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Identifier 000431920
Title Υπερανθεκτική επιληπτική κατάσταση σε παιδιά και νέους
Alternative Title Super refractory status epilepticus in children and youth
Author Στεφανογιάννη, Χρυσαυγή
Thesis advisor Μπριασούλης, Γεώργιος
Reviewer Ηλία, Σταυρούλα
Ζαγανάς, Ιωάννης
Abstract Background Refractory and super-refractory status epilepticus are neurological emergencies in children, associated with high morbidity and mortality. As refractory SE are defined the seizures that fail to respond to first- and second-line antiepileptic drugs and have to be controlled with continuous anesthetic infusions. As super-refractory SE is defined the status epilepticus that persists for&ge;24 hours after induction of anaesthesia or rebounds after reduction or withdrawal of the anaesthetic drugs. It’s a rare condition with an estimated incidence of 10-15% of total cases of SE. Causes vary and thorough and specialized investigation (metabolic, immunological, genetic) is often necessary, in order to determine the diagnosis and guide the therapeutic process. Except from antiepileptic and anaesthetic drugs, multiple novel treatments have been used over the last decades for the management of SRSE, but their effectiveness is often controversial and there are no specific therapeutic protocols in children. Immunomodulatory treatments (steroids, IVIG, plasmapheresis), ketogenic diet and surgical dissection of the epileptogenic focus are only some of the available therapeutic approaches. Objective of the present study was to describe status epilepticus in children, adolescents and young adults and identify its characteristics, with special focus on the most complicated group of super-refractory status epilepticus. Methods We conducted a retrospective, single-center study of SE, RSE and SRSE cases treated in the pediatric ICU and neurologic department of the University Hospital of Heraklion from 2009 to 2019. Particular emphasis was given to epidemiology, etiologic factors, modern diagnostic modalities, therapeutic approaches and outcome. According to international classification, the etiology of SE of all 3 groups was summarized into 5 categories: 1) acute symptomatic, 2) remote symptomatic with acute precipitating causes or remote symptomatic and progressive encephalopathies, 3) febrile SE and 4) idiopathic or cryptogenic. We also documented the special clinical characteristics of new onset refractory seizures in NORSE and FIRES. The above data was retrieved from the patient files and then statistically analysed. Specific subgroups were compared with each other and correlations were explored. Results A total of 86 patients with 93 cases of SE were included in our study. 49 were males (52,7%) and 44 were females (47,3%), with a median age of 5 years (IQR 1,86-9,5). 63 patients (67,7%) were diagnosed with RSE and 15 with SRSE (16.1%). The distribution of SE types was different between age groups, with SRSE manifesting more frequently as generalized tonic-clonic seizures in ages 10-16 years, and RSE in ages 1-5 years (p=0.018). Acute symptomatic was the most usual cause (60%) in the group that finally evolved to SRSE (p=0.04) and in the patients with FIRES (88.9%), compared to idiopathic causes which were predominant (53,8%) in patients with NORSE (p<0.001). The percentage of pathological EEG or CTs did not differ between the 3 groups, while abnormal MRI findings were more commonly documented in the group of SRSE (p=0.023). In cases where initial investigation was non-conclusive, definitive diagnosis was set by WES (7.5%), brain biopsy and viral detection with PCR, showing higher incidence of autoimmune, mitochondrial diseases and FIRES in the SRSE group (p=0.017). NORSE or FIRES were diagnosed in 25% of cases, clinically presenting as sudden RSE but more commonly SRSE (p<0.001). Between all AEDs and anesthetics (85%) administered, important role in seizure termination was only played by midazolam (p=0.001) and phenytoin (p=0.003). In cases of RSE/SRSE immunomodulary treatments (p=0.05) and ketogenic diet (p=0.005) were more frequently used, weaning off the anesthetics was prolonged (p<0.001) and so were mechanical ventilation and ICU/hospital length of stay (p<0.001). Mortality was 3.2% and concerned 3 patients aged &llt;5years (p=0.013), while a new neurological deficit was documented in 16,1% of cases. Prognosis was particularly poor in patients with NORSE or FIRES or with acute symptomatic SE and progressive encephalopathy (p<0.001). Conclusions Almost ¾ of SE treated in the pediatric ICU had RSE and 16,1% have SRSE. These patients had higher disease severity, longer hospitalization and worse prognosis. ¼ of cases had NORSE or FIRES, clinically manifesting as acute RSE but more often SRSE. Specialized investigation (neuroimaging, genetic, metabolic and immunological testing) is often necessary, in order to determine the diagnosis and guide the therapeutic process. Continuous infusion of midazolam is mostly effective when received in the highest recommended doses, combined with other anesthetics, AEDs and immunomodulatory treatments. Further research in this area is necessary and evidence-based protocols for the management of SRSE need to be established.
Language Greek
Subject Pediatric ICU
Seizures
ΜΕΘ παίδων
Σπασμοί
Issue date 2020-08-05
Collection   School/Department--School of Medicine--Department of Medicine--Post-graduate theses
  Type of Work--Post-graduate theses
Permanent Link https://elocus.lib.uoc.gr//dlib/a/7/d/metadata-dlib-1599812865-696356-17168.tkl Bookmark and Share
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