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Hippocampal Changes in Febrile Infection-Related Epilepsy Syndrome (FIRES).

Agarwal A, Sabat S, Thamburaj K, Kanekar S - Pol J Radiol (2015)

Bottom Line: Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature.In summary, acute post-infectious seizures are increasingly being recognized across the globe with the newly coined term 'Febrile infection-related epilepsy syndrome'(FIRES) for this group of immune-mediated epileptic encephalopathy in previously healthy children.This has three phases: episode of simple febrile infection, followed by acute refractory seizures and lastly the chronic phase of neuropsychological impairments and seizures.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Penn State University, Hershey, PA, U.S.A.

ABSTRACT

Background: Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature. However, post-infectious refractory afebrile form of seizures in previously healthy children is being increasingly recognized in around the world, which evolves into a chronic refractory form of epilepsy. The term 'Febrile infection-related epilepsy syndrome' (FIRES) has been proposed for these conditions and represents a refractory severe post-infectious epileptic condition in previously normal children.

Case report: We report the initial and follow-up MR imaging findings in a 5year-old with refractory epilepsy post-febrile seizures.

Conclusions: In summary, acute post-infectious seizures are increasingly being recognized across the globe with the newly coined term 'Febrile infection-related epilepsy syndrome'(FIRES) for this group of immune-mediated epileptic encephalopathy in previously healthy children. This has three phases: episode of simple febrile infection, followed by acute refractory seizures and lastly the chronic phase of neuropsychological impairments and seizures.

No MeSH data available.


Related in: MedlinePlus

MR examination done within 24 hours of status epilepticus. Contiguous T2 images reveal swelling and increased T2 signal of the right hippocampus (black arrows). The left hippocampus (white arrows) shows normal morphology and signal pattern.
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f1-poljradiol-80-391: MR examination done within 24 hours of status epilepticus. Contiguous T2 images reveal swelling and increased T2 signal of the right hippocampus (black arrows). The left hippocampus (white arrows) shows normal morphology and signal pattern.

Mentions: A four-year-old child was found on the floor with a right-sided gaze deviation and tremulousness. The father called 911 and the child was taken to a nearby community hospital where he received Lorazepam and Fosphenytoin. The seizure continued. A CT scan of the head done subsequently was unremarkable. Since the seizure did not stop, the child was transferred to our University Medical Center. The seizure stopped after around 3 hours. Subsequent, neurological examination was unremarkable, except for mildly decreased tone in the lower extremities. The child had no significant prior medical history except for sinus infection around 3 weeks back and was having fevers off and on. MR examination, done on the same day, revealed increased T2 signal and edema involving the right hippocampus (Figure 1) with no pathological contrast enhancement. Radiological differential included encephalitis and post-ictal changes. The latter was felt to be less likely given the isolated involvement of the hippocampus. Viral encephalitis was later ruled out clinically given the absence of fever and an unremarkable lumbar tap. Multifocal epileptiform discharges were recorded in the frontotemporal region on the interictal EEG performed on the second day. The patient was started on Keppra (200 mg b.i.d) and discharged on the third day with no other seizure activity noted in the interim. Over the following months the patient had multiple episodes of self-limiting seizures with frequent hospitalizations. The seizures were refractory with poor response to antiepileptic drugs. Multiple lumbar taps during the course were unremarkable. Interictal EEG obtained twice over a period of 6 months showed generalized epilepiform changes, with predominantly right frontal and temporal origin. No ictal EEG was obtained. Multiple MRI studies of the brain were obtained during that course. Images obtained around 7 months after the initial presentation showed signal changes and atrophy of the right hippocampus along with mild global parenchymal volume loss (Figure 2). There was no recurrence of status epilepticus. The child showed significant cognitive decline over that period.


Hippocampal Changes in Febrile Infection-Related Epilepsy Syndrome (FIRES).

Agarwal A, Sabat S, Thamburaj K, Kanekar S - Pol J Radiol (2015)

MR examination done within 24 hours of status epilepticus. Contiguous T2 images reveal swelling and increased T2 signal of the right hippocampus (black arrows). The left hippocampus (white arrows) shows normal morphology and signal pattern.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4548698&req=5

f1-poljradiol-80-391: MR examination done within 24 hours of status epilepticus. Contiguous T2 images reveal swelling and increased T2 signal of the right hippocampus (black arrows). The left hippocampus (white arrows) shows normal morphology and signal pattern.
Mentions: A four-year-old child was found on the floor with a right-sided gaze deviation and tremulousness. The father called 911 and the child was taken to a nearby community hospital where he received Lorazepam and Fosphenytoin. The seizure continued. A CT scan of the head done subsequently was unremarkable. Since the seizure did not stop, the child was transferred to our University Medical Center. The seizure stopped after around 3 hours. Subsequent, neurological examination was unremarkable, except for mildly decreased tone in the lower extremities. The child had no significant prior medical history except for sinus infection around 3 weeks back and was having fevers off and on. MR examination, done on the same day, revealed increased T2 signal and edema involving the right hippocampus (Figure 1) with no pathological contrast enhancement. Radiological differential included encephalitis and post-ictal changes. The latter was felt to be less likely given the isolated involvement of the hippocampus. Viral encephalitis was later ruled out clinically given the absence of fever and an unremarkable lumbar tap. Multifocal epileptiform discharges were recorded in the frontotemporal region on the interictal EEG performed on the second day. The patient was started on Keppra (200 mg b.i.d) and discharged on the third day with no other seizure activity noted in the interim. Over the following months the patient had multiple episodes of self-limiting seizures with frequent hospitalizations. The seizures were refractory with poor response to antiepileptic drugs. Multiple lumbar taps during the course were unremarkable. Interictal EEG obtained twice over a period of 6 months showed generalized epilepiform changes, with predominantly right frontal and temporal origin. No ictal EEG was obtained. Multiple MRI studies of the brain were obtained during that course. Images obtained around 7 months after the initial presentation showed signal changes and atrophy of the right hippocampus along with mild global parenchymal volume loss (Figure 2). There was no recurrence of status epilepticus. The child showed significant cognitive decline over that period.

Bottom Line: Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature.In summary, acute post-infectious seizures are increasingly being recognized across the globe with the newly coined term 'Febrile infection-related epilepsy syndrome'(FIRES) for this group of immune-mediated epileptic encephalopathy in previously healthy children.This has three phases: episode of simple febrile infection, followed by acute refractory seizures and lastly the chronic phase of neuropsychological impairments and seizures.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Penn State University, Hershey, PA, U.S.A.

ABSTRACT

Background: Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature. However, post-infectious refractory afebrile form of seizures in previously healthy children is being increasingly recognized in around the world, which evolves into a chronic refractory form of epilepsy. The term 'Febrile infection-related epilepsy syndrome' (FIRES) has been proposed for these conditions and represents a refractory severe post-infectious epileptic condition in previously normal children.

Case report: We report the initial and follow-up MR imaging findings in a 5year-old with refractory epilepsy post-febrile seizures.

Conclusions: In summary, acute post-infectious seizures are increasingly being recognized across the globe with the newly coined term 'Febrile infection-related epilepsy syndrome'(FIRES) for this group of immune-mediated epileptic encephalopathy in previously healthy children. This has three phases: episode of simple febrile infection, followed by acute refractory seizures and lastly the chronic phase of neuropsychological impairments and seizures.

No MeSH data available.


Related in: MedlinePlus