Author response: Neurologic outcome of postanoxic refractory status epilepticus after aggressive treatment

Neurology ◽  
2019 ◽  
Vol 93 (6) ◽  
pp. 278.1-278
Author(s):  
Simone Beretta
2019 ◽  
Vol 50 (03) ◽  
pp. 197-201
Author(s):  
S. Schmid ◽  
M. Wagner ◽  
C. Goetz ◽  
C. Makowski ◽  
P. Freisinger ◽  
...  

AbstractMitochondrial dynamics such as fission and fusion play a vital role in normal brain development and neuronal activity. DNM1L encodes a dynamin-related protein 1 (Drp1), which is a GTPase essential for proper mitochondrial fission. The clinical phenotype of DNM1L mutations depends on the degree of mitochondrial fission deficiency, ranging from severe encephalopathy and death shortly after birth to initially normal development and then sudden onset of refractory status epilepticus with very poor neurologic outcome. We describe a case of a previously healthy 3-year-old boy with a mild delay in speech development until the acute onset of a refractory status epilepticus with subsequent epileptic encephalopathy and very poor neurologic outcome. The de novo missense mutation in DNM1L (c.1207C > T, p.R403C), which we identified in this case, seems to determine a unique clinical course, strikingly similar to four previously described patients in literature with the identical de novo heterozygous missense mutation in DNM1L.


Neurology ◽  
2018 ◽  
Vol 91 (23) ◽  
pp. e2153-e2162 ◽  
Author(s):  
Simone Beretta ◽  
Anna Coppo ◽  
Elisa Bianchi ◽  
Clara Zanchi ◽  
Davide Carone ◽  
...  

ObjectiveTo investigate neurologic outcome of patients with cardiac arrest with refractory status epilepticus (RSE) treated with a standardized aggressive protocol with antiepileptic drugs and anesthetics compared to patients with other EEG patterns.MethodsIn the prospective cohort study, 166 consecutive patients with cardiac arrest in coma were stratified according to 4 independent EEG patterns (benign, RSE, generalized periodic discharges [GPDs], malignant nonepileptiform) and multimodal prognostic indicators. Primary outcomes were survival and cerebral performance category (CPC) at 6 months.ResultsRSE occurred in 36 patients (21.7%) and was treated with an aggressive standardized protocol as long as multimodal prognostic indicators were not unfavorable. RSE started after 3 ± 2.3 days after cardiac arrest and lasted 4.7 ± 4.3 days. A benign EEG pattern was recorded in 76 patients (45.8%); a periodic pattern (GPDs) was seen in 13 patients (7.8%); and a malignant nonepileptiform EEG pattern was recorded in 41 patients (24.7%). The 4 EEG patterns were highly associated with different prognostic indicators (low-flow time, clinical motor seizures, N20 responses, neuron-specific enolase, neuroimaging). Survival and good neurologic outcome (CPC 1 or 2) at 6 months were 72.4% and 71.1% for benign EEG pattern, 54.3% and 44.4% for RSE, 15.4% and 0% for GPDs, and 2.4% and 0% for malignant nonepileptiform EEG pattern, respectively.ConclusionsAggressive and prolonged treatment of RSE may be justified in patients with cardiac arrest with favorable multimodal prognostic indicators.


2019 ◽  
Vol 8 (1) ◽  
pp. 41-43
Author(s):  
Kashyap Narsingh Shakya ◽  
Anwesh Bhatta ◽  
Samjhana Phuyal ◽  
Arbind Yadav

Refractory status epilepticus, with persistence of seizures despite initial intravenous benzodiazepine and one secondline intravenous antiepileptic drug occurs in nine percent to 44% of the patients with status epilepticus. Aggressive treatment is needed to control such seizures with drug refractoriness. A case of a 2-year-old boy treated with intravenous levetiracetam with cessation of seizures, averting the need for general anesthesia is reported. Option of using levetiracetam in refractory status epilepticus is suggested.


2019 ◽  
Vol 101 ◽  
pp. 106374 ◽  
Author(s):  
Simone Beretta ◽  
Anna Coppo ◽  
Elisa Bianchi ◽  
Clara Zanchi ◽  
Davide Carone ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document