Unilateral holohemispheric central nervous system lesions associated with medically refractory epilepsy in the pediatric population: a retrospective series of hemimegalencephaly and Rasmussen's encephalitis

2014 ◽  
Vol 14 (6) ◽  
pp. 573-584 ◽  
Author(s):  
Doris D. Wang ◽  
Barlas Benkli ◽  
Kurtis I. Auguste ◽  
Paul A. Garcia ◽  
Joseph Sullivan ◽  
...  
Keyword(s):  
San Francisco ◽  
Refractory Epilepsy ◽  
Pediatric Population ◽  
Case Series ◽  
The Other ◽  
Class Iii ◽  
Seizure Reduction ◽  
Rasmussen’S Encephalitis ◽  
Pathology Data ◽  
Medically Refractory Epilepsy

Object Cortical malformations and inflammatory encephalopathy are among common etiologies for medically refractory epilepsy in children. On rare occasions, lesions can affect an entire cerebral hemisphere while sparing the other; the 2 processes that can manifest in this manner are hemimegalencephaly (HME) and Rasmussen's encephalitis (RE). Although the clinical course and radiological appearance between the 2 disorders are distinct, there is occasional overlapping pathology between RE and cortical migration disorders. One question that arises from these observations is whether RE and HME, diseases with holohemispheric involvement but apparently different etiologies, have any overlapping characteristics. Methods The authors performed a retrospective review of all patients with presumed diagnosis of HME or RE who underwent hemispherectomy at University of California, San Francisco, and reviewed their clinical presentation, imaging, and pathology data. Results The authors present the clinicopathological features of 14 pediatric patients with unilateral holohemispheric lesions associated with medically refractory epilepsy. Radiological and pathological assessment classified 7 of the patients as having hemimegalencephaly, while the other 7 were diagnosed as having RE. Four of the patients had unusual features suggestive of overlapping developmental and inflammatory (dual) pathology. All patients underwent hemispherectomies. Eight patients (57%) became seizure free (Engel Class I), 5 patients (36%) had rare seizures (Engel Class II), and 1 patient had significant seizure reduction (Engel Class III). Conclusions Based on this case series, HME and RE can be distinguished on the basis of their radiological and histological appearance, even though some cases may have overlapping features. Hemispherectomy was effective at eliminating seizures for both HME and RE.

Efficacy and safety of corpus callosotomy after vagal nerve stimulation in patients with drug-resistant epilepsy

2018 ◽  
Vol 128 (1) ◽  
pp. 277-286 ◽  
Author(s):  
Jennifer Hong ◽  
Atman Desai ◽  
Vijay M. Thadani ◽  
David W. Roberts
Keyword(s):  
Nerve Stimulation ◽  
Refractory Epilepsy ◽  
Case Series ◽  
Vagal Nerve ◽  
Vagal Nerve Stimulation ◽  
Class Iii ◽  
Corpus Callosotomy ◽  
Few Data ◽  
Medically Refractory Epilepsy

OBJECTIVEVagal nerve stimulation (VNS) and corpus callosotomy (CC) have both been shown to be of benefit in the treatment of medically refractory epilepsy. Recent case series have reviewed the efficacy of VNS in patients who have undergone CC, with encouraging results. There are few data, however, on the use of CC following VNS therapy.METHODSThe records of all patients at the authors' center who underwent CC following VNS between 1998 and 2015 were reviewed. Patient baseline characteristics, operative details, and postoperative outcomes were analyzed.RESULTSTen patients met inclusion criteria. The median follow-up was 72 months, with a minimum follow-up of 12 months (range 12–109 months). The mean time between VNS and CC was 53.7 months. The most common reason for CC was progression of seizures after VNS. Seven patients had anterior CC, and 3 patients returned to the operating room for a completion of the procedure. All patients had a decrease in the rate of falls and drop seizures; 7 patients experienced elimination of drop seizures. Nine patients had an Engel Class III outcome, and 1 patient had a Class IV outcome. There were 3 immediate postoperative complications and 1 delayed complication. One patient developed pneumonia, 1 developed transient mutism, and 1 had persistent weakness in the nondominant foot. One patient presented with a wound infection.CONCLUSIONSThe authors demonstrate that CC can help reduce seizures in patients with medically refractory epilepsy following VNS, particularly with respect to drop attacks.

Vagal nerve stimulation for the treatment of medically refractory epilepsy: a review of the current literature

2012 ◽  
Vol 32 (3) ◽  
pp. E12 ◽  
Author(s):  
David E. Connor ◽  
Menarvia Nixon ◽  
Anil Nanda ◽  
Bharat Guthikonda
Keyword(s):  
Nerve Stimulation ◽  
Refractory Epilepsy ◽  
Epileptiform Activity ◽  
Vagal Nerve ◽  
Vagal Nerve Stimulation ◽  
Class I ◽  
Class Iii ◽  
Surgical Evaluation ◽  
Pediatric Populations ◽  
Medically Refractory Epilepsy

Object The authors conducted a study to evaluate the published results of vagal nerve stimulation (VNS) for medically refractory seizures according to evidence-based criteria. Methods The authors performed a review of available literature published between 1980 and 2010. Inclusion criteria for articles included more than 10 patients evaluated, average follow-up of 1 or more years, inclusion of medically refractory epilepsy, and consistent preoperative surgical evaluation. Articles were divided into 4 classes of evidence according to criteria established by the American Academy of Neurology. Results A total of 70 publications were reviewed, of which 20 were selected for review based on inclusion and exclusion criteria. There were 2 articles that provided Class I evidence, 7 that met criteria for Class II evidence, and 11 that provided Class III evidence. The majority of evidence supports VNS usage in partial epilepsy with a seizure reduction of 50% or more in the majority of cases and freedom from seizure in 6%–27% of patients who responded to stimulation. High stimulation with a gradual increase in VNS stimulation over the first 6 weeks to 3 months postoperatively is well supported by Class I and II data. Predictors of positive response included absence of bilateral interictal epileptiform activity and cortical malformations. Conclusions Vagal nerve stimulation is a safe and effective alternative for adult and pediatric populations with epilepsy refractory to medical and other surgical management.

scholarly journals Modified hemispherectomy for infantile hemiparesis and epilepsy

2020 ◽  
Vol 11 (1) ◽  
pp. 380-390
Author(s):  
Yu-Hui Li ◽  
Dong-Sheng Li ◽  
Mei-Qing Wang ◽  
Kai Zhao ◽  
Bu-Lang Gao
Keyword(s):  
Medical Imaging ◽  
Refractory Epilepsy ◽  
The Other ◽  
Pathological Examination ◽  
Cerebral Peduncle ◽  
Long Term Follow Up ◽  
Healthy Side ◽  
Medically Refractory Epilepsy

AbstractObjectiveTo investigate the effect and medical imaging of modified hemispherectomy on patients with infantile hemiparesis and medically refractory epilepsy.Patients and methodsForty-three patients with infantile hemiparesis and refractory epilepsy who underwent hemispherectomy were enrolled. The treatment effect and medical imaging were analyzed.ResultsAnatomical hemispherectomy was successfully performed in all patients (100%). In all patients, the muscular tension decreased and the contracted limbs relaxed. In the pathological examination of the resected brain tissue, secondary cicatricial gyri with concomitant cortical dysplasia was present in 36 cases and polycerebellar gyrus malformation and porencephalia in the other 7 cases. Followed up for 7–15 years (mean 11.3), all patients were alive without a long-term sequela. Epilepsy was satisfactorily controlled, with complete seizure relief in 39 cases (91%) classified as Engel I and basic control in the other 4 (9%) defined as Engel II. The posthemispherectomy medical imaging demonstrated that the intracranial space on the operative side shrank, and the healthy cerebral hemisphere shifted markedly toward the hemispherectomy side, with expanded lateral ventricle on the healthy side and thickened skull and enlarged frontal sinus on the operative side. After 4–5 years, the intracranial space on the operative side disappeared in 75% of the patients, demonstrating enlarged cerebral peduncle on the healthy side.ConclusionFurther modified hemispherectomy in patients with infantile hemiparesis and medically refractory epilepsy demonstrated markedly ameliorated effects on epilepsy control and the prevention of superficial cerebral hemosiderosis in the long-term follow-up.

Management of patients with medically intractable epilepsy and anterior temporal lobe encephaloceles

2021 ◽  
pp. 1-8
Author(s):  
Mani Ratnesh S. Sandhu ◽  
Mauricio Mandel ◽  
Hari McGrath ◽  
Layton Lamsam ◽  
Pue Farooque ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Refractory Epilepsy ◽  
Preoperative Evaluation ◽  
Intractable Epilepsy ◽  
Class Iii ◽  
Single Institution ◽  
Scalp Eeg ◽  
Surgical Data ◽  
Medically Refractory Epilepsy

OBJECTIVE Temporal lobe encephaloceles (TLENs) are a significant cause of medically refractory epilepsy, but there is little consensus regarding their workup and treatment. This study characterizes these lesions and their role in seizures and aims to standardize preoperative evaluation and surgical management. METHODS Patients with TLEN who had undergone resective epilepsy surgery from December 2015 to August 2020 at a single institution were included in the study. Medical records were reviewed for each patient to collect relevant seizure workup information including demographics, radiological findings, surgical data, and neuropsychological evaluation. RESULTS For patients who presented to the authors’ program with suspected medically intractable temporal lobe epilepsy (219 patients), TLEN was considered to be the epileptogenic focus in 5.5%. Ten patients with TLEN had undergone resection and were included in this study. Concordance between ictal scalp electroencephalography (EEG) lateralization and TLEN was found in 9/10 patients (90%), and 4/10 patients (40%) had signs suggestive of idiopathic intracranial hypertension (IIH). Surgical outcome was reported in patients with at least 12 months of follow-up (9/10). Patients with scalp EEG findings concordant with the TLEN side had a good outcome (Engel class I: 7 patients, class II: 1 patient). One patient with discordant EEG findings had a bad outcome (Engel class III). No significant neuropsychological deficits were observed after the surgery. CONCLUSIONS TLENs are epileptogenic lesions that should be screened for in patients with medically refractory epilepsy who have signs of IIH and no other lesions on MRI. Restricted resection is safe and effective in patients with scalp EEG findings concordant with TLEN.

Efficacy of Vagal Nerve Stimulation in Children with Medically Refractory Epilepsy

Neurosurgery ◽  
2000 ◽  
Vol 47 (6) ◽  
pp. 1353-1358 ◽  
Author(s):  
Ravish V. Patwardhan ◽  
Benjamin Stong ◽  
E. Martina Bebin ◽  
Jan Mathisen ◽  
Paul A. Grabb
Keyword(s):  
Quality Of Life ◽  
Seizure Frequency ◽  
Nerve Stimulation ◽  
Refractory Epilepsy ◽  
Vagal Nerve ◽  
Seizure Type ◽  
Seizure Reduction ◽  
Medically Refractory Epilepsy

ABSTRACT OBJECTIVE The effects of vagal nerve stimulation (VNS) on seizure frequency and quality of life were analyzed retrospectively in children with medically refractory epilepsy. METHODS Thirty-eight children aged 11 months to 16 years underwent implantation of vagal nerve stimulators. Age of seizure onset, duration of epilepsy, and seizure type and frequency were recorded preoperatively. Age at implantation, length of follow-up, seizure type and frequency, and change in quality of life (QOL) were recorded postoperatively. Changes in QOL were assigned a QOL score by the caretakers on a visual analog scale of −1 (much worse) to +1 (much improved). RESULTS The median follow-up period was 12 months (range, 10–18 mo). Eleven (29%), 15 (39%), 5 (13%), and 7 (18%) children had greater than 90% reduction, 50 to 90% reduction, less than 50% reduction, and no reduction in seizure frequency, respectively. For all children, seizure reduction by seizure type was as follows: atonic (80%), absence (65%), complex partial (48%), and generalized tonicoclonic (45%). The mean change in QOL score was 0.61. Eighty-six percent of the children had QOL scores of 0.5 (improved) or higher. Follow-up of at least 6 months was associated with greater seizure reduction (P = 0.05) and higher QOL score (P < 0.01). Seizure reduction was greater in children with onset of epilepsy after 1 year of age (P < 0.05). The age of the child and duration of epilepsy were not associated with greater or lesser degrees of seizure reduction. CONCLUSION VNS provided improvements in seizure control for the majority of children regardless of age. QOL was improved in the majority of children with VNS. VNS should be considered for children with medically refractory epilepsy who have no surgically resectable focus.

scholarly journals Corpus callosotomy performed with laser interstitial thermal therapy

2019 ◽  
pp. 1-9 ◽  
Author(s):  
Jarod L. Roland ◽  
Syed Hassan A. Akbari ◽  
Afshin Salehi ◽  
Matthew D. Smyth
Keyword(s):  
Refractory Epilepsy ◽  
Effective Means ◽  
Thermal Therapy ◽  
Class Iii ◽  
Corpus Callosotomy ◽  
Invasive Approach ◽  
Laser Interstitial Thermal Therapy ◽  
Less Invasive ◽  
Medically Refractory Epilepsy

OBJECTIVECorpus callosotomy is a palliative procedure that is effective at reducing seizure burden in patients with medically refractory epilepsy. The procedure is traditionally performed via open craniotomy with interhemispheric microdissection to divide the corpus callosum. Concerns for morbidity associated with craniotomy can be a deterrent to patients, families, and referring physicians for surgical treatment of epilepsy. Laser interstitial thermal therapy (LITT) is a less invasive procedure that has been widely adopted in neurosurgery for the treatment of tumors. In this study, the authors investigated LITT as a less invasive approach for corpus callosotomy.METHODSThe authors retrospectively reviewed all patients treated for medically refractory epilepsy by corpus callosotomy, either partial or completion, with LITT. Chart records were analyzed to summarize procedural metrics, length of stay, adverse events, seizure outcomes, and time to follow-up. In select cases, resting-state functional MRI was performed to qualitatively support effective functional disconnection of the cerebral hemispheres.RESULTSTen patients underwent 11 LITT procedures. Five patients received an anterior two-thirds LITT callosotomy as their first procedure. One patient returned after LITT partial callosotomy for completion of callosotomy by LITT. The median hospital stay was 2 days (IQR 1.5–3 days), and the mean follow-up time was 1.0 year (range 1 month to 2.86 years). Functional outcomes are similar to those of open callosotomy, with the greatest effect in patients with a significant component of drop attacks in their seizure semiology. One patient achieved an Engel class II outcome after anterior two-thirds callosotomy resulting in only rare seizures at the 18-month follow-up. Four others were in Engel class III and 5 were Engel class IV. Hemorrhage occurred in 1 patient at the time of removal of the laser fiber, which was placed through the bone flap of a prior open partial callosotomy.CONCLUSIONSLITT appears to be a safe and effective means for performing corpus callosotomy. Additional data are needed to confirm equipoise between open craniotomy and LITT for corpus callosotomy.

Consequences of mesial temporal sparing temporal lobe surgery in medically refractory epilepsy

2021 ◽  
Vol 115 ◽  
pp. 107642
Author(s):  
Lilach Goldstein ◽  
Mitra Dehghan Harati ◽  
Kathryn Devlin ◽  
Joseph Tracy ◽  
Maromi Nei ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Refractory Epilepsy ◽  
Medically Refractory Epilepsy

A historical cohort of temporal lobe surgery for medically refractory epilepsy: a systematic review and meta-analysis to guide future nonrandomized controlled trial studies

2020 ◽  
Vol 133 (1) ◽  
pp. 71-78 ◽  
Author(s):  
Anthony T. Lee ◽  
John F. Burke ◽  
Pranathi Chunduru ◽  
Annette M. Molinaro ◽  
Robert Knowlton ◽  
...  
Keyword(s):  
Systematic Review ◽  
Temporal Lobe ◽  
Epilepsy Surgery ◽  
Refractory Epilepsy ◽  
Meta Analysis ◽  
Small Sample ◽  
Seizure Freedom ◽  
Level I ◽  
Study Designs ◽  
Medically Refractory Epilepsy

OBJECTIVERecent trials for temporal lobe epilepsy (TLE) highlight the challenges of investigating surgical outcomes using randomized controlled trials (RCTs). Although several reviews have examined seizure-freedom outcomes from existing data, there is a need for an overall seizure-freedom rate estimated from level I data as investigators consider other methods besides RCTs to study outcomes related to new surgical interventions.METHODSThe authors performed a systematic review and meta-analysis of the 3 RCTs of TLE in adults and report an overall surgical seizure-freedom rate (Engel class I) composed of level I data. An overall seizure-freedom rate was also collected from level II data (prospective cohort studies) for validation. Eligible studies were identified by filtering a published Cochrane meta-analysis of epilepsy surgery for RCTs and prospective studies, and supplemented by searching indexed terms in MEDLINE (January 1, 2012–April 1, 2018). Retrospective studies were excluded to minimize heterogeneity in patient selection and reporting bias. Data extraction was independently reverified and pooled using a fixed-effects model. The primary outcome was overall seizure freedom following surgery. The historical benchmark was applied in a noninferiority study design to compare its power to a single-study cohort.RESULTSThe overall rate of seizure freedom from level I data was 72.4% (55/76 patients, 3 RCTs), which was nearly identical to the overall seizure-freedom rate of 71.7% (1325/1849 patients, 18 studies) from prospective cohorts (z = 0.134, p = 0.89; z-test). Seizure-freedom rates from level I and II studies were consistent over the years of publication (R2< 0.01, p = 0.73). Surgery resulted in markedly improved seizure-free outcomes compared to medical management (RR 10.82, 95% CI 3.93–29.84, p < 0.01; 2 RCTs). Noninferiority study designs in which the historical benchmark was used had significantly higher power at all difference margins compared to using a single cohort alone (p < 0.001, Bonferroni’s multiple comparison test).CONCLUSIONSThe overall rate of seizure freedom for temporal lobe surgery is approximately 70% for medically refractory epilepsy. The small sample size of the RCT cohort underscores the need to move beyond standard RCTs for epilepsy surgery. This historical seizure-freedom rate may serve as a useful benchmark to guide future study designs for new surgical treatments for refractory TLE.

Congenital Diaphragmatic Hernia: Demographics and 30-day Outcomes in Adults

2020 ◽  
pp. 000313482096006
Author(s):  
Joseph G. Brungardt ◽  
Quinn A. Nix ◽  
Kurt P. Schropp
Keyword(s):  
Postoperative Complications ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Pediatric Population ◽  
Case Series ◽  
National Database ◽  
Improvement Program ◽  
American College Of Surgeons ◽  
Postoperative Diagnosis ◽  
Acs Nsqip Database

Background Congenital diaphragmatic hernia (CDH) is a pathology most often affecting the pediatric population, although adults can also be affected. Few studies exist of adults undergoing repair of this defect. Using a national database, we sought to determine demographics and outcomes of this population. Methods An analysis of the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database (2015-2018) was performed, capturing patients with postoperative diagnosis of CDH, distinct from ventral hernia. Two groups were created based upon surgical approach of open or minimally invasive (MIS) repair. Baseline demographics and outcomes were compared. Results 110 patients undergoing surgical correction of CDH were captured in the database. We found rates of return to the operating room (4.55%), postoperative respiratory failure (5.45%), and reintubation (3.64%) with no difference between groups. There was no mortality and no difference between groups in length of operation, discharge to home, or postoperative complications. Patients undergoing open repair had a longer length of stay than patients in the MIS group (6.47 ± 10.76 days vs. 3.68 ± 3.74 days, P = .0471). Mesh was used in MIS more often than the open group (47.30% vs. 5.56%, P < .0001). Discussion This study describes rates of postoperative complications in patients undergoing repair of CDH, and suggests outcomes those are equivalent between patients receiving open or MIS approaches. Further case series or retrospective studies are needed to further describe this population of patients.

scholarly journals Durable ventricular assist device implantation for systemic right ventricle: a case series

2020 ◽  
Author(s):  
Naoki Tadokoro ◽  
Satsuki Fukushima ◽  
Takaya Hoashi ◽  
Shin Yajima ◽  
Takura Taguchi ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Ventricular Assist Device ◽  
Case Series ◽  
Transoesophageal Echocardiography ◽  
Class Iii ◽  
Assist Device ◽  
Ventricular Assist ◽  
Systemic Right Ventricle ◽  
Computed Tomography Images ◽  
Atrial Switch

Abstract Background A systemic right ventricle (RV) after atrial switch in transposition of the great arteries (TGA) or congenitally corrected TGA (ccTGA) often results in advanced heart failure in adulthood. Case summary Four patients with INTERMACS Class III underwent durable ventricular assist device (VAD) implantation for a systemic RV. Two patients were diagnosed with ccTGA and underwent tricuspid valve replacement, and two were diagnosed with TGA in childhood and underwent Mustard repair. The two patients with ccTGA received an EVAHEART (Sun Medical, Nagano, Japan) and HeartMate 3 (Abbott Laboratories, Abbott Park, IL, USA) at the age of 56 years and 34 years, respectively. Of the patients with TGA, one received a Heartmate II at age 40 years, and one received a HeartMate 3 at age 40 years. All patients were weaned from cardiopulmonary bypass without subpulmonic VAD support and transferred to the intensive care unit with optimum VAD support. No in-hospital deaths, cerebrovascular accidents, or other major complications occurred. The post-VAD right heart catheter study showed a remarkable reduction in pulmonary capillary wedge pressure in all patients. Discussion The indications for and surgical technique of durable VAD implantation for a systemic RV after atrial switch of TGA or ccTGA have not been fully established. A durable VAD, including the HeartMate 3, was successfully implanted in four such patients in this study. Pre-operative three-dimensional computed tomography images and intraoperative transoesophageal echocardiography guidance helped to determine the positions of the inflow and pump.

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