Brain & Development 37 (2015) 612–617 www.elsevier.com/locate/braindev
Original article
Prognosis and demographic characteristics of SSPE patients in Istanbul, Turkey Serhat Guler a,⇑, Mehmet Kucukkoc b, Akın Iscan a a
Bezmialem Vakif University, School of Medicine, Department of Pediatric Neurology, Turkey b Bezmialem Vakif University, School of Medicine, Department of Pediatrics, Turkey
Received 16 July 2014; received in revised form 10 September 2014; accepted 10 September 2014
Abstract Aim: SSPE is a rare progressive, invariably fatal long-term complication of measles infection. In this study, we assessed the demographic and prognostic characteristics of 64 consecutive SSPE patients diagnosed at a tertiary center. Methods: The study had a retrospective design; data were obtained from patient records. Results: The study includes 64 patients diagnosed with SSPE. There was history of consanguineous marriage in 27 (42.2%) patients. The average patient lifespan was 3.8 years (45 days–12 years). The average patient age at diagnosis was 12.3 (range, 5– 17) years. A statistically significant correlation was found between the age at diagnosis and lifespan (p = 0.002). A statistically significant correlation was found between the incubation period and patient lifespan (p < 0.001). No significant correlation was found between duration in the intensive care unit and lifespan (p = 0.122). Routine physical therapy had no significant impact on the average lifespan (p = 0.619). No significant difference was found between the vaccination dose and lifespan (p = 0.651). Conclusions: High frequency of parental consanguinity in SSPE patients need to be evaluated as there might a genetic influence. Physical therapy and supportive treatments seems to have no affect on lifespan in SSPE patients. The age at diagnosis and incubation period might have an affect on prognosis and lifespan. Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Keywords: SSPE; Survival; Measles; Measles vaccine
1. Introduction Subacute sclerosing panencephalitis (SSPE) is chronic, persistent encephalitis that is secondary to measles infection and leads to demyelination in the central nervous system (CNS) [1]. Symptoms usually develop insidiously and progress to death within 1– 3 years. In SSPE, remission is rare; 5–10% of patients may show some improvement [2]. The incidence of SSPE ⇑ Corresponding author at: Adnan menderes bulvarı, Istanbul, Turkey. Tel.: +90 2124531700 7970, +90 5057588993 (mobile). E-mail addresses: [email protected] (S. Guler), [email protected] (M. Kucukkoc), [email protected] (A. Iscan).
was 2.2/1,000,000 in a study conducted in Istanbul, Turkey between 2002 and 2004. This rate was 0.06–1/ 1,000,000 in countries with routine immunization [3– 5]. In 2007 and 2008, the incidence of measles decreased in European countries [6]. Nevertheless, since 2009, a new measles pandemic began among adolescents and young adults [7]. In 2013, in European countries, 10,678 measles cases were reported [8]. These figures demonstrate that the incidence of SSPE may change again in coming years and highlights the need for a reconsideration of the disease. We evaluated demographic changes of the disease in the last 6 years in Istanbul. Istanbul has a population of
http://dx.doi.org/10.1016/j.braindev.2014.09.006 0387-7604/Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
S. Guler et al. / Brain & Development 37 (2015) 612–617
17 million people, with a high immigrant population. This is the first study that has assessed the effects of prognostic factors on survival in SSPE patients before and after diagnosis. 2. Material and methods The study included 69 consecutive patients diagnosed with SSPE at the Pediatric Neurology Department at Bezmialem Vakif University, Medical Faculty, between June 2007 and June 2013. The diagnosis was established primarily on clinical features such as progressive cognitive and/or behavioral changes, myoclonus as well as periodic complexes in EEG and raised antimeasles IgG in cerebrospinal fluid (CSF). Jabbour classification was used after initial diagnosis for clinical classification. All the patients in our SSPE cohort had died before the investigation in between 2007 and 2013. The initial diagnosis was made by Bezmialem Pediatric Neurology department as well as other clinics in Istanbul Turkey. The study had a retrospective design; data were obtained from patient records. Remaining information was gathered during phone conversations with family members. Due to outdated contact information, the families of five patients could not be reached and were excluded from the study. At the beginning of the telephone conversation, the families were informed about the purpose of the study and, after approval, arrangements were made so that both the mother and the father could join the conversation. The conversations were carried out by the same researcher according to the same pre-written text. Patient information such as demographic features of families, vaccination history, clinical stage (Jabbour stage), whether the subject had been diagnosed with measles, the selected treatment method, supportive care, duration of stay in the intensive care unit (ICU), complications, and cause of death were recorded according to parents speech and patient records. This study was approved by the Ethics Committee of the Medical Faculty at Bezmialem Vakif University. The study was carried out in accordance with the Helsinki Declaration. Statistical analyses were performed using the SPSS software (ver. 15). The t-test and the Mann– Whitney U-test were used to compare groups and Pearson’s correlation coefficient was used to analyze correlations. p values
Original article
Prognosis and demographic characteristics of SSPE patients in Istanbul, Turkey Serhat Guler a,⇑, Mehmet Kucukkoc b, Akın Iscan a a
Bezmialem Vakif University, School of Medicine, Department of Pediatric Neurology, Turkey b Bezmialem Vakif University, School of Medicine, Department of Pediatrics, Turkey
Received 16 July 2014; received in revised form 10 September 2014; accepted 10 September 2014
Abstract Aim: SSPE is a rare progressive, invariably fatal long-term complication of measles infection. In this study, we assessed the demographic and prognostic characteristics of 64 consecutive SSPE patients diagnosed at a tertiary center. Methods: The study had a retrospective design; data were obtained from patient records. Results: The study includes 64 patients diagnosed with SSPE. There was history of consanguineous marriage in 27 (42.2%) patients. The average patient lifespan was 3.8 years (45 days–12 years). The average patient age at diagnosis was 12.3 (range, 5– 17) years. A statistically significant correlation was found between the age at diagnosis and lifespan (p = 0.002). A statistically significant correlation was found between the incubation period and patient lifespan (p < 0.001). No significant correlation was found between duration in the intensive care unit and lifespan (p = 0.122). Routine physical therapy had no significant impact on the average lifespan (p = 0.619). No significant difference was found between the vaccination dose and lifespan (p = 0.651). Conclusions: High frequency of parental consanguinity in SSPE patients need to be evaluated as there might a genetic influence. Physical therapy and supportive treatments seems to have no affect on lifespan in SSPE patients. The age at diagnosis and incubation period might have an affect on prognosis and lifespan. Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Keywords: SSPE; Survival; Measles; Measles vaccine
1. Introduction Subacute sclerosing panencephalitis (SSPE) is chronic, persistent encephalitis that is secondary to measles infection and leads to demyelination in the central nervous system (CNS) [1]. Symptoms usually develop insidiously and progress to death within 1– 3 years. In SSPE, remission is rare; 5–10% of patients may show some improvement [2]. The incidence of SSPE ⇑ Corresponding author at: Adnan menderes bulvarı, Istanbul, Turkey. Tel.: +90 2124531700 7970, +90 5057588993 (mobile). E-mail addresses: [email protected] (S. Guler), [email protected] (M. Kucukkoc), [email protected] (A. Iscan).
was 2.2/1,000,000 in a study conducted in Istanbul, Turkey between 2002 and 2004. This rate was 0.06–1/ 1,000,000 in countries with routine immunization [3– 5]. In 2007 and 2008, the incidence of measles decreased in European countries [6]. Nevertheless, since 2009, a new measles pandemic began among adolescents and young adults [7]. In 2013, in European countries, 10,678 measles cases were reported [8]. These figures demonstrate that the incidence of SSPE may change again in coming years and highlights the need for a reconsideration of the disease. We evaluated demographic changes of the disease in the last 6 years in Istanbul. Istanbul has a population of
http://dx.doi.org/10.1016/j.braindev.2014.09.006 0387-7604/Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
S. Guler et al. / Brain & Development 37 (2015) 612–617
17 million people, with a high immigrant population. This is the first study that has assessed the effects of prognostic factors on survival in SSPE patients before and after diagnosis. 2. Material and methods The study included 69 consecutive patients diagnosed with SSPE at the Pediatric Neurology Department at Bezmialem Vakif University, Medical Faculty, between June 2007 and June 2013. The diagnosis was established primarily on clinical features such as progressive cognitive and/or behavioral changes, myoclonus as well as periodic complexes in EEG and raised antimeasles IgG in cerebrospinal fluid (CSF). Jabbour classification was used after initial diagnosis for clinical classification. All the patients in our SSPE cohort had died before the investigation in between 2007 and 2013. The initial diagnosis was made by Bezmialem Pediatric Neurology department as well as other clinics in Istanbul Turkey. The study had a retrospective design; data were obtained from patient records. Remaining information was gathered during phone conversations with family members. Due to outdated contact information, the families of five patients could not be reached and were excluded from the study. At the beginning of the telephone conversation, the families were informed about the purpose of the study and, after approval, arrangements were made so that both the mother and the father could join the conversation. The conversations were carried out by the same researcher according to the same pre-written text. Patient information such as demographic features of families, vaccination history, clinical stage (Jabbour stage), whether the subject had been diagnosed with measles, the selected treatment method, supportive care, duration of stay in the intensive care unit (ICU), complications, and cause of death were recorded according to parents speech and patient records. This study was approved by the Ethics Committee of the Medical Faculty at Bezmialem Vakif University. The study was carried out in accordance with the Helsinki Declaration. Statistical analyses were performed using the SPSS software (ver. 15). The t-test and the Mann– Whitney U-test were used to compare groups and Pearson’s correlation coefficient was used to analyze correlations. p values
