SUBAKUT SKLEROZAN PANENSEFALİTLİ HASTALARDA KLİNİK GİDİŞAT VE PROGNOZ Clinical Follow Up and Prognosis of Patients With Subacute Sclerosing Panencepahlitis

Amac: Bu calismada Eskisehir Osmangazi Universitesi Tip Fakultesi, Cocuk Sagligi ve Hastaliklari Anabilim Dali'nin Cocuk Noroloji Unitesinde yapilmistir. 1998 ile 2011 yillari arasinda Cocuk Noroloji Bilim dalinda SSPE tanisiyla izlenen 14 hasta retrospektif olarak incelenmistir. Gerec: Hastalarin son norolojik durumu norolojik defisit indeksi (NDl) ile tespit edilmistir. Hastalarin asilama durumu, klinik bulgulari goz bulgulari ve beyin goruntuleme yontemleri degerlendirilmistir. Yontemler: Hastaligi evrelerken Jabbour'un siniflamasi kullanildi. Hastalarin BOS inclemesi yapildi, BOS kiza- mik lgG ve lgM antikorlari lFA yontemi ile calisildi. Serum lgM kizamik antikoru, lgG kizamik antikoru ise ELISA yontemiyle calisildi. Bulgular: Toplam 14 hasta calismaya alindi. Hastalarin ortalama yasi 7.8 idi. Basvuru sikayetleri dusme atak- lari, konusma bozuklugu, myoklini, konvulziyon, kognitif degisiklik, gece korkulari, tremor, enkoprezis, davranis degisikligi, gorme kaybi, senkop, ensefalopati tablosuyla ile basvurmuslar. Sonuc: Hastalar sikayetleri basladiktan ortalama iki ay icerisinde hastaneye basvurmustu. Şikayet baslangici ile tani koyma arasinda gecen sure 2 -4 ay arasinda idi. Hastalarimizdan 3 tanesine akut fulminan bir seyir gelisti, biri hastanede sepsis nedeniyle diger 2'si taburculuktan sirasiyla 1.5, 2 ay sonra kaydedildi. Anahtar kelimeler: Subakut sklerozan panensefalit; Klinik bulgular; Prognoz ABSTRACT Purpose: ln this study, we retrospectivley evalutaed fourten patients admitted to Eskisehir Osmangazi University pediatric neurology departmen between the years 1998-2011. Material: Patient's last neurologic state is examined by neurologic deficiit index (NDl). Vaccination state, clinical findings, visual findings, cerebral imaging findings are evaluated. Methods: Jabbour classification is used for staging of the disease. Patients' cerebrospinal findings are recorded, CSF lgM andl g G anticore titers are studied with lFA method. Serum lgM andl g G anticore titers are studied with ELlSA method. Findings: 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. Result: Patients had admitted to the hospital within the two months of onset of the symptom. The duration between the beginning of the complaint and diagnosis was 2-4 months. Acute fulminating progress was seen in three patients, one patients died because of sepsis, the other two were died at follow-up 1.5 and two months after discharge from the hospital retrospectively. Key words: Subacute sclerosing panencepahlitis; Clinical findings; Prognosis