scholarly journals Case Report: PAFAH1B1 Mutation and Posterior Band Heterotopia With Focal Temporal Lobe Epilepsy Treated by Responsive Neurostimulation

2021 ◽  
Vol 12 ◽  
Author(s):  
Frank G. Gilliam ◽  
Paddy Ssentongo ◽  
Michael Sather ◽  
Yuka I. Kawasawa
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Somatic Mosaicism ◽  
Blood Analysis ◽  
Temporal Region ◽  
Depth Electrodes ◽  
Double Cortex Syndrome ◽  
Subcortical Band Heterotopia ◽  
Electrographic Seizures ◽  
Band Heterotopia

Subcortical band heterotopia (SBH), also known as double cortex syndrome, is a malformation of cortical development caused by inherited or somatic gene variants. We present a case of a young adult with posterior SBH and electroclinical features of focal neocortical temporal lobe epilepsy. Genomic blood analysis identified a pathogenic somatic mosaicism duplication variant of the PAFAH1B1 gene. Despite bilateral cortical MRI abnormalities, the interictal and ictal EEG findings indicated a focal epileptogenic region in the left posterior temporal region. Chronic responsive cortical neurostimulation across two four-contact depth electrodes placed 5 mm on either side of the maximal interictal spiking identified during intraoperative electrocorticography resulted in a consistent 28% reduction in duration of electrographic seizures and as well as constricted propagation. Although electrographic seizures continued, the family reported no clinical seizures and a marked improvement in resistant behaviors. This observation supports that focal neocortical neuromodulation can control clinical seizures of consistently localized origin despite genetic etiology, bilateral structural brain abnormalities, and continuation of non-propagating electrographic seizures. We propose that a secondary somatic mutation may be the cause of the focal neocortical temporal lobe epilepsy.

scholarly journals Direct and Collateral Alterations of Functional Cortical Circuits in a Rat Model of Subcortical Band Heterotopia

2018 ◽  
Vol 29 (10) ◽  
pp. 4253-4262 ◽  
Author(s):  
Vanessa Plantier ◽  
Françoise Watrin ◽  
Emmanuelle Buhler ◽  
Fanny Sandrine Martineau ◽  
Surajit Sahu ◽  
...  
Keyword(s):  
Barrel Cortex ◽  
Intractable Epilepsy ◽  
Pyramidal Cells ◽  
Cortical Region ◽  
Cortical Networks ◽  
Causative Gene ◽  
Double Cortex Syndrome ◽  
Subcortical Band Heterotopia ◽  
Migration Disorder ◽  
Band Heterotopia

Abstract Subcortical band heterotopia (SBH), also known as double-cortex syndrome, is a neuronal migration disorder characterized by an accumulation of neurons in a heterotopic band below the normotopic cortex. The majority of patients with SBH have mild to moderate intellectual disability and intractable epilepsy. However, it is still not clear how cortical networks are organized in SBH patients and how this abnormal organization contributes to improper brain function. In this study, cortical networks were investigated in the barrel cortex in an animal model of SBH induced by in utero knockdown of Dcx, main causative gene of this condition in human patients. When the SBH was localized below the Barrel Field (BF), layer (L) four projection to correctly positioned L2/3 pyramidal cells was weakened due to lower connectivity. Conversely, when the SBH was below an adjacent cortical region, the excitatory L4 to L2/3 projection was stronger due to increased L4 neuron excitability, synaptic strength and excitation/inhibition ratio of L4 to L2/3 connection. We propose that these developmental alterations contribute to the spectrum of clinical dysfunctions reported in patients with SBH.

scholarly journals Endonasal endoscopic surgery for temporal lobe epilepsy associated with sphenoidal encephalocele

2021 ◽  
Vol 12 ◽  
pp. 379
Author(s):  
Nobutaka Mukae ◽  
Daisuke Kuga ◽  
Daisuke Murakami ◽  
Noritaka Komune ◽  
Yusuke Miyamoto ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Temporal Region ◽  
Endoscopic Endonasal ◽  
Sphenoidal Sinus ◽  
High Frequency Oscillations ◽  
Depth Electrode ◽  
Electrode Recording ◽  
Subdural Electrode

Background: Temporal lobe epilepsy (TLE) associated with temporal lobe encephalocele is rare, and the precise epileptogenic mechanisms and surgical strategies for such cases are still unknown. Although the previous studies have reported good seizure outcomes following chronic subdural electrode recording through invasive craniotomy, only few studies have reported successful epilepsy surgery through endoscopic endonasal lesionectomy. Case Description: An 18-year-old man developed generalized convulsions at the age of 15 years. Despite treatment with optimal doses of antiepileptic drugs, episodes of speech and reading difficulties were observed 2–3 times per week. Long-term video electroencephalogram (EEG) revealed ictal activities starting from the left anterior temporal region. Magnetic resonance imaging revealed a temporal lobe encephalocele in the left lateral fossa of the sphenoidal sinus (sphenoidal encephalocele). Through the endoscopic endonasal approach, the tip of the encephalocele was exposed. A depth electrode was inserted into the encephalocele, which showed frequent spikes superimposed with high-frequency oscillations (HFOs) suggesting intrinsic epileptogenicity. The encephalocele was resected 8 mm from the tip. Twelve months postoperatively, the patient had no recurrence of seizures on tapering of the medication. Conclusion: TLE associated with sphenoidal encephalocele could be controlled with endoscopic endonasal lesionectomy, after confirming the high epileptogenicity with analysis of HFOs of intraoperative EEG recorded using an intralesional depth electrode.

Modelling the temporal region of patients with temporal lobe epilepsy

1987 ◽  
Vol 32 (1) ◽  
pp. 59-63 ◽  
Author(s):  
D F Rose ◽  
S Sato ◽  
P D Smith ◽  
J White
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Temporal Region

Simultaneous Electroencephalographic and Electocorticographic Recordings of Lateralized Periodic Discharges in Temporal Lobe Epilepsy

2020 ◽  
pp. 155005942097226
Author(s):  
Ayumi Sakata ◽  
Nobutaka Mukae ◽  
Takato Morioka ◽  
Shunya Tanaka ◽  
Takafumi Shimogawa ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Chronic Conditions ◽  
Medial Temporal Lobe ◽  
Temporal Cortex ◽  
Temporal Region ◽  
Pathophysiological Mechanism ◽  
Periodic Discharges ◽  
Pathological Conditions ◽  
Eeg Pattern

Objective Lateralized periodic discharges (LPDs), which constitute an abnormal electroencephalographic (EEG) pattern, are most often observed in critically ill patients with acute pathological conditions, and are less frequently observed in chronic conditions such as focal epilepsies, including temporal lobe epilepsy (TLE). Here we aim to explore the pathophysiological mechanism of LPD in TLE. Methods We retrospectively selected 3 patients with drug-resistant TLE who simultaneously underwent EEG and electrocorticography (ECoG) and demonstrated LPDs. We analyzed the correlation between the EEG and ECoG findings. Results In patients 1 and 2, LPDs were recorded in the temporal region of the scalp during the interictal periods, when repeated spikes followed by slow waves (spike-and-wave complexes; SWs) and periodic discharges (PDs) with amplitudes of >600 to 800 µV appeared in the lateral temporal lobe over a cortical area of >10 cm2. In patient 3, when the ictal discharges persisted and were confined to the medial temporal lobe, repeated SWs were provoked on the lateral temporal lobe. When repeated SWs with amplitudes of >800 µV appeared in an area of the lateral temporal lobe of >10 cm2, the corresponding EEG discharges appeared on the temporal scalp. Conclusions LPDs in patients with TLE originate from repeated SWs and PDs of the lateral temporal lobe, which might represent a highly irritable state of the lateral temporal cortex during both interictal and ictal periods.

Males with epilepsy, complete subcortical band heterotopia, and somatic mosaicism for DCX

Neurology ◽  
2002 ◽  
Vol 58 (10) ◽  
pp. 1559-1562 ◽  
Author(s):  
N. P. Poolos ◽  
S. Das ◽  
G. D. Clark ◽  
D. Lardizabal ◽  
J. L. Noebels ◽  
...  
Keyword(s):  
Somatic Mosaicism ◽  
Subcortical Band Heterotopia ◽  
Band Heterotopia

scholarly journals Prognostic Implication of Contralateral Secondary Electrographic Seizures in Temporal Lobe Epilepsy

Epilepsia ◽  
2000 ◽  
Vol 41 (11) ◽  
pp. 1444-1449 ◽  
Author(s):  
Ki Hyeong Lee ◽  
Yong D. Park ◽  
Don W. King ◽  
Kimford J. Meador ◽  
David W. Loring ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Prognostic Implication ◽  
Electrographic Seizures

scholarly journals A method for placing Heschl gyrus depth electrodes

2010 ◽  
Vol 112 (6) ◽  
pp. 1301-1307 ◽  
Author(s):  
Chandan G. Reddy ◽  
Nader S. Dahdaleh ◽  
Gregory Albert ◽  
Fangxiang Chen ◽  
Daniel Hansen ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Refractory Epilepsy ◽  
Brain Regions ◽  
Frameless Stereotaxy ◽  
Functional Connections ◽  
Depth Electrodes ◽  
Wide Range ◽  
Implantation Method ◽  
Heschl Gyrus

A wide range of devices is used to obtain intracranial electrocorticography recordings in patients with medically refractory epilepsy, including subdural strip and grid electrodes and depth electrodes. Penetrating depth electrodes are required to access some brain regions, and 1 target site that presents a particular technical challenge is the first transverse temporal gyrus, or Heschl gyrus (HG). The HG is located within the supratemporal plane and has an oblique orientation relative to the sagittal and coronal planes. Large and small branches of the middle cerebral artery abut the pial surface of the HG and must be avoided when planning the electrode trajectory. Auditory cortex is located within the HG, and there are functional connections between this dorsal temporal lobe region and medial sites commonly implicated in the pathophysiology of temporal lobe epilepsy. At some surgical centers, depth electrodes are routinely placed within the supratemporal plane, and the HG, in patients who require intracranial electrocorticography monitoring for presumed temporal lobe epilepsy. Information from these recordings is reported to facilitate the identification of seizure patterns in patients with or without auditory auras. To date, only one implantation method has been reported to be safe and effective for placing HG electrodes in a large series of patients undergoing epilepsy surgery. This well-established approach involves inserting the electrodes from a lateral trajectory while using stereoscopic stereotactic angiography to avoid vascular injury. In this report, the authors describe an alternative method for implantation. They use frameless stereotaxy and an oblique insertion trajectory that does not require angiography and allows for the simultaneous placement of subdural grid arrays. Results in 19 patients demonstrate the safety and efficacy of the method.

Safety and Usefulness of Insular Depth Electrodes Implanted Via an Oblique Approach in Patients with Epilepsy

2008 ◽  
Vol 62 (suppl_5) ◽  
pp. ONS471-ONS480 ◽  
Author(s):  
Afif Afif ◽  
Stephan Chabardes ◽  
Lorella Minotti ◽  
Philippe Kahane ◽  
Dominique Hoffmann
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Insular Cortex ◽  
Temporal Epilepsy ◽  
Depth Electrodes ◽  
Small Series ◽  
Group 3 ◽  
Group 2 ◽  
Oblique Approach ◽  
Group 1

Abstract Objective: This study investigates the feasibility, safety, and usefulness of depth electrodes stereotactically implanted within the insular cortex. Methods: Thirty patients with suspected insular involvement during epileptic seizure underwent presurgical stereotactic electroencephalographic recordings using 10 to 16 depth electrodes per patient. Among these, one or two electrodes were implanted via an oblique approach to widely sample the insular cortex. Results: Thirty-five insular electrodes were implanted in the 30 patients without morbidity. A total of 226 recording contacts (mean, 7.5 contacts/patient) explored the insular cortex. Stereotactic electroencephalographic recordings of seizures allowed the differentiation into groups: Group 1, 10 patients with no insular involvement; Group 2, 15 patients with secondary insular involvement; and Group 3, five patients with an initial insular involvement. In temporal epilepsy (n = 17), the insula was never involved at the seizure onset but was frequently involved during the seizures (11 out of 17). In fron-totemporal or frontal epilepsy, the insula was involved at the onset of seizure in five out of 13 patients. All patients in Groups 1 and 2 underwent surgery, with a seizure-free outcome in 76.2% of patients. In Group 3, only two of the five patients underwent surgery, with a poor outcome. In temporal lobe epilepsy, surgical outcome tended to be better in Group 1 compared with Group 2 in this small series: results were good in 83.3% (Engel I) versus 72.7%. Conclusion: Insula can be safely explored with oblique electrodes. In temporal lobe epilepsy, insular involvement does not significantly modify the short-term postoperative outcome. Future larger studies are necessary to clarify the long-term prognostic value of insular spread.

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