Development of hippocampal sclerosis after a complex febrile seizure

Merkenschlager, Andreas; Todt, Horst; Pfluger, Thomas; Bernhard, Matthias K.

doi:10.1007/s00431-008-0776-0

Cited by 9 publications

(8 citation statements)

References 15 publications

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“…Analysis of surgical specimens has shown that the hippocampus is often affected in intractable epilepsy even if the primary seizure focus is extratemporal (Riney et al., ). Evidence from case reports shows that injury caused by prolonged febrile seizures is probably one mechanism leading to hippocampal sclerosis (Merkenschlager et al., ). If the temporal lobe abnormalities observed on MRI are a consequence of seizure‐related damage, neuropathologic studies would be expected to confirm the existence of temporal lobe injury in patients with DS.…”

Section: Discussionmentioning

confidence: 99%

Dravet syndrome: New potential genetic modifiers, imaging abnormalities, and ictal findings

et al. 2013

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Summary Purpose Dravet syndrome is an autosomal dominant epileptic encephalopathy of childhood, which is caused mainly by SCN1A and PCHD19 mutations. Although Dravet syndrome is well recognized, the causes of acute encephalopathy are still elusive, and reported data on ictal electroencephalography (EEG) and structural brain abnormalities are scarce. Methods We studied 30 children who fulfilled the clinical criteria for Dravet syndrome. All patients were screened for SCN1A mutations and 25 for POLG mutations with bidirectional sequencing. Clinical data, including etiologic studies done as part of the clinical workup, were collected from hospital charts. Ictal video‐EEG recordings and magnetic resonance (MR) images were reanalyzed by the authors. Key Findings SCN1A mutations were found in 25 patients (83%). Two SCN1A mutation–negative patients had chromosomal translocations involving chromosomes 9 and X, and one had a mutation in PCDH19. Prolonged seizures were associated with acute encephalopathy in three SCN1A mutation–positive patients. One showed evidence of a significant hypoxic–ischemic event during status epilepticus. The other two demonstrated new persistent neurologic deficits postictally; they both carried heterozygous POLG variants (p.Trp748Ser or p.Gly517Val). Hippocampal sclerosis or loss of gray–white matter definition in the temporal lobe was observed in 7 of 18 patients who had MRI after age 3 years (39%). Motor seizures were recorded on video‐EEG for 15 patients, of whom 12 were younger than 6 years at recording; 11 patients (73%) showed posterior onsets. Significance Our data imply that a heterozygous X;9 translocation and rare POLG variants may modify the clinical features of Dravet syndrome. The latter may increase susceptibility for acute encephalopathy. Temporal lobe abnormalities are common in patients imaged after 3 years of age. Focal seizures seem to localize predominantly in the posterior regions in young children with Dravet syndrome.

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Section: Discussionmentioning

confidence: 99%

Dravet syndrome: New potential genetic modifiers, imaging abnormalities, and ictal findings

et al. 2013

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“…The febrile seizure can damage the hippocampus or other parts of the brain, but this does not exclude the possibility of a preexisting cerebral lesion causing the seizure. Merkenschlager et al demonstrated the development of an HS following a prolonged febrile seizure without any preexisting hippocampal lesion or positive family history [13]. Similarly, our case argues against a preexisting cerebral hemiatrophy that could be excluded by calculating the hemispheric ratio of the early MRI study.…”

Section: Discussionmentioning

confidence: 57%

Cerebral hemiatrophy associated with hippocampal sclerosis following a single prolonged febrile seizure

et al. 2011

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The etiological relation of prolonged febrile seizures with hippocampal sclerosis and cerebral hemiatrophy is controversial. Causal relationship is mainly adopted from retrospective statistical analysis and data from epilepsy surgery. We report a 17-month-old boy who had a prolonged febrile seizure with a transient postictal flaccid hemiparesis and anisocoria. Family history was unremarkable. Magnetic resonance imaging (MRI) revealed abnormal results in the right hippocampal area where diffusion-weighted sequences showed increased signal intensity consistent with acute neuronal edema. Repeat MRI 5 months later demonstrated sclerosis and atrophy of the right hippocampus in association with an increased T2-weighted signal and atrophy of the right frontal, temporal, and parietal lobe. In addition, 18-fluorodeoxyglucose positron emission tomography and 99mTc-ECD single-photon emission computed tomography revealed glucose hypometabolism and decreased perfusion in the right hemisphere, respectively. A final MRI, 12 months following the seizure, was widely unchanged. Interestingly, during a follow-up of 42 weeks, only minor motor deficits were observed. This case uniquely presents the acute onset of hippocampal sclerosis and, consecutively, cerebral hemiatrophy after a single febrile seizure. This suggests that a single prolonged febrile seizure may cause global morphological changes of the brain, not only affecting hippocampal formation.

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“…Several additional reports have demonstrated a similar progression of hippocampal edema to MTS, following febrile seizures in smaller cohorts [38–40]. Taken together, the evidence is clear that MTS may be acquired following prolonged febrile seizures in some patients.…”

Section: Imaging Biomarkers In Tle: Where Do We Stand?mentioning

confidence: 60%

Prospects for Imaging-related Biomarkers of Human Epileptogenesis: a Critical Review

Gomes

Shinnar

2011

Biomarkers Med.

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To facilitate the study of epileptogenesis in humans, noninvasive biomarkers of epileptogenesis are required. No validated biomarker is currently available, but brain imaging techniques provide many attractive candidates. In this article we examine the imaging features of temporal lobe epilepsy, focusing on those that may precede the onset of epilepsy and correlate with epileptogenesis. Hippocampal volumetry and T2 relaxometry are proposed as candidate biomarkers of epileptogenesis in temporal lobe epilepsy following febrile status epilepticus. Preliminary data suggest that these have promise, and the ongoing Consequences of Prolonged Febrile Seizures in Childhood (FEBSTAT) study will provide more conclusive evidence as to their validity. At this time there are no other clear candidates for imaging-related biomarkers of epileptogenesis in human studies.

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Development of hippocampal sclerosis after a complex febrile seizure

Cited by 9 publications

References 15 publications

Dravet syndrome: New potential genetic modifiers, imaging abnormalities, and ictal findings

Dravet syndrome: New potential genetic modifiers, imaging abnormalities, and ictal findings

Cerebral hemiatrophy associated with hippocampal sclerosis following a single prolonged febrile seizure

Prospects for Imaging-related Biomarkers of Human Epileptogenesis: a Critical Review

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