Browsing by Author "Kravljanac, Djordje (57219657195)"

Purpose: Evaluation of the etiology, clinical characteristics and outcome of the first status epilepticus (fSE) event in children. Method: The patients with fSE hospitalized in our Institute from 1995 to 2011 were included. The etiology was characterized as either known (symptomatic) or unknown (cryptogenic). Outcome was assessed at the end of hospitalization. Logistic regression analyses were used to assess predictors of the outcome, with odds ratio adjusted by age as a measure effect. Results: The study included 236 patients with a median age of 2.0 years (IQR 4.0). Etiology was identified as secondary to: defined electroclinical syndromes 108 (45.8), acute symptomatic conditions 63 (26.7%), unknown 24 (10.1%), progressive encephalopathy 23 (9.7%), or remote symptomatic 18 (7.6%). Recurrence rate was 16.9%, neurological consequences were in 24.6% and case-fatality ratio was 4.7%. The main predictors were for: a) death – progressive encephalopathy (OR = 14.68, 95% CI 4.06–23.11. p = 0.001); b) neurological sequelae – acute symtomatic (OR 3.44, 95% CI 4.82–6.47) p = 0.001, remote symptomatic (OR = 13.84, 95% CI 4.34–44.12. p = 0.001), progressive encephalopathy (OR = 3.94, 95% CI 1.64–9.56. p = 0.002), seizure duration >60 min (OR = 0.44, 95% CI 0.24–0.81. p = 0.001); c) seziure recurrence – acute symptomatic etiology (OR = 3.59, 95% CI 41.76–7.21. p = 0.001), seizure duration >60 min (OR = 0.30, 95% CI 0.15–0.61. p = 0.001). Conclusions: In children with fSE, exploring acute disorders and immediate etiological treatment is essential. The outcome of fSE is favorable concerning the recurrence rate, while neurological sequelae are seen in one quarter of the patients. The etiology and fSE duration are the main determinants of outcome. © 2018 British Epilepsy Association

Purpose: Evaluation of the etiology, clinical characteristics and outcome of the first status epilepticus (fSE) event in children. Method: The patients with fSE hospitalized in our Institute from 1995 to 2011 were included. The etiology was characterized as either known (symptomatic) or unknown (cryptogenic). Outcome was assessed at the end of hospitalization. Logistic regression analyses were used to assess predictors of the outcome, with odds ratio adjusted by age as a measure effect. Results: The study included 236 patients with a median age of 2.0 years (IQR 4.0). Etiology was identified as secondary to: defined electroclinical syndromes 108 (45.8), acute symptomatic conditions 63 (26.7%), unknown 24 (10.1%), progressive encephalopathy 23 (9.7%), or remote symptomatic 18 (7.6%). Recurrence rate was 16.9%, neurological consequences were in 24.6% and case-fatality ratio was 4.7%. The main predictors were for: a) death – progressive encephalopathy (OR = 14.68, 95% CI 4.06–23.11. p = 0.001); b) neurological sequelae – acute symtomatic (OR 3.44, 95% CI 4.82–6.47) p = 0.001, remote symptomatic (OR = 13.84, 95% CI 4.34–44.12. p = 0.001), progressive encephalopathy (OR = 3.94, 95% CI 1.64–9.56. p = 0.002), seizure duration >60 min (OR = 0.44, 95% CI 0.24–0.81. p = 0.001); c) seziure recurrence – acute symptomatic etiology (OR = 3.59, 95% CI 41.76–7.21. p = 0.001), seizure duration >60 min (OR = 0.30, 95% CI 0.15–0.61. p = 0.001). Conclusions: In children with fSE, exploring acute disorders and immediate etiological treatment is essential. The outcome of fSE is favorable concerning the recurrence rate, while neurological sequelae are seen in one quarter of the patients. The etiology and fSE duration are the main determinants of outcome. © 2018 British Epilepsy Association

The aim of the study was to explore whether diagnosis and managing children with progressive myoclonus epilepsy (PME) were improved during the last decade. Methods: The retrospective study included children with PME treated in the Institute during the last 25 years. Investigation time was divided in two periods (groups): before December 2010 (the first group) and after this period up to December 2019 (the second group). Inclusion criteria are as follows: patients aged from 0.2–18 years and with PME. Evaluated parameters are etiology, age at seizure onset, diagnosis delay, epilepsy phenotype, and, as a measure of epilepsy control — status epilepticus (SE) frequency and recurrence rate. Statistical analysis included the following tests: Chi-Square, Mann–Whitney, and analysis of variance (ANOVA), using SPSS version 25. Results: The study included 51 patients, 27 in the first, and 24 in the second group. The underlying diseases were: neuronal ceroid lipofuscinosis (NCL; 30), Gaucher (5), Niemann–Pick (4), mitochondrial (4), Lafora (3), Krabbe (2), and KCNC1 gene mutation (2). The average duration from initial symptoms to diagnosis was 3.2 ± 3 years (first group) vs. 1.4 ± 0.9 years (second). Both SE frequency rate (55.5% vs. 37.5%) and recurrence rate (66.7% vs. 22.2%) were higher in the first group, showing tendency towards, but not statistically significant difference. Conclusion: The diagnosis and epilepsy managing children with PME were improved during the last decade. Earlier genetic diagnosis, appropriate antiseizure medications, education of parents/caregivers of children in high risk for SE, and availability of effective prehospital rescue medications contributed to significantly decreased frequency and recurrence rate of SE. © 2020 Elsevier Inc.

The aim of the study was to explore whether diagnosis and managing children with progressive myoclonus epilepsy (PME) were improved during the last decade. Methods: The retrospective study included children with PME treated in the Institute during the last 25 years. Investigation time was divided in two periods (groups): before December 2010 (the first group) and after this period up to December 2019 (the second group). Inclusion criteria are as follows: patients aged from 0.2–18 years and with PME. Evaluated parameters are etiology, age at seizure onset, diagnosis delay, epilepsy phenotype, and, as a measure of epilepsy control — status epilepticus (SE) frequency and recurrence rate. Statistical analysis included the following tests: Chi-Square, Mann–Whitney, and analysis of variance (ANOVA), using SPSS version 25. Results: The study included 51 patients, 27 in the first, and 24 in the second group. The underlying diseases were: neuronal ceroid lipofuscinosis (NCL; 30), Gaucher (5), Niemann–Pick (4), mitochondrial (4), Lafora (3), Krabbe (2), and KCNC1 gene mutation (2). The average duration from initial symptoms to diagnosis was 3.2 ± 3 years (first group) vs. 1.4 ± 0.9 years (second). Both SE frequency rate (55.5% vs. 37.5%) and recurrence rate (66.7% vs. 22.2%) were higher in the first group, showing tendency towards, but not statistically significant difference. Conclusion: The diagnosis and epilepsy managing children with PME were improved during the last decade. Earlier genetic diagnosis, appropriate antiseizure medications, education of parents/caregivers of children in high risk for SE, and availability of effective prehospital rescue medications contributed to significantly decreased frequency and recurrence rate of SE. © 2020 Elsevier Inc.