New onset Refractory status Epilepticus(NORSE )with sequelae


Dr Joepaul Joy, Dr Vinayan KP, Dr Vysakh Anand(Ped Neuro), Dr C Jayakumar
 
Six and year five month old boy, first of NCM with normal perinatal and developmental history presented with acute cluster of seizures and encephalopathy following a febrile illness. 
Child was treated outside as Super refractory status epilepticus
Out side hospital history 
Child developed high grade intermittent fever lethargic . Had symptoms of URI and was on medications .On day 2, he complained of retro-orbital pain and headache. Following day, child awoke from sleep with altered sensorium. He again developed one episode of seizure suggestive of GTCS lasting 5-10minutes followed postictal drowsiness. One more event was noted, hence he was shifted to the PICU.Child has received phenytoin, valparin, phenobarbitone to control seizure . 
CSF study showed 6 cells, G 128 (154), P (78). He was started on Inj acyclovir, ceftriaxone. CSF for JE, West Nile HSV, Enterovirus were negative. 
SGOT/SGPT was deranged.


MRI brain with T2/FLAIR showed hyperintensities in bilateral temporoparietal, parietal occipital high frontoparietal lobes with diffusion restriction without enhancement- 
? Viral encephalitis, PRES, postictal.
In view of persistent seizures, child was mechanically ventilated, started on infusion midaz–>thiopental infusion–> iv ketamine. 
In view of suspected auto immune (AI)encephalitis– he has received IVMP @ 30MG/KG/DAY and IVIG @ 2g/kg from day 2 of admission. AI encephalitis panel was negative. Anticipating raised ICT, he has received 3% saline and mannitol. Considering atypical organism – he has received azithromycin and doxycycline. 
Endo tracheal aspirate grew candida for which he was started on Flucanazole. In view of persistent seizures, he has received clobazam, lacosamide, lamotrigine.
CSF CBNAAT was negative. Child received 11 days of meropenem, 12 days of vancomycin, 14 days of acyclovir from outside hospital.Outside labs showed hyponatremia OT/PT  95/77–>134/46 
CRP- negative, ESR- 75, Cultures- tracheal aspirate candida, blood, CSF and urine- negative.At the time of admission he was on : Inj levipil ,Inj Valproate, InjLacosamide, T Clobazam ,T lamotrigine, Midaz infusion, Pyridoxine .
Child was received in the PICU intubated. On examination GCS was E1VTM1 with decerebrate posturing. Pupils were equally reacting. Tone was increased, reflexes were brisk with ankle clonus present bilaterally. Blood routines showed elevated transaminases with SGOT/SGPT (50/75IU/L), calcium at 8.5mg/dl, and mildly elevated CRP. Bedside EEG was done which showed multifocal epileptiformabnormalities with moderate degree of electro cerebral dysfunction. Recorded 5 EEG seizures with various onset. In view of transaminitis, valproate was stopped and child was initiated on syrup L carnitine .Child was continued on injection meropenem and acyclovir. The child was extubated on day 4 of admission and was on minimal oxygen support. MRI brain showed extensive bilateral ( left > right ) cortical/ gyral thickening with T2/ FLAIR hyperintensities seen involving almost the entire left hemisphere, with some sparing of the superior frontal gyrus, part of cingulate gyrus, antermedial temporal lobe and on the right involving the posterior temporal, medial occipital and scattered areas of the frontal cortex. Bilateral hippocampi, more on left were similarly involved. These regions show restricted diffusion ad post contrast gyrifrom enhancement.