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Clinical Cases and Reviews in Epilepsy

Super-refractory status epilepticus in children:
what happens when anesthetic therapy is not enough?

Brief report/Clinical case, 80 - 87
doi: 10.11138/ccre/2017.2.1.080
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Introduction: to describe the clinical features, treatment options and outcome of Super-Refractory Status Epilepticus (SRSE) in a population of children aged ≤16 years with different etiology.
Methods: we retrospectively analyzed the population with status epilepticus admitted to the “Bambino Gesù” Pediatric Hospital between 2007 and 2016, identifying patients with SRSE. We examined medical history before SRSE including neurological status, preceding epilepsy, antiepileptic treatment, SE episodes. Etiology was classified as progressive/ metabolic (PM), remote symptomatic (RS), acute symptomatic (AS). We evaluated clinical and EEG features of SRSE, and all treatments including anesthetics, antiepileptic drugs and “other” treatments administered according to the single case. Outcome (further epilepsy/SE and neurological status) was assessed after ≥6 months.
Results: We identified 20 children (12 male/8 female) with SRSE with a mean age at SRSE of 5.9 years (0.7-16.6). Before SRSE 16 patients (80%) had epilepsy with episodes of SE in 8 (50%).
According to etiology 9 patients (45%) were classified as PM (8 mitochondrial, 1 folate deficiency), 7 (35%) as RS (4 genetic epilepsy, 3 focal cortical dysplasia), 4 (20%) as AS (2 CNS vasculitis, 1 stroke, 1 GABA-A receptor encephalitis). SRSE mean duration was 23 days (4-92). The most used anesthetics were midazolam (18), thiopental (15), propofol (11), ketamine (4). Treatments other than conventional AEDs were steroids (17), immunosuppressants (7), Vitamins (8), KD (5), Bromide (3), hypothermia (2), VNS (1). At last follow-up (mean 3.8 years), 12 patients (60%) showed a worsening of the neurological status, 9 (45%) presented further episodes of SE and 4 (20%) SRSE, 2 (10%) underwent epilepsy surgery and 2 (10%) died.
Significance: SRSE is a severe condition affecting mostly children with acute imbalance of the neuronal functions due to energy failure (progressive pathology), immune-mediated damage and symptomatic epilepsy; although the treatment remains
daunting, the use of “other” treatments can be very helpful in selected cases.

Vol. 2 (No. 1) 2017 Janaury - December

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  1. Super-refractory status epilepticus in children:
    what happens when anesthetic therapy is not enough?

    Caputo D., Stoppa F., Vigevano F., Fusco L.
    doi: 10.11138/ccre/2017.2.1.080