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|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 4 | Page : 628-629
An interesting case of super-refractory status epilepticus
P Gupta, S Patel, R Ranjan, CS Agrawal
Department of Neurology, Sir Ganga Ram Hospital,New Delhi - 110 060, India
|Date of Web Publication||4-Aug-2015|
Department of Neurology, Sir Ganga Ram Hospital,New Delhi - 110 060
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta P, Patel S, Ranjan R, Agrawal C S. An interesting case of super-refractory status epilepticus. Neurol India 2015;63:628-9
Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic encephalopathy with a yet undefined etiology.  It is a rare disorder with an explosive onset and a potentially fatal course. It is seen in previously healthy children and adolescents following the onset of a nonspecific febrile illness.  It is now turning out to be an important differential diagnosis of refractory and super-refractory status epilepticus (SRSE) in its acute phase. Refractory status epilepticus is defined as status epilepticus that continues despite treatment with benzodiazepines and one antiepileptic drug. SRSE has been used to describe status epilepticus that continues beyond 24 h following initiation of an anesthetic agent.  Only a few case reports and case series of FIRES have been reported. This case was unique because this patient responded well to intravenous immunoglobulin (IVIG) therapy and recovered well without any sequelae. To the best of our knowledge, positron emission tomography-computed tomography (PET-CT) findings of FIRES have also not been described in the past.
A 16-year-old male patient presented with fever for 3 days followed by recurrent seizures. His magnetic resonance imaging (MRI) brain and cerebrospinal fluid (CSF) examination were unremarkable except for mild pleocytosis. He was managed with multiple antiepileptics, antibiotics, and antiviral agents. His auto-immune makers were sent, and he was given IV steroids. Despite treatment, he was having recurrent seizures, so he was referred to our institution. In view of RSE, he was put on ventilatory support. Ongoing antiepileptics and antivirals were continued. He was started on midazolam infusion, but his electroencephalography (EEG) continued to show epileptiform discharges [Figure 1]. He subsequently developed hypotension and was managed with vasopressors. His midazolam infusion was tapered but his EEG continued to show generalized (bifrontal-predominant) discharges. His viral markers, HIV, scrub typhus, dengue, herpes, and mycoplasma serology were negative. Repeat contrast-enhanced MRI (CEMRI) brain was normal. PET-CT revealed focal areas of increased fluorodeoxyglucose uptake in bilateral cerebral hemispheres that were likely to be of inflammatory etiology [Figure 2]. We considered the possibility of autoimmune encephalitis and FIRES. So, we gave a complete course of intravenous immunoglobulin (2 g/kg). He improved gradually and was extubated. His electroencephalogram (EEG) improved markedly. Unfortunately, he developed recurrent seizures and had to be re-intubated and started on midazolam infusion. He later developed Gram-negative septicemia and acute kidney injury and needed dialysis support. He was tracheostomized and gradually weaned off. He recovered and became seizure free, conscious, alert, and oriented and did not develop any sequel.
|Figure 1: Electroencephalograph of the patient showing status epilepticus even after 24 h of intubation|
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|Figure 2: Positron emission tomography scan of the patient showing increased fluorodeoxyglucose uptake|
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Febrile infection-related epilepsy syndrome is a catastrophic epilepsy syndrome that represents an extreme form of epileptic disease subsequent to a febrile episode. It is seen in both children and adults, but is more commonly seen in children. The mean age for FIRES was 8 years in one study.  Our patient was 16-year-old. In most of the instances, fever has disappeared by the time the first seizures occur. An infection related pathogenesis is suspected, but no direct causes have been established. There may be genetic etiology for the disease as seen in Dravet syndrome, but as yet, no causative genes have been identified. An immune basis for FIRES has also been speculated as certain autoimmune antibodies have been found to be elevated in some patients. However, most FIRES patients are mainly autoantibody negative and immune therapy resistant.  The seizure types are mainly focal, and focal with secondary generalization. Our patient had both. In EEG, the onset of seizures is commonly temporal followed by frontal. In our case, the periodic discharges were generalized, but more over bifrontal region, followed by the left temporal region. CEMRI brain is usually normal.  PET-findings in FIRES have not been described frequently in the past. CSF shows normal findings or mild pleocytosis and no pathogen. Tests for the metabolic disease are usually negative.  Patients with FIRES require immediate hospitalization. An anti epileptic drug therapy is often ineffective. A ketogenic diet has been beneficial in some cases especially if initiated early. In one study, 2 out of 8 patients responded to intravenous immunoglobulin (at a dose of 2 g/kg) which was given following the failure of 3 antiepileptics drugs.  The outcome of FIRES is poor with a death rate of up to 30% and mental retardation in 66-100% of survivors with learning difficulties and neurological sequel in the chronic phase. Our patient survived after 3 weeks of ventilatory support without developing a sequel. The mean requirement for ventilatory support was 41 days in one of the previous studies. 
To conclude , febrile infection-related epilepsy syndrome is a rare but a serious syndrome. It should be suspected when a patient presents with acute onset refractory seizures following a simple febrile illness in a previously healthy child. Each case of FIRES with a good recovery should be reported as the course of illness and response to treatment is variable. The accumulated experience will help the clinicians to form some guidelines regarding the treatment as the management at present is largely based on occasional case reports and case series.
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[Figure 1], [Figure 2]