BRIEF COMMUNICATION Challenges in diagnosing SSPE Ozdem Erturk & Beyhan Karslıgil & Ozlem Cokar & Zuhal Yapıcı & Veysi Demirbilek & Candan Gurses & Cengiz Yalcinkaya & Aysen Gokyigit & Guher Saruhan Direskeneli & Sibel Yentur & Emel Onal & Gulden Yılmaz & Aysin Dervent Received: 3 May 2011 /Accepted: 28 September 2011 /Published online: 15 October 2011 # Springer-Verlag 2011 Abstract Purpose The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties. Methods We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2 years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6 months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study. Results Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria. Conclusions We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization Keywords Subacute sclerosing panencephalitis . Seizure . Differential diagnosis . Clinical course Introduction Subacute sclerosing panencephalitis (SSPE) is a progres- sive degenerative encephalitis of the central nervous system caused by the persistent infection of measles virus [6]. It usually develops after a latency period of 2–10 years following measles infection [7]. The incidence of the disease depends on the occurrence of clinical measles. O. Erturk : V. Demirbilek (*) : C. Yalcinkaya : A. Dervent Department of Neurology, Cerrahpasa Faculty of Medicine, Istanbul University, 34098 Fatih, Istanbul, Turkey e-mail: demirbilek@istanbul.edu.tr B. Karslıgil Department of Neurology, Tam Med Hospital, Gaziantep, Turkey O. Cokar Department of Neurology, Haseki Hospital, Istanbul, Turkey Z. Yapıcı : C. Gurses : A. Gokyigit Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey G. S. Direskeneli : S. Yentur Department of Physiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey E. Onal Department of Public Health, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey G. Yılmaz Department of Microbiology, Faculty of Medicine, Yeditepe University, Istanbul, Turkey Childs Nerv Syst (2011) 27:2041–2044 DOI 10.1007/s00381-011-1603-x