SUBAKUT SKLEROZAN PANENSEFALİTLİ HASTALARDA KLİNİK GİDİŞAT VE PROGNOZ

Yıl 2015, Cilt: 5 Sayı: 2, 0 - 0, 01.06.2015

Sevgi Yimenicioğlu , Coşkun Yarar , Arzu Ekici , Ayten Yakut Kürşat Bora Çarman

Öz

Amaç: Bu çalışmada Eskişehir Osmangazi Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı’nın Çocuk Nöroloji Ünitesinde yapılmıştır. 1998 ile 2011 yılları arasında Çocuk Nöroloji Bilim dalında SSPE tanısıyla izlenen 14 hasta retrospektif olarak incelenmiştir

Purpose: In this study we evalutaed fourten patients retrospectivley that had admitted to Eskişehir Osmangazi University pedıatric neurology departmen between the years 1998-2011.

 Gereç: Hastaların son nörolojik durumu nörolojik defisit indeksi(NDI) ile tespit edilmiştir. Hastaların aşılama durumu, klinik bulguları göz bulguları ve beyin görüntüleme yöntemleri değerlendirilmiştir. 

Material: Patient’s last neurologic state is examined by neurologic deficiıt index (NDI).  Vaccination state, clinical findings, visual findings, cerebral imaging findings are evaluated.

 Yöntemler: Hastalığı evrelerken Jabbour'un sınıflamasi kullanıldı. Hastaların BOS inclemesi yapıldı, BOS kızamık IgG ve IgM antikorları IFA yöntemi ile çalışıldı. Serum IgM kızamık antikoru, IgG kızamık antikoru ise ELİSA yöntemiyle çalışıldı.Jabbour classification is used for staging of the disease. Patients’ cerebrospinal findings are recorded, CSF IgM andI g G anticore titers are studied with IFA method. Serum IgM andI g G anticore titers are studied with ELISA method.

 Bulgular: Toplam 14 hasta çalışmaya alındı. Hastaların ortalama yaşı 7.8 idi. Başvuru şikayetleri düşme atakları, konuşma bozukluğu, myoklini, konvulziyon, kognitif değişiklik, gece korkuları, tremor, enkoprezis, davranış değişikliği, görme kaybı, senkop, ensefalopati tablosuyla ile başvurmuşlar.

Fourteen patients are included in the study. Patients median age was 7.8. Presenting symptoms were drop attacks, speech disturbances, myoclonus, convulsions, cognitive dysfunction, night terrors, tremor, encopresis, changes in behaviour, vision loss, syncope, encepaholpathy.   

Sonuç : Hastalar şikâyetleri başladıktan ortalama iki ay içerisinde hastaneye başvurmuştu, Şikayet başlangıcı ile tanı koyma arasında geçen süre 2 -4 ay  arasında idi. Hastalarımızdan 3 tanesine akut fulminan bir seyir gelişti, biri hastanede sepsis nedeniyle diğer 2’si taburculuktan sırasıyla 1.5,  2 ay sonra exitus oldular. 

Patients had admitted to the hospital within the two months of the symptom beginning. The duration between the beginning of the complaint and diagnosisi was 2-4 months. Acute fulminating progress was seen in three of our patients, one of our patients died because of sepsis, the other two died 1.5 and two months after discharge from the hospital retrospectively.  

Kaynakça

• - Dyken P, DuRant R, Shmunes P: Subacute sclerosing panencephalitis surveillance- United States. MMWR 1982, 31:585-588.
• - Onal AE, Gurses C, Direskeneli GS, Yilmaz G, Demirbilek V, Yentur SP, Ozel S, Yapici Z, Tumerdem Y, Gökyigit A. Subacute sclerosing panencephalitis surveillance study in Istanbul. Brain Dev. 2006;28(3):183-9.
• - Bellini WJ, Rota JS, Lowe LE, Katz RS, Dyken PR, Zaki SR, et al. Subacute sclerosing panencephalitis: more cases of this disease are prevented by measles immunization than was previously recognized. J Infect Dis 2005;192:1686–93.
• - Dyken PR. Subacute sclerosing panencephalitis. Current status. Neurol Clin. 1985;3(1):179-96.
• - Dyken P R, Swift A , DuRant R H. Long -term follow-up of patients with subacute sclerosing panencephalitis treated with inosiplex. Ann Neurol 1982; 11: 359–64.
• - Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J. 2002; 78(916):63-70
• - Jabbour JT, Garcia JH, Lemmi H, et al. Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. JAMA 1969; 207: 2248-54.
• - Garg RK. Subacute sclerosing panencephalitis . J Neurol. 2008;255(12):1861-71.
• - Campbell C, Levin S, Humphreys P, et al. Subacute sclerosing panencephalitis: results of the Canadian paediatric surveillance program and review of the literature. BMC Pediatrics 2005; 15: 5-47.
• - World Health Organization (2006) Global Advisory Committee on Vaccine Safety, 1–2 December 2005. Wkly Epidemiol Rec 81:15–19
• - Campbell H, Andrews N, Brown KE, Miller E (2007) Review of the effect of measles vaccination on the epidemiology of SSPE. Int J Epidemiol 36: 1334–1348
• - Reuter D1, Schneider-Schaulies J. Measles virus infection of the CNS: human disease, animal models, and approaches to therapy . . Med Microbiol Immunol. 2010;199(3):261-71.
• - Yilmaz D, Aydin OF, Senbil N, Yuksel D (2006) Subacute sclerosing panencephalitis: is there something different in the younger children? Brain Dev 28: 649–652.
• - Manayani DJ1, Abraham M, Gnanamuthu C, Solomon T, Alexander M, Sridharan G. SSPE - the continuing challenge: a study based on serological evidence from a teritary care centre in India. Indian J Med Microbiol. 2002;20(1):16-8.
• - Herguner MO, Altunbasak S , Baytok V . Patients w ith acute, fulminant form of SSPE. Turk J Pediatr 2007; 49: 422–5.
• - Akram M, Naz F, Malik A, Hamid H. Clinical profile of subacute sclerosing panencephalitis. J Coll Physicians Surg Pak 2008; 18: 485–8.
• - Kissani N, Ouazzani R, Belai di H, Ouahabi H, Chkili T. Epileptic seizures and e pilepsy i n subacu te sclerosing panencephalitis (report of 30 cases). Neurophysiol Clin 2 001; 31:398 –405
• - Ozturk A, Gurses C, Baykan B, Gokyigit A, Eraksoy M Subacute sclerosing panencephalitis: clinical and magnetic resonance imaging evaluation of 36 patien ts. J Child Neurol 2002;17:25– 29
• - Prashant h LK, Taly AB, Sinha S, Ravi V. Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital. J Child Neurol 2007; 22:683 – 688.
• - Yuksel D, Sonmez PA, Yilmaz D, Senbil N, Gurer Y.Ocular findings in subacute sclerosing panencephalitis. Ocul Immunol Inflamm. 2011;19(2):135-8.)
• - Serdaroğlu A, Gücüyener K, Dursun I, Aydin K, Okuyaz C, Subaşi M, Or M, Ozkan B. Macular retinitis as a first sign of subacute sclerosing panencephalitis: the importance of early diagnosis. Ocul Immunol Inflamm. 2005;13(5):405-10.
• - Cochereau-Massin I, Gaudric A, Reinert P, Lehoang P, Rousselie F, Coscas G. Changes in the fundus in subacute sclerosing panencephalitis. Apropos of 23 cases J Fr Ophtalmol. 1992;15(4):255-61.
• - R Tandon, S Khanna, MC Sharma, S Seshadri, V Menon. Subacute sclerosing panencephalitis presenting as optic neuritis. Indian J Ophthalmol. 1999;47(4):250-2.
• - Erturk O, Karslıgil B, Cokar O, Yapici Z, Demirbilek V, Gurses C, Yalcinkaya C, Gokyigit A, Direskeneli GS, Yentur S, Onal E, Yilmaz G, Dervent A. Challenges in diagnosing SSPE. Childs Nerv Syst. 2011;27(12):2041-4.
• - Salehi HR, Delgado E, Wolf SM, McGoldrick PE, Moshé SL. Subacute sclerosing panencephalitis with atypical features. Pediatr Neurol. 2005 ;33(4):280-2.
• - Mgone CS, Mgone JM, Takasu T, Miki K, Kawanishi R, Asuo PG, Kono J, Komase K, Alpers MP. Clinical presentation of subacute sclerosing panencephalitis in Papua New Guinea. Trop Med Int Health. 2003;8(3):219-27.
• - Markand OM, Pasnzi JG. The electroencephalogram in subacute sclerosing panencephalitis. Arch Neurol 1975; 32: 719-726.
• - Brismar J, Gascon GG, Von Steyern KV et al : Subacute sclerosing panencephalitis: Evaluation with CT and MR. AJNR 1996; 17 : 761-722
• - Öz B. Subacute sclerosing panencephalitis: Case report and literature review. Cerrahpaşa J Med. 2000; 31 (2): 107-112
• - Trivedi R, Gupta RK, Agarawal A, Hasan KM, Gupta A, Prasad KN, Bayu G, Rathore D, Rathore RK, Narayana PA. Assessment of white matter damage in subacute sclerosing panencephalitis using quantitative diffusion tensor MR imaging. AJNR Am J Neuroradiol. 2006;27(8):1712-6.
• - Chang TW, Heel RC: Ribavirin and inosiplex: a review of their present status in viral diseases. Drugs 1981, 22:111-128.
• - Anlar B: Subacute sclerosing panencephalitis: Diagnosis and drug treatment options. CNS Drugs 1997, 7:111-120.
• - Anlar B, Yalaz K, Kose G, Saygi S. ß-Interferon plus inosiplex in the treatment of subacute sclerosing panencephalitis. J Child Neurol 1998, 13:557-559.
• - Tomoda A, Nomura K, Shiraishi S, Hamada A, Ohmura T, Hosoya M, et al. Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan. Brain Dev 2003;25:514–517.
• - Hosoya M, Mori S, Tomoda A, Mori K, Sawaishi Y, Kimura H, Shigeta S, Suzuki H. Pharmacokinetics and effects of ribavirin following intraventricular administration for treatment of subacute sclerosing panencephalitis. Antimicrob Agents Chemother. 2004; 48(12):4631–4635.
• - Gascon GG; International Consortium on Subacute Sclerosing Panencephalitis Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-alpha in subacute sclerosing panencephalitis (SSPE): international multicenter study. J Child Neurol. 2003;18(12):819-27.
• - Demir E, Aksoy A, Anlar B, Sönmez FM (2007) Atypical presentations of SSPE: a clinical study in four cases. Turk J Pediatr 49:295 –300
• - Marjanovi c BD, Stojanov LM, Z amurov ic DR , Pasic SS, Kravljanac R M, Djordjevi MS (2003) F ul minant subacute sclerosing panencephalitis: two cases with atypical presentation. Pediatr Neurol 29:63– 65