Abstract

 
 
 
 Seizure is a common problem in many paediatric patients. When seizures persist even after 24 hrs of anaesthetic treatment it is known as Super Refractory Status Epilepticus (SRSE). We report a 10 years old girl admitted with fever, altered sensorium and GTCS with a provisional diagnosis of viral encephalitis. In the line of SRSE infusion thiopental was also started after giving first line antiepileptic as per guidelines. Seizures persisted despite six anti-epileptic drugs, steroids and infusion anaesthetic. MRI brain was suggestive of T2W Flair hyperintensity in B/L hippocampal and Para-hippocampal gyrus and CSF and blood positive for Mumps IgM. This was suggestive of Mesial temporal sclerosis (MTS) which is the scarring and loss of neurons in the deepest portion of the temporal lobe due to oxygen starvation to the brain, head trauma, brain infection or without any apparent cause. Dramatic response was seen after 36-48 hours of IVIG administration.
 
 
 
Highlights
Paediatric patients presenting with an encephalitic syndrome represent a diagnostic challenge for evaluation and appropriate treatment
Symptoms of mesial temporal sclerosis are generally associated with focal epilepsy.[1]
The hallmark of mesial temporal sclerosis on MR imaging as in our case is an atrophic hippocampus associated with hyperintense signal on long-repetition-time sequences confined to the hippocampus.[5,8]
Summary
Paediatric patients presenting with an encephalitic syndrome represent a diagnostic challenge for evaluation and appropriate treatment. Symptoms of mesial temporal sclerosis are generally associated with focal (partial) epilepsy.[1] Seizures that persist even after giving two antiepileptic drugs (1st line being a BZD) is said to be refractory status epilepticus. MRI showed FLAIR hyperintensity of hippocampal and parahippocampal gyrus (Mesial Temporal Sclerosis, Figure 1 and 2). Neuroimagings including CT and MRI/MRA of the brain revealed no intracranial or neurovascular lesions This ruled out vascular or mass lesions as causes of her encephalopathy. HIV, herpes and syphilis screens were negative At this juncture, the possibility of an autoimmune phenomenon was entertained as all objective data were unrevealing. On day seven of admission due to persistent seizures IVIG was given at 2 gm/kg over five days in the line of autoimmune encephalitis. After three months repeat MRI revealed resolution of the previous abnormalities
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