Subacute sclerosing panencephalitis SSPE Invited Editor

Abstract

Subacute sclerosing panencephalitis SSPE is a neurodegenerative disease with a poor prognosis It is characterized by the persistent infection of the brain by the measles virus Mechanisms causing persistence and reactivation of measles virus in SSPE are unknown Patients usually have behavioral changes myoclonus dementia visual disturbances and pyramidal and extrapyramidal signs No treatment has a proven role A decline in frequency has been noticed in most of the developed countries whereas it continues to be high in developing countries Effective immunization against measles is the only solution presently available to the problem of this dreaded disease We aimed to give an overall review on SSPE in this paper Turk Arch Ped 2008; 43: 72 9 Key words: Child measles subacute sclerosing panencephalitis

Keywords

• -

Subakut sklerozan panansefalit SSPE Çağrılı Editör

Öz

Subakut sklerozan panansefalit SSPE nörodejeneratif bir hastalıktır Kızamık virüsünün beyinde oluşturduğu süregen enfeksiyon ile belirgindir Virüsün süregenliğinin ve reaktivasyonunun mekanizmaları bilinmemektedir Hastalarda genellikle davranış değişiklikleri miyoklonus demans görme bozukluğu piramidal ve ekstrapiramidal bulgular vardır Hastalığın henüz etkili ve kesin bir tedavisi yoktur Gelişmiş ülkelerde sıklığında azalma olmakla birlikte gelişmekte olan ülkelerde sık görülmektedir Kızamığa karşı etkin aşılama bu hastalık için şu andaki tek çözümdür Bu yazıda SSPE ile ilgili kapsamlı bilgi verilmesi amaçlandı Türk Ped Arş 2008; 43: 72 9 Anahtar kelimeler: Çocuk kızamık subakut sklerozan panansefalit

Anahtar Kelimeler

• Çocuk, kızamık, subakut sklerozan panansefalit

Kaynakça

1. Dyken PR. Neuroprogressive disease of postinfectious origin: a review of a resurging subacute sclerosing panencephalitis (SSPE). MRDD Research Reviews 2001; 7: 217-25.
2. Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 2002; 78; 63-70.
3. Greenfield JG. Encephalitis and encephalomyelitis in England and Wales during last decade. Brain 1950; 73: 141-66.
4. Dawson JR. Cellular inclusions in cerebral lesions of epidemic encephalitis. Am J Pathol 1933; 9: 7-15.
5. Park SY, Kohl S. Subacute Sclerosing Panencephalitis in an Identical Twin. Pediatrics 1999; 104: 1390-4.
6. Connolly JH, Allen IV, Hurwitz IJ, et al. Measles virus antibody and antigen in subacute sclerosing panencephalitis. Lancet 1967; 1: 542-4.
7. Onal AE, Gürses C, Direskeneli GS, et al. Subacute sclerosing panencephalitis surveillance study in Istanbul. Brain Dev 2006; 28: 183-9.
8. Halsey NA, Modlin JF, Jabbour JT, et al. Risk factors in suba- cute sclerosing panencephalitis: a case-control study. Am J Epidemiol 1980; 111: 415-24.

9. Anlar B, Köse G, Gürer Y, et al. Changing epidemiological features of subacute sclerosing panencephalitis. Infection 2001; 29: 192-5.
10. Rota PA, Bellini WJ. Update on the global distribution of ge- notypes of wild type measles viruses. JID 2003:187 (Suppl 1).
11. Rima BK, Duprex WP. Morbilliviruses and human disease. J Pathol 2006; 208: 199-214.
12. Schneider-Schaulies S, ter Meulen V. Pathogenic aspects of measles virus infections. Arch Virol Suppl 1999; 15: 139-58.
13. Norrby E, Kristensson K. Measles virus in the brain. Brain Res Bull 1997; 44: 213-20.
14. Rima BK, Duprex WP. Molecular mechanisms of measles vi- rus persistence. Virus Research 2005; 111: 132-47.
15. Bellini WJ, Rota JS, Lowe LE, et al. Subacute sclerosing pa- nencephalitis: more cases of this fatal disease are prevented by measles Immunization than was previously recognized. J infect Dis 2005; 192: 1686-93.
16. Miki K, Komase K, Mgone CS, et al. Molecular analysis of measles virus genome derived from SSPE and acute measles patients in Papua New Guinea. J Med Virol 2002; 68:105-12.
17. Cosby SL, Duprex WP, Hamill LA, et al. Approaches in the un- derstanding of morbillivirus neurovirulence. J Neurovirol 2002; 8 Suppl 2: 85-90.
18. Schneider-Schaulies J, Meulen V, Schneider-Schaulies S. Measles infection of the central nervous system. J Neurovirol 2003; 9: 247-52.
19. McQuaid S, Cosby SL. An immunohistochemical study of the distribution of the measles virus receptors, CD46 and SLAM, in normal human tissues and subacute sclerosing panencep- halitis. Lab Invest 2002; 82: 403-9.
20. Schneider-Schaulies J, ter Meulen V, Schneider-Schaulies S. Measles virus interactions with cellular receptors: consequen- ces for viral pathogenesis. J Neurovirol 2001; 7: 39.
21. Nester MJ. Use of a brief assessment examination in a study of subacute sclerosing panencephalitis. J Child Neurol 1996; 11: 173-80.
22. Anlar B, Guven A, Kose G, et al. Lymphocyte subsets, TNF alpha and interleukin-4 levels in treated and untreated suba- cute sclerosing panencephalitis patients. J Neuroimmunol 2005; 163: 195-8.
23. Aysun S, Sanal O, Renda Y, et al. Cell mediated immunity in patients with subacute sclerosing panencephalitis. Brain Dev 1984; 6: 391-6.
24. Griffin DE, Ward BJ. Differential CD4 T cell activation in me- asles. J Infect Dis 1993;168: 275-81.
25. Yentür SP, Gürses C, Demirbilek V. Alterations in cell-media- ted immune response in subacute sclerosing panencephalitis. J Neuroimmunol 2005;170: 179-85.
26. Karp CL, Wysocka M, Wahl LM, et al. Mechanisms of sup- pression of cell-mediated immunity by measles virus. Science 1996; 273: 228- 31.
27. Saruhan-Direskeneli G, Gürses C, Demirbilek V, et al. Eleva- ted interleukin-12 and CXCL10 in subacute sclerosing panen- cephalitis. Cytokine 2005; 32: 104-10.
28. Haspolat S, Anlar B, Kose G, Coskun M, Yegin O. Interleukin- 1beta, interleukin-1 receptor antagonist levels in patients with subacute sclerosing panencephalitis and the effects of diffe- rent treatment protocols. J Child Neurol 2001; 16: 417-20.
29. Gilden DH, Devlin ME, Burgoon MP, Owens GP. The search for virus in multiple sclerosis brain. Mult Scler 1996; 2: 179-83.
30. Burgoon MP, Keays KM, Owens GP, et al. Laser-capture mic- rodissection of plasma cells from subacute sclerosing panen- cephalitis brain reveals intrathecal disease-relevant antibodi- es. Proc Natl Acad Sci U S A 2005; 102: 7245-50.
31. Anlar B, Pinar A, Yasar Anlar F, et al. Viral studies in the ce- rebrospinal fluid in subacute sclerosing panencephalitis. J In- fect 2002; 44: 176-80.
32. Tasdemir N, Ece A, Tekes S, et al. Angiotensin-converting enzyme and angiotensin II type 1 receptor gene polymorp- hisms in children with subacute sclerosing panencephalitis. Am J Med Genet B Neuropsychiatr Genet 2006; 141: 445-8.
33. Gungor S, Olmez A, Pinar Arikan Firat, Haliloğlu G, Anlar B. Serum retinol and beta-carotene levels in subacute sclerosing panencephalitis. J Child Neurol 2007; 22: 341-3.
34. Jabbour JT, Garcia JH, Lemmi H, et al. Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. JA- MA 1969; 207: 2248-54.
35. Campbell C, Levin S, Humphreys P, et al. Subacute sclerosing panencephalitis: results of the Canadian paediatric surveillan- ce program and review of the literature. BMC Pediatrics 2005; 15: 5-47.
36. Dyken P, Dotson P, Maertens P. The neurological disability in- dex (NDI) in subacute sclerosing panencephalitis (SSPE). In: Bergamini F, Defanti CA, Ferrante P (eds). Subacute Sclero- sing Panencephalis: a reappraisal. Bergamo: Elsevier Science Publishers B.V. (Biomedical Division), 1986: 109-17.
37. Pampiglione G, Harden A. Neurophysiological aspects of SSPE. In: Program of the Second International Symposium on Subacute Sclerosing Panencephalitis. Bergamo, Elsevier Press, 1985: 33.
38. Anlar B, Yalaz K, Ustacelebi S. Symptoms and clinical signs, laboratory data in 80 cases of subacute sclerosing panencep- halitis. Rev Neurol 1988; 144: 829-32.
39. Salehi H, Wolf S, McGoldrick P, Moshé S. Subacute sclero- sing panencephalitis with atypical features. Pediatr Neurol 2005; 33: 280-2.
40. Demirbilek V, Benbir G, Cokar O, et al. Tonic seizures in su- bacute sclerosing panencephalitis: a video-illustration of two cases. Epileptic Disord 2005; 7: 221-5.
41. Kubota T, Okumura A, Takenaka J, et al. A case of subacute sclerosing panencephalitis preceded by epileptic seizures: evolutional EEG changes. Brain Dev 2003; 25: 279-82.
42. Aydin OF, Karakurt C, Senocak F, et al. Heart rate variability and autonomic dysfunction in SSPE. Pediatr Neurol 2005; 32: 184-9.
43. Chung BH, Ip PP, Wong VC, et al. Acute fulminant subacute sclerosing panencephalitis with absent measles and PCR stu- dies in cerebrospinal fluid. Pediatr Neurol 2004; 31: 222-4.
44. Simsek E, Ozturk A, Yavuz C, Kocabay K. Subacute sclero- sing panencephalitis (SSPE) associated with congenital me- asles infection. Turk J Pediatr 2005; 47: 58-62.
45. Tan H, Orhan A, Buyukavci M, Kocer I. Pseudotumor cerebri secondary to subacute sclerosing panencephalitis. J Child Neurol 2004; 19: 627-9.
46. Honarmand S, Glaser CA, Chow E, et al. Subacute sclerosing panencephalitis in the differential diagnosis of encephalitis. Neurology 2004; 63: 1489-93.
47. Serdaroglu A, Gucuyener K, Dursun I, et al. Macular retinitis as a first sign of subacute sclerosing panencephalitis: the importance of early diagnosis. Ocul Immunol Inflamm 2005; 13: 405-10.
48. Aydin OF, Orer H, Gurer YK. Subacute sclerosing panencephali- tis: a cause of acute vision loss. Pediatr Neurol 2004; 31: 214-7.
49. Dyken PR. Subacute sclerosing panencephalitis. Neurol Clin 1985; 3: 179-95.
50. Ekmekci O, Karasoy H, Gokcay A, Ulku A. Atypical EEG fin- dings in subacute sclerosing panencephalitis. Clin Neuroph- ysiol 2005; 116: 1762-7.
51. Anlar B, Saatci I, Kose G, Yalaz K. MRI findings in subacute sclerosing panencephalitis. Neurology 1996;47:1278-83.
52. Anlar B, Soylemezoglu F, Aysun S, et al. Tissue inflammatory response in subacute sclerosing panencephalitis (SSPE). J Child Neurol 2001; 16: 895-900.
53. Ozturk A, Gurses C, Baykan B, et al. Subacute sclerosing pa- nencephalitis: clinical and magnetic resonance imaging eva- luation of 36 patients. J Child Neurol 2002; 17: 25-9.
54. Aydin K, Tatli B, Ozkan M, et al. Quantification of neurometa- bolites in subacute sclerosing panencephalitis by 1H-MRS. Neurology 2006; 67: 911-3.
55. Tha KK, Terae S, Kudo K, et al. Early detection of subacute scle- rosing panencephalitis by high b-value diffusion-weighted ima- ging: a case report. J Comput Assist Tomogr 2006; 30: 126-30.
56. Trivedi R, Gupta RK, Agarawal A, et al. Assessment of white matter damage in subacute sclerosing panencephalitis using quantitative diffusion tensor MR imaging. AJNR Am J Neuro- radiol 2006; 27: 1712-6.
57. Dyken PR, Swift A, DuRant RH. Long-term follow-up of pati- ents with subacute sclerosing panencephalitis treated with inosiplex. Ann Neurol 1982;11:359-64.
58. Maimone D, Grimaldi LM, Incorpora G, et al. Intrathecal inter- feron in subacute sclerosing panencephalitis. Acta Neurol Scand 1988; 78: 161-6.
59. Gascon GG. International Consortium on Subacute Sclerosing Panencephalitis. Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-alp- ha in subacute sclerosing panencephalitis (SSPE): internatio- nal multicenter study. J Child Neurol 2003; 18: 819-27.
60. Gascon G, Yamani S, Crowell J, et al. Combined oral isopri- nosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev 1993; 15: 346-55.
61. Anlar B, Aydin OF, Guven A, et al. Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencepha- litis. Clin Ther 2004; 26: 1890-4.
62. Güngör S, Anlar B, Turan N, et al. Antibodies to borna disea- se virus in subacute sclerosing panencephalitis. Ped Infect Dis J 2005; 24: 833-4.
63. Del Toro-Riera M, Macaya-Ruiz A, Raspall-Chaure M, et al. Subacute sclerosing panencephalitis: combined treatment with interferon alpha and intraventricular ribavirin. Rev Neurol 2006; 42: 277-81.
64. Hosoya M, Shigeta S, Mori S, et al. High-dose intravenous ri- bavirin therapy for subacute sclerosing panencephalitis. Anti- microb Agents Chemother 2001; 45: 943-55.
65. Anlar B, Gucuyener K, Imir T, et al. Cimetidine as an immuno- modulator in subacute sclerosing panencephalitis: a double blind, placebo-controlled study. Pediatr Infect Dis J 1993; 12: 578-81.
66. Gurer YK, Kukner S, Sarica B. Intravenous gamma-globulin treatment in a patient with subacute sclerosing panencephali- tis. Pediatr Neurol 1996; 14: 72-4.
67. Al Rajeh S. Response to cytotoxic therapy in a female patient from Saudi Arabia. Published in correspondence section in the Lancet in 1994.
68. Nakayama T, Fujino M, Yoshida N. Molecular epidemiology of measles virus in Japan. Ped Int 2004; 46: 214-23.
69. Campbell H, Andrews N, Brown KE, Miller E. Review of the ef- fect of measles vaccination on the epidemiology of SSPE. Int J Epidemiol 2007; 36: 1334-48.
70. Oldstone MB, Dales S, Tishon A, et al. A role for dual viral hits in causation of subacute sclerosing panencephalitis. J Exp Med 2005; 202: 1185-90.
71. Otaki M, Sada K, Kadoya H, et al. Inhibition of measles virus and subacute sclerosing panencephalitis virus by RNA inter- ference. Antiviral Research 2006; 70: 105-11.