Ictal EEG Patterns in Band Heterotopia

Grant, Arthur C.; Rho, Jong M.

doi:10.1046/j.1528-1157.2002.42601.x

Cited by 13 publications

(7 citation statements)

References 13 publications

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“…Patients with thin bands show a later onset of seizures which originate mostly in the temporal lobe as shown by scalp EEG [41] . In some cases the discharges evolve into complex partial seizures [42] . On the other hand, patients with thick bands usually start epilepsy early in infancy with mostly generalized seizures, and interictal multifocal abnormalities are generally observed on scalp EEG [43] .…”

Section: Discussionmentioning

confidence: 99%

Epilepsy in Dcx Knockout Mice Associated with Discrete Lamination Defects and Enhanced Excitability in the Hippocampus

et al. 2008

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Patients with Doublecortin (DCX) mutations have severe cortical malformations associated with mental retardation and epilepsy. Dcx knockout (KO) mice show no major isocortical abnormalities, but have discrete hippocampal defects. We questioned the functional consequences of these defects and report here that Dcx KO mice are hyperactive and exhibit spontaneous convulsive seizures. Changes in neuropeptide Y and calbindin expression, consistent with seizure occurrence, were detected in a large proportion of KO animals, and convulsants, including kainate and pentylenetetrazole, also induced seizures more readily in KO mice. We show that the dysplastic CA3 region in KO hippocampal slices generates sharp wave-like activities and possesses a lower threshold for epileptiform events. Video-EEG monitoring also demonstrated that spontaneous seizures were initiated in the hippocampus. Similarly, seizures in human patients mutated for DCX can show a primary involvement of the temporal lobe. In conclusion, seizures in Dcx KO mice are likely to be due to abnormal synaptic transmission involving heterotopic cells in the hippocampus and these mice may therefore provide a useful model to further study how lamination defects underlie the genesis of epileptiform activities.

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Section: Discussionmentioning

confidence: 99%

Epilepsy in Dcx Knockout Mice Associated with Discrete Lamination Defects and Enhanced Excitability in the Hippocampus

et al. 2008

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“…The severity of the clinical presentation correlates with the thickness of the band. The challenges for transition to adult care are the management of refractory epilepsy and intellectual disability . Similar to FLNA‐related PVNH, genetic information is important for counseling patients, whose children have a 50% risk …”

Section: Diffuse Cortical Malformationsmentioning

confidence: 99%

Transition issues for children with diffuse cortical malformations, multifocal postnatal lesions, (infectious and traumatic) and Lennox–Gastaut and similar syndromes

2014

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SUMMARYPatients with epilepsy may have diffuse, serious brain disorders including genetically determined, multilobar malformations, traumatic brain injury, encephalitis and meningitis, and the many causes of Lennox-Gastaut syndrome. Transition to adult care needs to consider concomitant intellectual disability, refractory epilepsy, underlying cause, and other nonneurologic but significant problems, especially for genetic etiologies. Adult epilepsy care coupled with dedicated primary/family care is essential. A multidisciplinary setting may be optimal to address the many issues of clinical care, decision making, custody, and ongoing supervision.

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“…Interictal EEG findings correlate with seizure types and include multifocal and generalized epileptiform discharges. 5 –9 However, ictal EEG findings have rarely been reported. 5,6,10,11…”

Section: Introductionmentioning

confidence: 99%

Ictal/Interictal EEG Patterns and Functional Neuroimaging Findings in Subcortical Band Heterotopia: Report of Three Cases and Review of the Literature

et al. 2008

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Subcortical band heterotopia (SBH) is a rare, genetic disorder of neuronal migration, which is seen almost exclusively in females. Little is known about the functionality of the band heterotopia, in terms of both physiology and pathology, in this malformation. Patients are reported to have several different types of seizures, which are usually drug resistant. Interictal EEG findings are known to correlate with the type of seizures, however less is known about the ictal EEG patterns. We present 3 female patients who were investigated at our center with video-scalp EEG monitoring, interictal single photon emission computed tomography (SPECT), functional magnetic resonance (MR) imaging (fMRI) and MR spectroscopy (MRS) besides routine MR imaging. They had several different types of seizures, and one of them reported also having circling seizures that have not been reported previously in patients with SBH. Ictal EEG recordings were remarkable for their unusual patterns of propagation. The findings in structural and functional neuroradiological investigations are discussed in light of the literature.

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Ictal EEG Patterns in Band Heterotopia

Cited by 13 publications

References 13 publications

Epilepsy in Dcx Knockout Mice Associated with Discrete Lamination Defects and Enhanced Excitability in the Hippocampus

Epilepsy in Dcx Knockout Mice Associated with Discrete Lamination Defects and Enhanced Excitability in the Hippocampus

Transition issues for children with diffuse cortical malformations, multifocal postnatal lesions, (infectious and traumatic) and Lennox–Gastaut and similar syndromes

Ictal/Interictal EEG Patterns and Functional Neuroimaging Findings in Subcortical Band Heterotopia: Report of Three Cases and Review of the Literature

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