Palliation for catastrophic nonlocalizing epilepsy: a retrospective case series of complete corpus callosotomy at a single institution.

OBJECTIVE: In this study, the authors describe their 10-year single-institution experience with single-step complete corpus callosotomy (CCC) for seizure management in pediatric and adult patients with catastrophic, medically refractory, nonlocalizing epilepsy at Advent Health Orlando. METHODS: The authors conducted a retrospective observational study of patients aged 6 months to 49 years who underwent clinically indicated CCC for drug-resistant nonlocalizing epilepsy at Advent Health Orlando between July 2011 and July 2021. Follow-up ranged from 12 months to 10 years. RESULTS: Of the 101 patients (57% of whom were male) who met eligibility criteria, 81 were pediatric patients and 20 were ≥ 18 years. All patients had seizures that appeared poorly lateralized on both electroencephalograms and clinical semiological studies. Of 54 patients with drop seizures before CCC, 29 (54%) achieved stable freedom from drop seizures after CCC. Of the 101 patients, 14 (13.9%) experienced stable resolution of all types of clinical seizures (International League Against Epilepsy classes 1 and 2). The most common postoperative neurological complication was a transient disconnection syndrome, observed in 50% of patients; of those patients, 73% experienced syndrome resolution within 2 months after surgery, and all resolved by the 2-year follow-up. Formal neuropsychological test results were stable in 13 patients assessed after CCC. CONCLUSIONS: CCC is an effective and well-tolerated palliative surgical technique. In this study, drop attacks were reduced after CCC but could recur for the first time as late as 44 months after surgery. Other seizure types were also reduced postoperatively but could recur for the first time as late as 28 months after surgery. Nearly 14% of patients achieved stable and complete freedom from seizures after CCC. Re-evaluation after CCC can reveal lateralized seizure onset in some patients.

Epilepsy Surgery in Children versus Adults.

Epilepsy is one of the most common chronic neurological disorder affecting 6-7 per 1000 worldwide. Nearly one-third of patients with newly diagnosed epilepsy continue to have recurrent seizures despite adequate trial of more than two anti-seizure drugs : drug-resistant epilepsy (DRE). Children with DRE often experience cognitive and psychosocial co-morbidities requiring more urgent and aggressive treatment than adults. Epilepsy surgery can result in seizure-freedom in approximately two-third of children with improvement in cognitive development and quality of life. Understanding fundamental differences in etiology, co-morbidity, and neural plasticity between children and adults is critical for appropriate selection of surgical candidates, appropriate presurgical evaluation and surgical approach, and improved overall outcome.

Usefulness of intraoperative insular electrocorticography in modified functional hemispherectomy.

BACKGROUND: The insular cortex is not routinely removed in modified functional hemispherectomy due to the risk of injury to the main arteries and to deep structures. Our study evaluates the safety and usefulness of applying intraoperative electrocorticography (ECoG) on the insular during the hemispherectomy. METHODS: We included all patients who underwent insular ECoG during a modified functional hemispherectomy from 2012 to 2015. After the surgery, the decision for further resection of the insular cortex was made based on the presence of electrographic seizures on ECoG. RESULTS: The study included 19 patients (age, 6.4 ± 4.7 years, mean ± standard deviation). Electrographic seizures were identified in 5 patients (26.3%). Sixteen of the 19 patients (84.2%) became seizure-free with a follow-up duration of 3.1 ± 0.6 years and no vascular complication occurred. CONCLUSIONS: Intraoperative insular ECoG monitoring can be performed safely while providing a tailored approach for insular resection during modified hemispherectomy.

Impact of intraoperative 3-T MRI with diffusion tensor imaging on hemispherectomy.

OBJECTIVE Hemispherectomy can produce remarkable seizure control of medically intractable hemispheric epilepsy in children, but some patients continue to have seizures after surgery. A frequent cause of treatment failure is incomplete surgical disconnection of the abnormal hemisphere. This study explores whether intraoperative 3-T MRI with diffusion tensor imaging (DTI) during hemispherectomy can identify areas of incomplete disconnection and allow complete disconnection during a single surgery. METHODS The charts of 32 patients with epilepsy who underwent hemispherectomy between January 2012 and July 2014 at the Florida Hospital for Children were reviewed. Patients were grouped as having had curative or palliative hemispherectomy. To assess the completeness of disconnection when the surgeon considered the operation completed, intraoperative 3-T MRI-DTI was performed. If incomplete disconnection was identified, additional surgery was performed until MRI-DTI sequences confirmed satisfactory disconnection. Seizure outcome data were collected via medical records at last follow-up. RESULTS Of 32 patients who underwent hemispherectomy, 23 had curative hemispherectomy and 9 had palliative hemispherectomy. In 11 of 32 surgeries, the first intraoperative MRI-DTI sequences suggested incomplete disconnection and additional surgery followed by repeat MRI-DTI was performed. Complete disconnection was accomplished in 30 of 32 patients (93.8%). Two of 32 disconnections (6.3%) were incomplete on postoperative imaging. Cross-sectional results showed that 21 of 23 patients (91.3%) who had curative hemispherectomy remained free of seizures (International League Against Epilepsy Class 1) at a median follow-up of 1.7 years (range 0.4-2.9 years). The longitudinal seizure freedom after curative hemispherectomy was 95.2% (SE 0.05) at 6 months, 90.5% (SE 0.06) at 1 year, and 90.5% (SE 0.05) at 2 years. CONCLUSIONS Intraoperative 3-T MRI-DTI sequences can identify incomplete disconnection during hemispherectomy and allow higher rates of complete disconnection in a single surgery. Higher rates of complete disconnection seem to achieve better seizure-free outcome following modified functional hemispherectomy.

Bilateral intracranial EEG with corpus callosotomy may uncover seizure focus in nonlocalizing focal epilepsy.

PURPOSE: To evaluate the value of a new multi-stage surgical procedure using bilateral intracranial electroencephalogram (iEEG) prior and post complete corpus callosotomy (CC) for epileptogenic focus localization. METHOD: Thirty patients with drug-resistant epilepsy underwent bilateral iEEG monitoring to localize epileptogenic focus for surgical treatment. Among them, bisynchronous epileptogenic activities were found in 9 pediatric patients. These 9 patients then received complete CC and continued bilateral iEEG monitoring for further seizure localization. Final surgical treatment decisions were made based on the bilateral iEEG findings post complete CC. The entire multi-stage procedure was performed during the same hospital stay. We retrospectively studied the data from the 9 patients. RESULTS: Seizure onset was lateralized in 3 patients who later received functional hemispherectomy. In another 4 patients, seizure onset was localized, resulting in resective surgery. Bilateral multiple subpial transection was performed on 1 patient with identified bilateral independent seizure onset. One patient did not have seizures following complete CC leading to removal of electrodes without any further resection. Subsequent follow-up showed favorable outcome in all patients: seizure-free in 7, more than 90% reduction in 2. None of the patients experienced surgery related complications during the procedure and follow-up period. CONCLUSION: The multi-stage surgical procedure utilizing iEEG monitoring with CC is a viable option for select patients with catastrophic non-localizing epilepsy. Further study is necessary to find the optimal selection criteria for use of this novel approach.

Real-time functional mapping with electrocorticography in pediatric epilepsy: comparison with fMRI and ESM findings.

SIGFRIED (SIGnal modeling For Real-time Identification and Event Detection) software provides real-time functional mapping (RTFM) of eloquent cortex for epilepsy patients preparing to undergo resective surgery. This study presents the first application of paradigms used in functional magnetic resonance (fMRI) and electrical cortical stimulation mapping (ESM) studies for shared functional cortical mapping in the context of RTFM. Results from the 3 modalities are compared. A left-handed 13-year-old male with intractable epilepsy participated in functional mapping for localization of eloquent language cortex with fMRI, ESM, and RTFM. For RTFM, data were acquired over the frontal and temporal cortex. Several paradigms were sequentially presented: passive (listening to stories) and active (picture naming and verb generation). For verb generation and story processing, fMRI showed atypical right lateralizing language activation within temporal lobe regions of interest and bilateral frontal activation with slight right lateralization. Left hemisphere ESM demonstrated no eloquent language areas. RTFM procedures using story processing and picture naming elicited activity in the right lateral and basal temporal regions. Verb generation elicited strong right lateral temporal lobe activation, as well as left frontal lobe activation. RTFM results confirmed atypical language lateralization evident from fMRI and ESM. We demonstrated the feasibility and usefulness of a new RTFM stimulation paradigm during presurgical evaluation. Block design paradigms used in fMRI may be optimal for this purpose. Further development is needed to create age-appropriate RTFM test batteries.

Resection of ictal high-frequency oscillations leads to favorable surgical outcome in pediatric epilepsy.

PURPOSE: Intracranial electroencephalography (EEG) is performed as part of an epilepsy surgery evaluation when noninvasive tests are incongruent or the putative seizure-onset zone is near eloquent cortex. Determining the seizure-onset zone using intracranial EEG has been conventionally based on identification of specific ictal patterns with visual inspection. High-frequency oscillations (HFOs, >80 Hz) have been recognized recently as highly correlated with the epileptogenic zone. However, HFOs can be difficult to detect because of their low amplitude. Therefore, the prevalence of ictal HFOs and their role in localization of epileptogenic zone on intracranial EEG are unknown. METHODS: We identified 48 patients who underwent surgical treatment after the surgical evaluation with intracranial EEG, and 44 patients met criteria for this retrospective study. Results were not used in surgical decision making. Intracranial EEG recordings were collected with a sampling rate of 2,000 Hz. Recordings were first inspected visually to determine ictal onset and then analyzed further with time-frequency analysis. Forty-one (93%) of 44 patients had ictal HFOs determined with time-frequency analysis of intracranial EEG. KEY FINDINGS: Twenty-two (54%) of the 41 patients with ictal HFOs had complete resection of HFO regions, regardless of frequency bands. Complete resection of HFOs (n = 22) resulted in a seizure-free outcome in 18 (82%) of 22 patients, significantly higher than the seizure-free outcome with incomplete HFO resection (4/19, 21%). SIGNIFICANCE: Our study shows that ictal HFOs are commonly found with intracranial EEG in our population largely of children with cortical dysplasia, and have localizing value. The use of ictal HFOs may add more promising information compared to interictal HFOs because of the evidence of ictal propagation and followed by clinical aspect of seizures. Complete resection of HFOs is a favorable prognostic indicator for surgical outcome.

Neuromagnetic evidence of impaired cortical auditory processing in pediatric intractable epilepsy.

PURPOSE: We aimed to determine the changes in neural correlates of auditory information processing such as auditory detection, encoding, and sensory discrimination in pediatric patients with intractable epilepsy. METHODS: In this magnetoencephalography (MEG) study, 10 patients and 10 age- and gender-matched healthy controls were investigated with the multi-feature mismatch negativity (MMN) paradigm. Latencies and amplitudes of M100, M150, M200, and MMN event-related fields were evaluated. RESULTS: All event-related fields in response to standard stimuli (M100, M150 and M200) and responses to occasional five deviant sounds, deviating from the standard stimuli either in duration, frequency, intensity, location, or by including a silent gap were reduced in amplitude in epilepsy patients compared with healthy controls. CONCLUSIONS: Our study suggests that auditory information processing is impaired in patients with drug-resistant epilepsy, being evident both in stimulus feature encoding (as reflected by changes of early event-related components, e.g., M100) and in cortical sound discrimination (as reflected by MMNm). The neural changes involving diminished M100 as well as MMNms for all five deviant sound types suggest wide-spread auditory information processing impairments in these patients.

[Endoscopic treatment of a pediatric patient with acute pancreatitis caused by anomalous union of pancreaticobiliary duct combined with incomplete pancreatic divisum].

The most common causes of acute pancreatitis are microlithiasis and alcohol. In pediatrics, anomalies in pancreaticobiliary system should be considered as possible causes. Among many anomalies, pancreas divisum associated with anomalous pancreaticobiliary ductal union (APBDU) is very rare. APBDU is associated with acute pancreatitis, choledochal cyst, and gallbladder cancer. Pancreas divisum is also a well known cause of acute recurrent pancreatitis. In adult cases with such conditions, the role of endoscopic management including sphincterotomy or stenting through the Santorini duct is well documented. However, it is still controversial to perform endoscopic retrograde cholangiopancreatography in pediatrics. Herein, we experienced a case of 4 year 7 month old female patient suffered from recurrent attacks of acute pancreatitis, which were caused by APBDU and incomplete pancreas divisum. She was treated by endoscopic sphincterectomy of both openings to the Santorinis and Wirsungs ducts. Thus, we report this interesting case with literature review.

Outcome of surgical treatment in non-lesional intractable childhood epilepsy.

PURPOSE: The aim of this study is to investigate seizure outcomes following epilepsy surgery in non-lesional neocortical pediatric epilepsy. METHODS: We performed a retrospective study on 27 patients with intractable childhood epilepsy who received epilepsy surgery between 1999 and 2006 at Sang-gye Paik Hospital and Severance Children's Hospital. None of the patients had any detectable lesions on MRI. Surgical outcome was assessed at least 2 years postoperatively; clinical characteristics, surgical outcome, pathologic findings, types of surgery, localizing features on SPECT, FDG-PET, and long-term video-EEG were reviewed. RESULTS: Eighteen patients (67%) demonstrated an Engel class I outcome postoperatively. The mean follow-up duration was 4.3 years (range, 2.2-9 years). Eighteen out of 27 cases (67%) showed focal localizing features on ictal SPECT, and 21 of 27 cases (78%) showed abnormal findings on PET. Single lobectomy was the most common procedure, and was performed in 20 patients (74%). Multilobar resection was performed in seven patients (26%). Cortical dysplasia was the most common finding during pathological examination, and was observed in 15 (56%) cases. In addition, gliosis was found in two patients (7%) and non-specific pathological findings were described in 10 patients (37%). CONCLUSIONS: In children with intractable epilepsy and a MRI demonstrating no abnormal lesions, epileptic surgery should be strongly considered when cortical pathology can be identified from other studies.

[A case of giant Brunner's gland hyperplasia combined with adenomyomatous hyperplasia].

Brunner's gland hyperplasia is a rare tumor of the duodenum and might also be an unusual cause of gastrointestinal bleeding. In symptomatic patients, treatment requires either surgical resection or endoscopic polypectomy. We report a case of upper gastrointestinal bleeding from a pedunculated Brunner's gland hyperplasia in the duodenal bulb. Endoscopic resection using the detachable snare and hemoclipping was instituted to remove a large pedunculated polyp. The pathologic diagnosis was Brunner's gland hyperplasia with adenomyomatous hyperplasia.