Process From Diagnosis To Treatment Of 3 SSPE Cases Presented At The Late Age

Abstract

Subacute sclerosing panencephalitis (SSPE) is diagnosed with clinical evaluation including history and physical evaluation along with electroencephalogram (EEG), magnetic resonance imaging of the brain and cerebrospinal fluid (CSF) analysis. A total of 3 patients with SSPE (mean age=25.3 years) who were admitted to the Department of Neurology at Uludağ University Faculty of Medicine, were evaluated retrospectively. Clinical staging was performed using Risk and Haddad's classification. Time to SSPE diagnosis from initial symptoms ranged from 10 days to 3 months; the patients presented to a physician two months after their symptoms were recognized; weakening of mental capacities attracted the attention of members of their families. EEG examinations revealed slow baseline activity and penetrating slow discharges with regular high amplitudes every 8-10 seconds. Although immunoglobulin (Ig) M for measles was negative, IgG was positive in the cerebrospinal fluid and serum samples of all patients. All patients received isoprinosine, and various doses of valproic acid and clonazepam during their treatment. One patient died 13 months after the diagnosis, the other two continue to be followed up. SSPE infection should be considered in central nervous system-involved rapidly-progressing late-onset adult diseases because there has recently been a rapid increase in the number of reported cases of SSPE.Keywords: Subacute sclerosing panencephalitis, prognosis, late presentation Subakut slerozan panensefalit (SSPE) elektroensefalografi (EEG), kranyal manyetik rezonans (MR) görüntüleme ve klinik belirtileri değerlendirip, hikayeleri, fizik muayenedeki değişikliği ve EEG ve beyin omurilik sıvısı (BOS) incelemeleri ile tanı konur. Uludağ Üniversitesi Tıp Fakültesi, Nöroloji Kliniği'nde toplam 3 SSPE'li (yaş ortalaması=25,3) hasta retrospektif olarak değerlendirildi. SSPE tanısı semptomların başlangıcından 10 gün ile 3 ay arasında konuldu. EEG incelemelerinde 8-10 saniye süreli yüksek amplitüdlü yavaş dalgalar ve temel aktivitede yavaşlama gözlendi. BOS örneklerinde kızamık IgM negatif iken IgG pozitif, serumda hepsi pozitif idi. Valproik asit ve klonazepam değişik dozlarda ve sürede kullanıldı tüm olgulara Isoprinosine verildi. Bir olgu 13 ay sonra kaybedildi, diğer olgular takip edilmektedir. SSPE enfeksiyonu santral sinir sistemini tutup hızlıca progrese olmaktadır ve son zamanlarda geç yaşta görülen SSPE olgularının sunumları hızla artmaktadır.