Concluded Case

Sero negative Autoimmune Enfephalitis with Refractory Status Epileptic

1yr10m old baby, perfectly healthy till 4 days ago when she developed fever; Almost continuous ranging from 100- 101°F, developed rt upper limb myoclonic jerks intermittently.on the 3rd day of fever developed generalised recurrent episode of generalised fits.Despite iv midaz the seizures were uncontrolled & hence transferred. Baby was intubated & ventilated getting brief duration GTCS wiyh intermittent myoclonic jerks.Exam showed spasticity both lower limbs with bilateral pyramidal signs.All blood work including vasculitis screening, autoimmune disease work up, CSf analysis including autoimmune encephalitis ,blood & urine for leukodysyrophy etc were -ve. MRI encloded. kindly give yr opinion


Concluded answer

Thanks Curofy and all Doctors who answered the case. Final diagosis : Seronegative autoimmune encephalopathy with super refractoryStatus Epilepticus. Autoimmune work up including NMDA,AMPA 1 & 2,CASPR 2/ VGKC,LG1,GABA done - ve. Antinuclear antibodies and anti DNA done to exclude vasculitis. Baby was on ventilator with multiple drugs and thiopentone ,seizures were uncontrolled. Started on Methyl prednisolone 30 mg / kg / day for 5 days. No change in clinical status. Continuous EEg recording showed diffuse delta waves. Afyer 5 days IVIg started for 5 days. Started reducing thiopental and discontinued still seizures occurred. On almost all available Antiepileptic tried in the optimum dosage. Rituximab 375mg/ m2 started weekly.Two months the child was in the hospital. Finally seizures could control with Sy Sodiu V,Leviteracetam,clonazepam,topiramate ( all other drugs discontinued).At the time of discharge spasticity reduced. Now the child is started walking . last visit in this march.

All Answers

Viral etiology?

Degenerative disorder

Febrile convulsions

This is not febrile convulsion. Kindly review the MRI film.

Globoid cell leukodystrophy / Metachromatic/ leukodystrophy Estimation of aryl sulfatase, galacto cerebrosidase. Please show details of MRI report, is there any chance of Cortical dysplasia associated.

All the above investigated.No cortical dysplasis

Metabolic diseases?

Mam kindly rpt investigations related to sepsis &detail History of prenatal &postnatal &mother's health &feeding

Already mentioned the baby was perfectly healthy till the age of 1yr & 10 months

Can be stroke or epileptic encephalopathies

Ma'am what are the possibilities...Since all the blood work, csf analysis, and blood and urine analysis for the leukodystrophies are negative..Parainfectious myoclonic seizure and sepsis associated encephalopathy ....toxic leucoencephalopathy...

Tòxic/ septic/ Leukodystrophy all investigated All- ve

Congratulations! Your case has been selected as Case of the day and you have been awarded 5 points for sharing the case. Keep posting your interesting cases, Happy Curofying!

If seizures uncontrolled by midaz, give her levepril iv. And orally omnacortil5mg half tablet BD.

Now the seizures are fairly under control.why you want to give omnacortil ? .The clinical details at the time admission in the hospitals
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