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

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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.

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What was child's development prior to illness ? Is there any history of consanguinity ? Any previous sibling died / affected ? What is the head circumference ? Blood & CSF lactate level done ?

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Can be fever induced refractory epilepsy syndrome

Can be stroke or epileptic encephalopathies

Degenerative disorder

Viral etiology?

Encephalitis

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.

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
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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
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