progressive rubella panencephalitis. Arch. Neurol. 40: 98– 100.
2. Agnarsdottir, G., 1977. Subacute sclerosing panencephalitis, p. 22– 29. In A. P. Waterson (ed.), Recent Advances in Clinical Virology. Churchill Livingstone, London, United Kingdom.
3. Allen, I. V.,, S. McQuaid,, J. McMahon,, J. Kirk,, and R. McConnell. 1996. The significance of measles virus antigen and genome distribution in the CNS in SSPE for mechanisms of viral spread and demyelination. J . Neuropathol. Exp. Neurol. 55: 471– 480.
4. Andrews, P. I.,, M. A. Dichter,, S. F. Berkovic,, M. R. Newton,, and J . O. McNamara. 1996. Plasmapheresis in Rasmussen's encephalitis. Neurology 46: 242– 246.
5. Anlar, B .,, I. Saatci,, G. Kose,, and K. Yalaz. 1996. MRI findings in subacute sclerosing panencephalitis. Neurology 47: 1278– 1283.
6. Anlar, B.,, K. Yalaz,, F. Oktem,, and G. Kose. 1997. Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular a-interferon. Neurology 48: 526– 528.
7. Anlar, B.,, F. Soylemezoglu,, B. Elibol,, T. Dalkara,, S. Aysun,, G. Kose,, D. Belen,, and K. Yalaz. 1999. Apoptosis in brain biopsies of subacute sclerosing panencephalitis patients. Neuropediatrics 30: 239– 242.
8. Asher, D. M., 1997. Slow viral infection, p. 199– 221. In W. M. Scheld,, R. J. Whitley,, and D. T. Durack (ed.), Infections of the Central Nervous System. Lippincott-Raven, Philadelphia, Pa..
9. Bancher, C.,, H. Leitner,, K. Jellinger,, H. Eder,, U. Setinek,, P. Fischer,, J. Wegiewl,, and H. M. Wisneiwski. 1996. On the relationship between measles virus and Alzheimer neurofibrillary tangles in subacute sclerosing panencephalitis. Neurobiol. Aging 17: 527– 533.
10. Baram, T. Z.,, I. Gonzalez-Gomez,, Z.-D. Xie,, D. Yao,, E. H. Gilles,, M. D. Nelson, Jr.,, H. T. Nguyen,, and J. Peters. 1994 - Subacute sclerosing panencephalitis in an infant: diagnostic role of viral genome analysis. Ann. Neurol. 36: 103– 108.
11. Berger, J . R.,, S. Raffanti,, A. Svenningsson,, M. McCarthy,, S. Snodgrass,, and L. Resnick. 1991. The role of HTLV in HIVI neurologic disease. Neurology 41: 197– 202.
12. Bien, C. G.,, J. Bauer,, T. L. Deckwerth,, H. Wiendl,, M. Deckert,, O. D. Wiestler,, J . Schramm,, C. E. Elger,, and H. Lassman. 2002a. Destruction of neurons by cytotoxic T cells: a new pathogenic mechanism in Rasmussen's encephalitis. Ann. Neurol 51: 311– 318.
13. Bien, C. G.,, H. Urbach,, M. Deckert,, J. Schramm,, O. D. Wiestler,, H. Lassman,, and C. E. Elger. 2002b. Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology. Neurobgy 58: 250– 257.
14. Billeter, M. A.,, R. Cattaneo,, P. Spielhofer,, K. Kaelin,, M. Huber,, A. Schmid,, K. Baczko,, and V. ter Meulen. 1994. Generation and properties of measles virus mutations typically associated with subacute sclerosing panencephalitis. Ann. N.Y. Acad. Sci. 724: 367– 377.
15. Black, F. L.,, R. J. Biggar,, R. B . Lai,, A. A. Baggai,, and J . P. Filho. 1996. Twenty-five years of HTLV type II follow-up with a possible case of tropical spastic paraparesis in the Kayapo, a Brazilian Indian tribe. AIDS Res. Hum. Retrovir. 12: 1623– 1627.
16. Blattner, W. A., 1997. Human lymphotropic viruses: HTLV-I and HTLV-II, p. 683– 705. In D. D. Richman,, R. J. Whitle,, and F. G. Hayden (ed.), Clinical Virology. Churchill Livingstone, New York, N.Y..
17. Bode, L.,, P. Reckwald,, W. E. Severus,, R. Stoyloff,, R. Ferszt,, D. E. Dietrich,, and H. Ludwig. 2001. Borna disease virusspecific circulating immune complexes, antigenemia, and free antibodies—the key marker triplet determining infection and prevailing in severe mood disorders. Mol. Psychiatry 6: 481– 491.
18. Booss, J .,, and J. H. Kim,. 1990. Evidence for a viral etiology of multiple sclerosis, p. 41– 61. In S. D. Cook (ed.), Handbook of Multiple Sclerosis. Marcel Dekker, New York, N.Y..
19. Bouteille, M.,, C. Fontaine,, C. Vedrenne,, and J. Delarue. 1965. Sur un cas d'encephalite subaigiie a inclusions: etude anatomoclinique et ultrastructurale. Rev. Neurol. 113: 454– 458.
20. Brismar, J .,, G. G. Gascon,, K. V. von Steyem,, and S. Bohlega. 1996. Subacute sclerosing panencephalitis: evaluation with CT and MR. Am. J . Neuroradiol. 17: 761– 772.
21. Brown, H. R.,, N. L. Goller,, R. D. Rudelli,, J . Dymecki,, and H. M. Wisniewski. 1989. Postmortem detection of measles virus in non-neural tissues in subacute sclerosing panencephalitis. Ann. Neurol. 26: 263– 268.
22. Buchholz, C. J.,, D. Roller,, P. Devaux,, C. Mumenthaler,, J. Schneider-Schaulies,, W. Braun,, D. Gerlier,, and R. Cattaneo. 1997. Mapping of the primary binding site of measles virus to its receptor CD46. J . Biol. Chem. 272: 22072– 22079.
23. Carbone, K. M. 2001. Borna disease virus and human disease. Clin. Microbiol. Rev. 14: 513– 527.
24. Carney, P. R. 2001. Rasmussen's syndrome: intractable epilepsy and progressive neurological deterioration from a unilateral central nervous system disease. CNS Spectr. 6: 398, 409– 416.
25. Case Records of the Massachusetts General Hospital. 1998. Case 15-1998. N. Engl. J. Med. 338: 1448– 1456.
26. Cathomen, T.,, B. Mrkic,, D. Spehner,, R. Drillien,, R. Naef,, J. Pavlovic,, A. Aguzzi,, M. A. Billeter,, and R. Cattaneo. 1998. A matrix-less measles virus is infectious and elicits extensive cell fusion: consequences for propagation in the brain. EMBO J. 17: 3899– 3908.
27. Cattaneo, R.,, A. Schmid,, M. A. Billeter,, R. D. Sheppard,, and S. A. Udem. 1988. Multiple viral mutations rather than host factors cause defective measles virus gene expression in a subacute sclerosing panencephalitis cell line. J . Virol. 62: 1388– 1397.
28. Centers for Disease Control and Prevention. 1993. Recommendations for counseling persons infected with human Tlymphotrophic diseases, types I and II. Morb. Mortal. Wkly. Rep. 42( RR-9): 1– 13.
29. Chen, T. T.,, I. Watanabe,, W. Zeman,, and J. Mealey. 1969. Subacute sclerosing panencephalitis: propagation of measles virus from brain biopsy in tissue culture. Science 163: 1193– 1194.
30. Cobb, W., and D. Hill. 1950. Electroencephalogram in subacute progressive encephalitis. Brain 73: 392– 404.
31. Commissionon Classification and Terminology of the International League Against Epilepsy. 1989. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 30: 389– 399.
32. Connolly, J. H.,, I. V. Allen,, L. J. Hurwitz,, and J . H. D. Millar. 1967. Measles-virus antibody and antigen in subacute sclerosing panencephalitis. Lancet i: 542– 544.
33. Corsellis, J. 1951. Subacute sclerosing panencephalitis: clinical and pathological report of 2 cases. J . Ment. Sci. 97: 570– 583.
34. Coyle, P. K.,, and J. S. Wolinsky. 1981. Characterization of immune complexes in progressive rubella panencephalitis. Ann. Neurol. 9: 557– 562.
35. Cremer, N. E.,, L. S. Oshiro,, M. L. Wei,, E. H. Lennette,, H. H. Itabashi,, and L. Camay. 1975. Isolation of rubella virus from brain in chronic progressive panencephalitis. J. Gen. Virol. 29: 143– 153.
36. Cupler, E. J.,, M. Leon-Monzon,, J . Miller,, C. Semino-Mora,, T. L. Anderson,, and M. C. Dalakas. 1996. Inclusion body myositis in HIV-1 and HTLV-1 infected patients. Brain 119: 1887– 1893.
37. Cutewa, J.,, and E. Osetowska,. 1961. A chronic form of subacute sclerosing panencephalitis (a case with a history of 5 years): clinical and pathological study, p. 386– 404. In L. van Bogaert,, J. Radermecker,, J. Hozay,, and A. Lowenthal (ed.), Encephalitides. Elsevier, Amsterdam, The Netherlands.
38. Dorig, R. E.,, A. Marcil,, A. Chopra,, and C. D. Richardson. 1993. The human CD46 molecule is a receptor for measles virus (Edmonston strain). Cell 75: 295– 305.
39. Dubois-Dalcq, M.,, L. H. Barbosa,, and J . L. Sever. 1974. Comparison between productive and latent subacute sclerosing panencephalitis viral infection in vitro. An electron microscopic and immunoperoxidase study. Lab. Investig. 30: 241– 250.
40. Dyken, P. R. 1985. Subacute sclerosing panencephalitis: current status. Neurol. Clin. 3: 179– 196.
41. Dyken, P. R. 1989. Changing character of subacute sclerosing panencephalitis in the United States. Pediatr. Neurol. 5: L339– 341.
42. Esiri, M. M.,, D. R. Oppenheimer,, B. Brownell,, and M. Haire. 1982. Distribution of measles antigen and immunoglobulincontaining cells in the CNS in subacute sclerosing panencephalitis (SSPE) and atypical measles encephalitis. J . Neurol. Sci. 53: 29– 43.
43. Fournier, J . G.,, M. Tardieu,, P. Lebon,, O. Robain,, G. Ponsot,, S. Rozenblatt,, and M. Bouteille. 1985. Detection of measles virus RNA in lymphocytes from peripheral-blood and brain perivascular infiltrates of patients with subacute sclerosing panencephalitis. N. Engl.J. Med. 313: 910– 915.
44. Freeman, J. F. 1969. The clinical spectrum and early diagnosis of Dawson's encephalitis. With preliminary notes on treatment. J . Pediatr. 75: 590– 603.
45. Gessain, A.,, F. Barin,, J. C. Vernant,, O. Gout,, L. Maurs,, A. Calender,, and G. deThe. 1985. Antibodies to human Tlymphotropic virus type-I in patients with tropical spastic paraparesis. Lancet ii: 407– 410.
46. Godec, M. S.,, D. M. Asher,, P. T. Swoveland,, Z. A. Eldah,, S. M. Feinstone,, L. G. Goldfarb,, C. J . Gibbs, Jr.,, and D. C. Gajdusek. 1990. Detection of measles virus genomic sequences in SSPE brain tissue by polymerase chain reaction. J. Med. Virol. 30: 237– 244.
47. Gokcil, Z.,, Z. Odbasi,, S. Demirkaya,, E. Eroglu,, and O. Vural. 1999. a-Interferon and isoprinisine in adult-onset subacute sclerosing panencephalitis. J . Neurol. Sci. 162: 62– 64.
48. Goldfarb, L. G.,, and D. C Gajdusek. 1992. Viliusk encephalomyelitis in the Iakut people of Siberia. Brain 115: 961– 978.
49. Gosztonyi, G.,, and H. Ludwig. 1984. Borna disease of horses. An immunohistological and virological study of naturally infected animals. Acta Neuropathol. 64: 213– 221.
50. Gout, O.,, M. Baulac,, A. Gessain,, F. Semah,, F. Saal,, J . Peries,, C. Cabrol,, C. Foucault-Fretz,, D. Laplane,, F. Sigaux,, and G. de The. 1990. Rapid development of myelopathy after HTLV-I infection acquired by transfusion during cardiac transplantation. N. Engl. J . Med. 322: 383– 388.
51. Gout, O.,, A. Gassain,, M. Iba-Zizen,, S. Kouzan,, F. Bolgert,, G. de The,, and O. Lyon-Caen. 1991. The effect of zidovudine on chronic myelopathy associated with HTLV-I. J . Neurol. 238: 108– 110.
52. Graves, M. C. 1984 - Subacute sclerosing panencephalitis. Neurol. Clin. 2: 267– 280.
53. Green, S. H.,, and J . D. Wirtschafter. 1973. Ophthalmoscopic findings in subacute sclerosing panencephalitis. Br. J . Ophthalmol. 57: 780– 787.
54. Greenfield, J. 1950. Encephalitis and encephalomyelitis in England and Wales during the last decade. Brain 73: 141– 166.
55. Greenlee, J . E.,, and J . W. Rose. 2000. Controversies in neurological infectious diseases. Semin. Neurol. 20: 375– 386.
56. Greten, T. E.,, J. E. Slansky,, R. Kubota,, S. S. Soldan,, E. M. Jaffee,, T. P. Leist,, D. M. Pardoll,, S. Jacobson,, and J. P. Schneck. 1998. Direct visualization of antigen-specific T cells: HTLV-1 Tax 11-19-specific CD8(+) T cells are activated in peripheral blood and accumulate in cerebrospinal fluid from HAM-TSP patients. Proc. Natl. Acad. Sci. USA 95: 7568– 7573.
57. Griffin, D. E.,, and W. J . Bellini,. 1996, Measles virus, p. 1267– 1312. In B. N. Fields,, D. M. Knipe,, and P. M. Howley (ed.), Fields Virohgy. Raven, Philadelphia, Pa..
58. Gutewa, J.,, and E. Osetowska,. 1961. A chronic form of subacute sclerosing panencephalitis (a case with a history of 5 years), clinical and pathological study, p. 386– 404. In L. Bogaert,, J. Radermecker,, J. Hozay,, and A. Lowenthal (ed.), Encephalitides. Elsevier, Amsterdam, The Netherlands.
59. Hall, W. W.,, and P. W. Choppin. 1981. Measles-virus proteins in the brain tissue of patients with subacute sclerosing panencephalitis: absence of the M protein. N. Engl. J . Med. 304: 1152– 1155.
60. Hall, W. W.,, R. A. Lamb,, and P. W. Choppin. 1979. Measles and subacute sclerosing panencephalitis virus proteins: lack of antibodies to the M protein in patients with subacute sclerosing panencephalitis. Proc. Natl. Acad. Sci. USA 76: 2047– 2051.
61. Halsey, N. A.,, J . F. Modlin,, J. T. Jabbour,, L. Dubey,, D. L. Eddins,, and D. D. Ludwig. 1980. Risk factors in subacute sclerosing panencephalitis: a case-control study. Am. J . Epidemiol. 111: 415– 424.
62. Hanissian, A. S.,, J . T. Jabbour,, S. de Lamerens,, J . H. Garcia,, and L. Horta-Barbosa. 1972. Subacute encephalitis and hypogammaglobulinemia. Am. J . Dis. Child. 123: 151– 155.
63. Hara, H.,, M. Morita,, T. Iwaki,, T. Hatae,, Y. Itoyama,, T. Kitamoto,, S. Akizuki,, I. Goto,, and T. Watanabe. 1994. Detection of human T lymphotrophic virus type I (HTLV-I) proviral DNA and analysis of T cell receptor V beta CDR3 sequences in spinal cord lesions of HTLV-I-associated myelopathy-tropical spastic paraparesis. J . Exp. Med. 180: 831– 839.
64. Harrington, W. J ., Jr.,, W. A. Sheremata,, S. R. Snodgrass,, S. Emerson,, S. Phillips,, and J. R. Berger. 1991. Tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM): treatment with an anabolic steroid danazol. AIDS Res. Hum. Retrovir. 7: 1031– 1034.
65. Hart, Y. M.,, M. Cortez,, F. Andermann,, P. Hwang,, D. R. Fish,, O. Dulac,, K. Silver,, N. Fejerman,, H. Cross,, A. Sherwin,, and R. Caraballo. 1994- Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients. Neurology 44: 1030– 1036.
66. Herndon, R. M.,, and L. J. Rubinstein. 1968. Light and electron microscopy observations on the development of viral particles in the inclusions of Dawson's encephalitis (subacute sclerosing panencephalitis). Neurology 18: 8– 20.
67. Hollsberg, P.,, and D. A. Hafler. 1993. Pathogenesis of diseases induced by human lymphotropic virus Type I infection. N. Engl. J. Med. 328: 1173– 1182.
68. Horta-Barbosa, L.,, D. A. Fuccillo,, J . L. Sever,, and W. Zeman. 1969, Subacute sclerosing panencephalitis: isolation of measles virus from a brain biopsy. Nature 221: 974.
69. Huttenlocher, P. R.,, and R. H. Mattson. 1979. Isoprinosine in subacute sclerosing panencephalitis. Neurology 29: 763– 771.
70. Ichiyama, T.,, S. Houdou,, T. Kisa,, K. Ohno,, and K. Takeshita. 1990. Varicella with delayed hemiplegia. Pediatr. Neurol. 6: 279– 281.
71. Ikeda, K.,, H. Akiyama,, H. Kondo,, T. Arai,, N. Arai,, and S. Yagishita. 1995. Numerous glial fibrillary tangles in oligodendroglia in cases of subacute sclerosing panencephalitis with neurofibrillary tangles. Neurosci. Lett. 194: 133– 135.
72. Izumo, S.,, I. Goto,, Y. Itoyama,, T. Okajma,, S. Watanabe,, Y. Kuroda,, S. Araki,, M. Mori,, S. Nagataki,, S. Matsukura,, T. Akamine,, M. Nakagawa,, I. Yamamoto,, and M. Osame. 1996. Interferon-alpha is effective in HTLV-I myelopathy: a multicenter, randomized, double-blind, controlled trial. Neurology 46: 1016– 1021.
73. Jabbour, J . T.,, D. A. Duenas,, J . L. Sever,, H. M. Krebs,, and L. Horta-Barbosa. 1972. Epidemiology of subacute sclerosing panencephalitis (SSPE). A report of the SSPE registry. JAMA 220: 959– 962.
74. Jabbour, J. X.,, J. H. Garcia,, H. Lemmi,, J. Ragland,, D. A. Duenas,, and J. L. Sever. 1969. Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. JAMA 207: 2248– 2254.
75. Jacobson, S.,, T. Lehky,, M. Nishimura,, S. Robinson,, D. E. McFarlin,, and S. Dhib-Jalbut. 1993. Isolation of HTLV-II from a patient with chronic, progressive neurological disease clinically indistinguishable from HTLV-1-associated myelopathy/ tropical spastic paraparesis. Ann. Neurol. 33: 392– 396.
76. Jan, J. E.,, A. J. Tingle,, G. Donald,, M. Kettyls,, W. St. J. Buckler,, and C. L. Dolman* 1979. Progressive rubella panencephalitis: clinical course and response to isoprinosine. Dev. Med. Child Neurol. 21: 648– 652.
77. Jay, V.,, L. E. Becker,, H. Otsubo,, M. Cortez,, P. Hwang,, H. J . Hoffman,, and M. Zielenska. 1995. Chronic encephalitis and epilepsy (Rasmussen's encephalitis): detection of cytomegalovirus and herpes simplex virus 1 by the polymerase chain reaction and in situ hybridization. Neurology 45: 108– 117.
78. Jeffery, K. J .,, K. Usuku,, S. E. Hall,, W. Matsumoto,, G. P. Taylor,, J. Procter,, M. Bunce,, G. S. Ogg,, K. I. Welsh,, J. N. Weber,, A. L. Lloyd,, M. A. Nowak,, M. Nagai,, D. Kodama,, S. Izumo,, M. Osame,, and C. R. Bangham. 1999. HLA alleles determine human T-lymphotropic virus-I (HTLV-I) proviral load and the risk of HTLV-I-associated myelopathy. Proc. Nad. Acad. Sci. USA 96: 3848– 3853.
79. Jenis, E. J .,, M. R. Kneiser,, P. A. Rothanse,, G. E. Jensen,, and R. M. Scott. 1973. Subacute sclerosing panencephalitis— immunostructural localization of measles virus antigen. Arch. Pathol. 95: 81– 89.
80. Jones, C. E.,, P. R. Dyken,, P. R. Huttenlocher,, J. T. Jabbour,, and K. W. Maxwell. 1982. Inosiplex therapy in subacute sclerosing panencephalitis. A multicentre, nonrandomised study in 98 patients. Lancet i: 1034– 1037.
81. Kira, J.-L.,, K. Fujihara,, Y. Itoyama,, I. Goto,, and K. Hasuo. 1991. Leukoencephalopathy in HTLV-I-associated myelopathy/ tropical spastic paraparesis: MRI analysis and a two year followup study after corticosteroid therapy. J . Neurol. Sci. 106: 41– 49.
82. Kirk, J .,, A. L. Zhou,, S. McQuaid,, S. L. Cosby,, and I. V. Allen. 1991. Cerebral endothelial cell infection by measles virus in subacute sclerosing panencephalitis: ultrastructural and in situ hybridization evidence. Neuropathol. Appl. Neurobiol. 17: 289– 297.
83. Kreth, H. W.,, R. Dunker,, H. Rodt,, and R. Meyermann. 1982. Immunohistochemical identification of T-lymphocytes in the central nervous system of patients with multiple sclerosis and subacute sclerosing panencephalitis. J . Neuroimmunol. 2: 177– 183.
84. Lawrence, D. M.,, C. E. Patterson,, T. L. Gales,, J . L. D'Orazio,, M. M. Vaughn,, and G. F. Rail. 2000. Measles virus spread between neurons requires cell contact but not CD46 expression, syncytium formation, or extracellular virus production. J. Virol. 74: 1908– 1918.
85. Leach, J . P.,, D. W. Chadwick,, J. B. Miles,, and I. K. Hart. 1999. Improvement in adult-onset Rasmussen's encephalitis with long-term immunomodulatory therapy. Neurology 52: 738– 742.
86. Lehky, T. J .,, M. C. Levin,, R. Kubota,, R. N. Bamford,, A. N. Flerage,, S. S. Soldan,, T. A. Fleisher,, L. E. Top,, S. Light,, H. E. McFarland,, T. A. Waldmann,, and S. Jacobson. 1998. Reduction in HTLV-I proviral load and spontaneous lymphoproliferation in HTLV-I-associated myelopathy/tropical spastic paraparesis patients treated with humanized anti-tac. Ann. Neurol. 44: 942– 947.
87. Leon-Monzon, M.,, I. Ilia,, and M. C Dalakas. 1994. Polymyositis in patients infected with human T-cell leukemia virus type I. The role of the virus in cause of disease. Ann. Neurol. 36: 643– 649.
88. Levin, M. C.,, S. M. Lee,, F. Kalume,, Y. Morcos,, F. C. Dohan, Jr.,, K. A. Hasty,, J. C. Callaway,, J. Zunt,, D. Desiderio,, and J. M. Stuart. 2002. Autoimmunity due to molecular mimicry as a cause of neurological disease. Nat. Med. 8: 509– 513.
89. Lin, F. H.,, and H. Thormar. 1980. Absence of M protein in a cell-associated subacute sclerosing panencephalitis virus. Nature 285: 490– 492.
90. Link, H.,, M. Panelius,, and A. A. Salmi. 1973. Immunoglobulins and measles antibodies in subacute sclerosing panencephalitis. Arch. Neurol. 28: 23– 30.
91. Lipkin, W. I.,, M. Hornig,, and T. Briese. 2001. Borna disease virus and neuropsychiatric disease—a reappraisal. Trends Microbiol. 9: 295– 298.
92. Malamud, N.,, W. Haymaker,, and H. Pinkerton. 1950. Inclusion encephalitis with clinicopathologic report of 3 cases. Am. J . Pathol. 26: 133– 153.
93. Mandybur, T. I.,, A. S. Nagpaul,, Z. Pappas,, and W. J . Niklowitz. 1977. Alzheimer neurofibrillary change in subacute sclerosing panencephalitis. Ann. Neurol. 1: 103– 107.
94. Manns, A.,, M. Hisada,, and L. LaGrenade. 1999. Human Tlymphotropic virus type I infection. Lancet 353: 1951– 1958.
95. McArthur, J. C.,, J. W. Griffin,, D. R. Cornblath,, D. E. Griffin,, T. Tesoriero,, R. Kunel,, C. J. Gibbs,, H. Farzadegan,, and R. T. Johnson. 1990. Steroid-responsive myeloneuropathy in a man dually infected with HIV-I and HTLV-I. Neurology 40: 938– 944.
96. McKendall, R. R.,, J . Oas,, and M. D. Lairmore. 1991. HTLV- 1 associated myelopathy endemic in Texas-born residents and isolation of virus from CSF cells. Neurology 41: 831– 836.
97. McLachlan, R.S.,, J . P. Girvin,, W. T. Blume,, and H. Reichman. 1993. Rasmussen's chronic encephalitis in adults. Arch. Neurol. 50: 269– 274.
98. McLachlan, R. S.,, S. Levin,, and W. T. Blume. 1996. Treatment of Rasmussen's syndrome with ganciclovir. Neurology 47: 925– 928.
99. McLean, C. A.,, C. L. Masters,, V. A. Vladimirtsev,, I. A. Prokhorova,, L. G. Goldfarb,, D. M. Asher,, A. I. Vladimirtsev,, V. P. Alekseev,, and D. C. Gajdusek. 1997. Viiiuisk encephalomyelitis—review of the spectrum of pathological changes. Neuropathol. Appl. Neurobiol. 23: 212– 217.
100. McQuaid, S.,, I. V. Allen,, J . McMahon,, and J . Kirk. 1994. Association of measles virus with neurofibrillary tangles in subacute sclerosing panencephalitis: a combined in situ hybridization and immunocytochemical investigation. Neuropathol. Appl. Neurobiol. 20: 103– 110.
101. Modlin, J. E.,, N. A. Halsey,, D. L. Eddins,, J. L. Conrad,, J. T. Jabbour,, L. Chien,, and H. Robinson. 1979. Epidemiology of subacute sclerosing panencephalitis. J . Pediatr. 94: 231– 236.
102. Moore, G. R.,, U. Traugott,, L. C. Scheinberg,, and C. S. Raine. 1989. Tropical spastic paraparesis: a model of virus-induced, cytotoxic T-cell-mediated demyelination? Ann. Neurol. 26: 523– 530.
103. Murphy, E. L.,, J . Fridey,, J . W. Smith,, J . Engstrom,, R. A. Sacher,, K. Miller,, J . Gibble,, J . Stevens,, R. Thomson,, D. Hansma,, J . Kaplan,, R. Khabbaz,, and G. Nemo. 1997. HTLV-associated myelopathy in a cohort of HTLV-I and HTLV-II-infected blood donors. The REDS investigators. Neurobgy 48: 315– 320.
104. Nagano, I.,, S. Nakamura,, M. Yoshioka,, and K. Kogure. 1991. Immunocytochemical analysis of the cellular infiltrate in brain lesions in subacute sclerosing panencephalitis. Neurobgy 41: 1639– 1642.
105. Nakamura, T. 2000. Immunopathogenesis of HTLV-l-associated myelopathy/tropical spastic paraparesis. Ann. Med. 32: 600– 607.
106. Naniche, D.,, G. Varior-Krishnan,, F. Cervoni,, T. F. Wild,, B. Rossi,, C. Rabourdin-Combe,, and D. Gerlier. 1993. Human membrane cofactor protein (CD46) acts as a cellular receptor for measles virus. J . Virol. 67: 6025– 6032.
107. Narayan, O.,, S. Herzog,, K. Frese,, H. Scheefers,, and R. Rott. 1983. Behavioral disease in rats caused by immunopathological responses to persistent borna virus in the brain. Science 220: 1401– 1403.
108. Ning, X.,, M. Ayata,, M. Kimura,, K. Komase,, K. Furukawa,, T. Seto,, N. Ito,, M. Shingai,, I. Matsunaga,, T. Yamano,, and H. Ogura. 2002. Alterations and diversity in the cytoplasmic tail of the fusion protein of subacute sclerosing panencephalitis virus strains isolated in Osaka, Japan. Virus Res. 86: 123– 131.
109. Ogawa, M.,, H. Okubo,, Y. Tsuji,, N. Yasui,, and K. Someda. 1973. Chronic progressive encephalitis occurring 13 years after Russian spring-summer encephalitis. Neurol. Sci. 19: 363– 373.
110. Oguni, H.,, E. Andermann,, and T. B. Rasmussen. 1992. The syndrome of chronic encephalitis and epilepsy. A study based on the MNI series of 48 cases. Adv. Neurol. 57: 419– 433.
111. Osame, M.,, and J . C McArthur,. 1992. Neurologic manifestations of infection with human T cell lymphotropic virus type I, p. 1331– 1339. In A. K. Asbury,, G. M. McKhann,, and W. I. McDonald (ed.), Diseases of the Nervous System. Clinical Neurobiology, 2nd ed. W.B. Saunders, Philadelphia, Pa..
112. Osame, M.,, M. Matsumoto,, K. Usuku,, S. Izumo,, N. Ijichi,, H. Amitani,, M. Tara,, and A. Igata. 1987. Chronic progressive myelopathy associated with elevated antibodies to human Tlymphotropic virus type I and adult T-cell leukemialike cells. Ann. Neurol. 21: 117– 122.
113. Oyanagi, S.,, V. ter Meulen,, M. Katz,, and H. Koprowski. 1971. Comparison of subacute sclerosing panencephalitis and measles viruses: an electron microscope study. J. Virol. 7: 176– 187.
114. Ozden, S.,, A. Gessain,, O. Gout,, and J . MikoL 2001. Sporadic inclusion body myositis in a patient with human T cell leukemia virus type I-associated myelopathy. Clin. Infect. Dis. 32: 510– 514.
115. Ozturk, A.,, C. Gurses,, B. Baykan,, A. Gokyigit,, and M. Eraksoy. 2002. Subacute sclerosing panencephalitis: clinical and magnetic resonance imaging evaluation of 35 patients. J. Child. Neurol. 17: 25– 29.
116. Paula-Barbosa, M. M.,, R. Brito,, C. A. Silva,, R. Faria,, and C. Cruz. 1979. Neurofibrillary changes in the cerebral cortex of a patient with subacute sclerosing panencephalitis (SSPE). Acta Neuropathol. 48: 157– 160.
117. Payne, E. E.,, J. V. Baublis,, and H. H. Itabashi. 1969. Isolation of measles virus from cell cultures of brain from a patient with subacute sclerosing panencephalitis. N. Engl. J . Med. 281: 585– 589.
118. PeBenito, R.,, S. H. Naqvi,, M. M. Area,, and R. Schubert. 1997. Fulminating subacute sclerosing panencephalitis: case report and literature review. Clin. Pediatr. 36: 149– 154.
119. Perier, O.,, L. Thiry,, J. J . Vanderhaegen,, and S. Pelc. 1968. Attempts at experimental transmission and electron microscopic observations in subacute sclerosing panencephalitis. Neurology 18: 138– 143.
120. Perrin, L. H.,, and M. B. A. Oldstone. 1977. The formation and fate of virus antigen-antibody complexes. J. Immunol. 118: 316– 322.
121. Petrov, P. A. 1970. V. Vilyuisk encephalitis in the Yakut Republic (USSR). Am. J . Trop. Med. Hyg. 19: 146– 150.
122. Power, C.,, S. D. Poland,, W. T. Blume,, J. R. Girvin,, and G. P. A. Rice. 1990. Cytomegalovirus and Rasmussen's encephalitis. Lancet 336: 1282– 1284.
123. Puccioni-Sohler, M.,, M. Rios,, S. M. E Carvalho,, R. R. Goncalves,, C. Oliveira,, R. B. Correa,, S. Novis,, P. D. Oliveira,, and C. Bianco. 2001. Diagnosis of HAM-TSP based on CSF proviral DNA and HTLV-I antibody index. Neurobgy 57: 725– 727.
124. Rasmussen, T.,, J. Olszewski,, and J. Lloyd-Smith. 1958. Focal seizures due to chronic localized encephalitis. Neurobgy 8: 435– 445.
125. Reyes, M. G.,, R. Fresco,, S. Chokroverty,, and E. Q. Salud. 1976. Viruslike particles in granulomatous angiitis of the central nervous system. Neurology 26: 797– 799.
126. Risk, W. S.,, and F. S. Haddad. 1979. The variable natural history of subacute sclerosing encephalitis. A study of 118 cases from the Middle East. Arch. Neurol. 36: 610– 614.
127. Risk, W. S.,, F.S. Haddad,, and R. Chemali. 1978. Substantial long-term improvement in subacute sclerosing panencephalitis. Six cases from the Middle East and a review of the literature. Arch. Neurol. 35: 494– 502.
128. Rittner, C.,, E. M. Meier,, B. Stradmann,, C. M. Giles,, R. Kochling,, E. Mollenhauer,, and H. W. Kreth. 1984. Partial C4 deficiency in subacute sclerosing panencephalitis. Immunogenetics 20: 407– 415.
129. Rogers, S. W.,, P. L Andrews,, L. C. Gahring,, T. Whisenand,, K. Cauley,, B . Crain,, T. E. Hughes,, S. E Heinemann,, and J . O. McNamara. 1994. Autoantibodies to glutamate receptor Glu R3 in Rasmussen's encephalitis. Science 265: 648– 651.
130. Rott, R.,, S. Herzog,, B . Fleischer,, A. Winokur,, J . Amsterdam,, W. Dyson,, and H. Koprowski. 1985. Detection of serum antibodies to Borna disease virus in patients with psychiatric disorders. Science 228: 755– 756.
131. Rudge, P.,, A. AH,, and J . K. Cruickshank. 1991. Multiple sclerosis, tropical spastic paraparesis and HTLV-I infection in Afro- Carribbean patients in the United Kingdom. J . Neurol. Neurosurg. Psychiatr. 54: 689– 694.
132. Saha, V.,, T. J. John,, P. Mukundan,, C. Gnanamuthu,, S. Prabhakar,, G. Arjundas,, Z. A. Sayeed,, G. Kumaresan,, and K. Srinivas. 1990. High incidence of subacute sclerosing panencephalitis in South India. Epidemiol. Infect. 104: 151– 156.
133. Said, G.,, C. Goulon-Goeau,, C. Lacroix,, A. Feve,, H. Descamps,, and M. Fouchard. 1988. Inflammatory lesions of peripheral nerve in a patient with human T lymphotropic virus type Iassociated myelopathy. Ann. Neurol. 24: 275– 277.
134. Scaravilli, F.,, and F. Gray,. 1993. Opportunistic infections, p. 49– 120. In F. Gray (ed.), Atlas of the Neuropathobgy of HIV Infection. Oxford University Press, Oxford, United Kingdom.
135. Schmid, A.,, P. Spielhofer,, R. Cattaneo,, K. Baczko,, V. ter Meulen,, and M. A. Billeter. 1992. Subacute sclerosing panencephalitis is typically characterized by alterations in the fusion protein cytoplasmic domain of the persisting measles virus. Virology 188: 910– 915.
136. Schneider-Schaulies, S.,, and V. ter Meulen. 1999. P athogenic aspects of measles virus infections. Arch. ViroL Suppl. 15: 139– 158.
137. Sebire, G.,, L. Meyer,, and S. Chabrier. 1999. Varicella as a risk factor for cerebral infarction in childhood: a case-control study. Ann. Neurol. 45: 679– 680.
138. Sever, J . L. 1983. Persistent measles infection of the central nervous system: subacute sclerosing panencephalitis. Rev. Infect. Dis. 5: 467– 473.
139. Shaw, C. M.,, G. C Buchan,, and C. B. Carlson. 1967. Myxovirus as a possible etiologic agent in subacute inclusion body encephalitis. N. Engl. J. Med. 277: 511– 515.
140. Singer, K. C.,, A. E. Lang,, and O. Suchowersky. 1997. Adultonset subacute sclerosing panencephalitis: case reports and review of the literature. Mov. Disord. 12: 342– 353.
141. Smadja, D.,, R. Bellance,, P. Cabre,, S. Arfi,, and J. C. Vernanat. 1995. Clinical characteristics of HTLV-1 associated dermato-polymyositis. Seven cases from Martinique. Acta Neurol. Scand. 92: 206– 212.
142. Sotrel, A.,, S. Rosen,, M. Ronthal,, and D. B. Ross. 1983. Subacute sclerosing panencephalitis: an immune complex disease? Neurobgy 33: 885– 890.
143. Staeheli, P.,, C. Dauder,, J . Hausmann,, E. Ehrensperger,, and M. Schwemmie. 2000. Epidemiology of Borna disease virus. J . Gen. Virol. 81: 2123– 2135.
144. Stone, R. 2002. Siberia's deadly stalker emerges from the shadows. Science 296: 642– 645.
145. Tan, C. T.,, K. J . Goh,, K. T. Wong,, S. A. Sarji,, K. B . Chua,, N. K. Chew,, P. Murugasu,, Y. L . Toh,, H. T. Chong,, K. S. Tan,, T. Thayaparan,, S. Kumar,, and M. R. Jusoh. 2002. Relapsed and late-onset Nipah encephalitis. Ann. Neurol. 51: 703– 708.
146. Taylor, G. P.,, S. E. Hall,, S. Navarrete,, C. A. Michie,, R. Davis,, A. D. Witkover,, M. Rossor,, M. A. Nowak,, P. Rudge,, E. Matutes,, C. R. Bangham,, and J. N. Weber. 1999. Effect of lamivudine on human T-cell leukemia virus type 1 (HTLV-1) DNA copy number, T-cell phenotype, and anti-tax cytotoxic Tcell frequency in patients with HTLV-1-associated myelopathy. J.Virol. 73: 10289– 10295.
147. Tellez-Nagel, I.,, and D. H. Harter. 1966. Subacute sclerosing leukoencephalitis. I. Clinico-pathological, electron microscopic and virological observations. J . Neuropathol. Exp. Neurol. 25: 560– 581.
148. Tien, R. D.,, B . C Ashdown,, D. V. Lewis, Jr.,, M. R. Atkins,, and P. C. Burger. 1992. Rasmussen's encephalitis: neuroimaging findings in four patients. Am. J . Radiol. 158: 1329– 1332.
149. Toga, M.,, D. Dubois,, M. Berard,, M. E Tripier,, J . P. Cesorini,, and R. Choux. 1969. [Ultrastructural study of 4 cases of subacute sclerosing leukoencephalitis. J Acta Neuropathol. 14: 1– 13.
150. Tomoda, A.,, S. Shiraishi,, M. Hosoya,, A. Hamada,, and T. Miike. 2001. Combined treatment with interferon-alpha and ribavirin for subacute sclerosing panencephalitis. Pediatr. Neurol. 24: 54– 59.
151. Townsend, J. J.,, J . R. Baringer,, J . S. Wolinsky,, N. Malamud,, J . P. Mednick,, H. S. Panitch,, R. A. T. Scott,, L. S. Oshiro,, and N . E. Cremer. 1975. Progressive rubella panencephalitis. Late onset after congenital rubella. N. Engl. J . Med. 292: 990– 993.
152. Townsend, J . J.,, W. G. Stroop,, J . R. Baringer,, J . S. Wolinsky,, J . H. McKerrow,, and B . O. Berg. 1982. Neuropathology of progressive rubella panencephalitis after childhood rubella. Neurology 32: 185– 190.
153. Ueda, K.,, T. Imamura,, X. Kawaguchi,, H. Tamari,, and X. Kambara. 1982. Subacute sclerosing panencephalitis. An autopsy case with impaired cellular immunity. Acta Pathol. Jpn. 32: 1103– 1110.
154. Vaheri, A.,, I. Julkunen,, and M.-L. Koskiniemi. 1982. Chronic encephalomyelitis with specific increase in intrathecal mumps antibodies. Lancet ii: 685– 688.
155. Valdimarsson, H.,, G. Agnarsdottir,, and P. J. Lachmann,. 1979. Subacute sclerosing panencephalitis, p. 406– 418. In F. Rose (ed.), Clinical Neuroimmunology. Blackwell Scientific Publications, Oxford, United Kingdom.
156. Van Bogaert, L. 1945. Une leuco-encephalite scleronsante subaigiie. J. Neurol. Neurosurg. Psychiatr. 8: 101– 120.
157. Vandvik, B.,, J . Natvig,, and E. Norrby,. 1974. Immunopathological studies in subacute sclerorising panencephalitis and multiple sclerosis with special reference to evidence for homogeneous antibody responses to measles virus within the central nervous system, p. 448– 463. In A. Nowoslawski (ed.), Schering Symposium on Immunopathobgy. Pergamon Press, Oxford, United Kingdom.
158. Vandvik, B .,, M. L. Weil,, M. Grandien,, and E. Norrby. 1978. Progressive rubella virus panencephalitis: synthesis of oligoclonal virus-specific IgG antibodies and homogeneous free fight chains in the central nervous system. Acta Neurol. Scand. 57: 53– 64.
159. Vinters, H. V.,, R. Wang,, and C. A. Wiley. 1993. Herpesviruses in chronic encephalitis associated with intractable childhood epilepsy. Hum. Pathol. 24: 871– 879.
160. Wechsler, S.,, and H. Meissner. 1982. Measles and SSPE viruses: similarities and differences. Prog. Med. Virol 28: 65– 95.
161. Wechsler, S. L.,, H. L. Weiner,, and B. N . Fields. 1979. Immune response in subacute sclerosing panencephalitis: reduced antibody response to the matrix protein of measles virus. J . Immunol. 123: 884– 889.
162. Weil, M. L.,, H. H. Itabashi,, N. E. Cremer,, L. S. Oshiro,, E. H. Lennette,, and L. Camay. 1975. Chronic progressive panencephalitis due to rubella virus simulating subacute sclerosing panencephalitis. N. Engl. J . Med. 292: 994– 998.
163. White, H. H.,, J. H. Kepes,, C. H. Kirkpatrick,, and R. N. Schimke. 1972. Subacute encephalitis and congenital hypogammaglobulinemia. Arch. Neurol. 26: 359– 365.
164. Wiendl, H.,, C. G. Bien,, P. Bemasconi,, B . Fleckenstein,, C. E. Elger,, J . Dichgans,, R. Mantegazza,, and A. Melms. 2001. Glu R3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen's encephalitis. Neurology 57: 1511– 1514.
165. Wolinsky, J. S.,, R. C. Dau,, E. Buimovici-Klein,, J. Mednick,, B. O. Berg,, P. B. Lang,, and L. Z. Cooper. 1979. Progressive rubella panencephalitis: immunovirological studies and results of isoprinosine therapy. Clin. Exp. Immunol 3S: 397– 404.
166. Wolinsky, J. S., 1989. P rogressive rubella encephalitis, p. 405– 416. In R. R. McKendall (ed.), Handbook of Clinical Neurology, vol. 12, of Revised Series, vol. 56. Elsevier Science, B.V., Amsterdam, The Netherlands.
167. Wolinsky, J . S.,, and M. McCarthy,. 1995. Rubella, p. 19– 45. In J. S. Porterfield (ed.), Exotic Viral Infections. Chapman and Hall, London, United Kingdom.
168. World Health Organization. 1989. Virus diseases: human T lymphotropic virus type I, HTLV-I. Wkly. Epidem. Rec. 64: 382– 383.
169. Yaqub, B . A. 1996. Subacute sclerosing panencephalitis (SSPE): early diagnosis, prognostic factors, and natural history. J . Neurol. Sci. 139: 227– 234.

References: V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V. 
 V.