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Gary W Mathern - One of the best experts on this subject based on the ideXlab platform.

  • long term patient reported outcomes of visual field defects and compensatory mechanisms in patients after cerebral Hemispherectomy
    2021
    Co-Authors: Elana Meer, Gary W Mathern, Monica F Chen, Monika Jones, Stacy L Pineles
    Abstract:

    BACKGROUND In cases of intractable epilepsy resistant to drug therapy, Hemispherectomy is often the only treatment option to mitigate seizures; however, the true long-term subjective visual outcomes are relatively unexplored. In this study, we sought to determine and characterize patient-reported visual function years after Hemispherectomy. METHODS This was an observational study conducted on a large cohort of children with seizure disorder treated with cerebral Hemispherectomy. An online survey was sent to parents with questions to assess subjective visual function with a variety of questions from presence of visual field defects after Hemispherectomy, to improvement over time, compensatory mechanisms used, and development of strabismus. RESULTS This survey was emailed to 248 parents of previously evaluated children who agreed to be re-surveyed, 48 (20%) of which responded. The average age at Hemispherectomy was approximately 5 (±4) years, and the average time after Hemispherectomy was 7 (±5) years. Thirty-nine patients (81%) were seizure-free after 1 surgery and 85% (n = 41) were seizure-free after ≥1 surgeries. Thirty-four (71%) experienced a visual field defect after surgery, but 25 (52%) experienced subjective improvement over time. Thirty-eight (79%) used compensatory mechanisms, such as head tilting, with 16 (33%) patients experiencing subjective improvement over time. Twenty-seven (56%) patients experienced a decrease in visual acuity after surgery with 12 (25%) experiencing subjective improvement over time. CONCLUSION In a large cohort examining patient-reported visual outcomes years after Hemispherectomy, most patients experienced strabismus and/or visual field defects. However, more than half reported improvements and compensatory mechanisms (exotropic strabismus and ipsilateral esotropic strabismus) over time, presumably to enhance visual field function. By exploring subjective visual and cognitive function, this paper uniquely characterizes patient-reported improvements over time, and provides motivation for larger longitudinal studies using more quantitative measures of visual function and improvement after Hemispherectomy.

  • epilepsy surgery for rasmussen encephalitis the ucla experience
    2020
    Co-Authors: Nikhil Bellamkonda, Gary W Mathern, Westley H Phillips, Jiashu Chen, Alexander M Tucker, Cassia Maniquis, Aria Fallah
    Abstract:

    Objective Rasmussen encephalitis (RE) is a rare inflammatory neurological disorder typically involving one hemisphere and resulting in drug-resistant epilepsy and progressive neurological decline. Here, the authors present seizure outcomes in children who underwent epilepsy surgery for RE at a single institution. Methods The records of consecutive patients who had undergone epilepsy surgery for RE at the UCLA Mattel Children's Hospital between 1982 and 2018 were retrospectively reviewed. Basic demographic information, seizure history, procedural notes, and postoperative seizure and functional outcome data were analyzed. Results The cohort included 44 patients, 41 of whom had sufficient data for analysis. Seizure freedom was achieved in 68%, 48%, and 22% of the patients at 1, 5, and 10 years, respectively. The median time to the first seizure for those who experienced seizure recurrence after surgery was 39 weeks (IQR 11-355 weeks). Anatomical Hemispherectomy, as compared to functional Hemispherectomy, was independently associated with a longer time to postoperative seizure recurrence (HR 0.078, p = 0.03). There was no statistically significant difference in postoperative seizure recurrence between patients with complete Hemispherectomy and those who had less-than-hemispheric surgery. Following surgery, 68% of the patients could ambulate and 84% could speak regardless of operative intervention. Conclusions A large proportion of RE patients will have seizure relapse after surgery, though patients with anatomical hemispherectomies may have a longer time to postoperative seizure recurrence. Overall, the long-term data in this study suggest that hemispheric surgery can be seen as palliative treatment for seizures rather than a cure for RE.

  • etiology and age modifies subjective visual function after cerebral Hemispherectomy
    2019
    Co-Authors: Monica F Chen, Gary W Mathern, Elana Meer, Monika Jones, Federico G Velez, Stacy L Pineles
    Abstract:

    Background:Cerebral Hemispherectomy is typically used to treat patients with pharmacoresistant epilepsy. Visual-related outcomes are relatively unstudied in this population, aside from the knowledg...

  • corticospinal tract atrophy and motor fmri predict motor preservation after functional cerebral Hemispherectomy
    2018
    Co-Authors: Anthony C Wang, Aria Fallah, Gary W Mathern, Robert T Buckley, George M Ibrahim, Andrew Poliakov, Page I Wang, Kelly L Collins, Alexander G Weil, Hillary Shurtleff
    Abstract:

    OBJECTIVE The potential loss of motor function after cerebral Hemispherectomy is a common cause of anguish for patients, their families, and their physicians. The deficits these patients face are individually unique, but as a whole they provide a framework to understand the mechanisms underlying cortical reorganization of motor function. This study investigated whether preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI) could predict the postoperative preservation of hand motor function. METHODS Thirteen independent reviewers analyzed sensorimotor fMRI and colored fractional anisotropy (CoFA)-DTI maps in 25 patients undergoing functional Hemispherectomy for treatment of intractable seizures. Pre- and postoperative gross hand motor function were categorized and correlated with fMRI and DTI findings, specifically, abnormally located motor activation on fMRI and corticospinal tract atrophy on DTI. RESULTS Normal sensorimotor cortical activation on preoperative fMRI was significantly associated with severe decline in postoperative motor function, demonstrating 92.9% sensitivity (95% CI 0.661-0.998) and 100% specificity (95% CI 0.715-1.00). Bilaterally robust, symmetric corticospinal tracts on CoFA-DTI maps were significantly associated with severe postoperative motor decline, demonstrating 85.7% sensitivity (95% CI 0.572-0.982) and 100% specificity (95% CI 0.715-1.00). Interpreting the fMR images, the reviewers achieved a Fleiss' kappa coefficient (κ) for interrater agreement of κ = 0.69, indicating good agreement (p < 0.01). When interpreting the CoFA-DTI maps, the reviewers achieved κ = 0.64, again indicating good agreement (p < 0.01). CONCLUSIONS Functional Hemispherectomy offers a high potential for seizure freedom without debilitating functional deficits in certain instances. Patients likely to retain preoperative motor function can be identified prior to Hemispherectomy, where fMRI or DTI suggests that cortical reorganization of motor function has occurred prior to the operation.

  • complex syntax in the isolated right hemisphere receptive grammatical abilities after cerebral Hemispherectomy
    2015
    Co-Authors: Stella De Bode, Gary W Mathern, Lieselotte Smets, Stanley Dubinsky
    Abstract:

    article i nfo Objectives: In this study, we explored the syntactic competence of the right hemisphere (RH) after left cerebral Hemispherectomy, on the premise that it (syntactic competence) is known to be one of the most strongly left- lateralized language functions. As basic syntactic development for individuals in this subject pool has already been extensively explored, we focused instead on the investigation of complex syntactic constructions that are normally acquired later in childhood, i.e., between 7 and 9 years of age. Methods: Grammatical competence in 10 participants who had undergone left cerebral Hemispherectomy was compared to that of a group of normally developing children, with the two groups matched by the size of their vocabulary. The two tests we used for this research were created by the 1st language acquisition linguists and weredesignedtotestsetsofconstructionscategorizedanddifferentiatedbytheorderinwhichtheyarenormally acquired and by the type of grammatical competence that they involve. Results: We found that both groups followed the same developmental sequence of syntactic development with five (50%) postsurgical participants (all with prenatal etiologies) reaching nearly mature command of sentence grammar. Seizures negatively impacted performance on all tests. Conclusions: The isolated RH has the potential to support the complex grammatical categories that emerge rela- tively late in the normal acquisition of English by native speakers. Successful performance may be related to the timing of the initial insult and seizure control following Hemispherectomy.

William Bingaman - One of the best experts on this subject based on the ideXlab platform.

  • vasospasm following Hemispherectomy a case report of a novel complication
    2020
    Co-Authors: Amr Morsi, Ahsan N. V. Moosa, Andres Maldonado, Dennis Lal, Elia Pestanaknight, William Bingaman
    Abstract:

    Background Hemispherectomy has been shown to be successful in treating medically intractable epilepsy, with favorable seizure-free outcomes. However, the procedure is technically challenging with high rates of in-hospital complications. We present a unique case of functional Hemispherectomy complicated by diffuse cerebral vasospasm and subsequent death in a patient with COL4A1 gene mutation. Case Description A 17-year-old boy presented with right hemispheric epilepsy and a previously diagnosed autosomal dominant heterozygous COL4A1 gene mutation (c.4380T>G;p.Cys1460Trp). Functional Hemispherectomy was performed without complications. On postoperative day 8, he developed an acute decline in neurologic status requiring urgent intubation for airway protection. Magnetic resonance imaging revealed areas of restricted diffusion throughout bilateral hemispheres that was explained by severe vasospasm and minimal cerebral blood flow seen on cerebral angiography. Intra-arterial calcium channel blocker infusion and balloon angioplasty were attempted without improvement in perfusion. With a worsening clinical picture, he was transitioned to comfort care and died. Conclusions This is the first report in the literature describing global vasospasm and delayed cerebral ischemia following Hemispherectomy in a patient carrying COL4A1 gene mutation. We postulate that his COL4A1 gene mutation might have resulted in this exaggerated vasospasm despite minimal residual postoperative subarachnoid hemorrhage burden. This hypothesis needs to be studied in animal models of this genetic disorder.

  • Hemispherectomy in adults and adolescents seizure and functional outcomes in 47 patients
    2019
    Co-Authors: Robert A Mcgovern, Ahsan N. V. Moosa, Lara Jehi, Elaine Wyllie, Ajay Gupta, Jorge Gonzalezmartinez, Robyn M Busch, Lisa Ferguson, Imad Najm, William Bingaman
    Abstract:

    Objective To examine longitudinal seizure and functional outcomes after Hemispherectomy in adults and adolescents. Methods We reviewed 47 consecutive patients older than 16 years who underwent Hemispherectomy between 1996 and 2016 at our center. Clinical, electroencephalographic (EEG), imaging, neuropsychological, surgical, and functional status data were analyzed. Results Thirty-six patients were 18 years or older at surgery; 11 were aged between 16 and 18 years. Brain injury leading to hemispheric epilepsy occurred before 10 years of age in 41 (87%) patients. At a mean follow-up of 5.3 postoperative years (median = 2.9 years), 36 (77%) had Engel class I outcome. Longitudinal outcome analysis showed 84% seizure freedom (Engel IA) at 6 months, 76% at 2 years, and 76% at 5 years and beyond, with stable longitudinal outcomes up to 12 years from surgery. Multivariate analysis demonstrated that acute postoperative seizures and contralateral interictal spikes at 6-month follow-up EEG were associated with seizure recurrence. Patients who could walk unaided preoperatively and had no cerebral peduncle atrophy on brain magnetic resonance imaging were more likely to experience worsening of motor function postoperatively. Otherwise, postoperative ambulatory status and hand function were unchanged. Of the 19 patients who completed neuropsychological testing, 17 demonstrated stable or improved postoperative outcomes. Significance Hemispherectomy in adults is a safe and effective procedure, with seizure freedom rates and functional outcome similar to those observed in children.

  • volumetric analysis of cerebral peduncles and cerebellar hemispheres for predicting hemiparesis after Hemispherectomy
    2016
    Co-Authors: Jeffrey P Mullin, Lara Jehi, William Bingaman, Pranay Soni, Sungho Lee, Ahsan Moosa Naduvil Valappi, Jorge Gonzalezmartinez
    Abstract:

    BACKGROUND In some cases of refractory epilepsy, Hemispherectomy is the final invasive treatment option. However, predictors of postoperative hemiparesis in these patients have not been widely studied. OBJECTIVE To investigate how the volumetric analysis of cerebral peduncles and cerebellar hemispheres in patients who have undergone Hemispherectomy may determine prognostic implications for postoperative hemiparesis. METHODS Twenty-two patients who underwent Hemispherectomy at our institution were retrospectively included. Using iPlan/BrainLAB (BrainLAB, Feldkirchen, Germany) imaging software and a semiautomatic voxel-based segmentation method, we calculated the preoperative cerebral peduncle and cerebellar hemisphere volumes. Cerebral peduncle and cerebellar hemisphere ratios were compared between patients with worsened or unchanged/better hemiparesis postoperatively. RESULTS The ratios of ipsilateral/contralateral cerebral peduncles (0.570 vs 0.828; P = .02) and contralateral/ipsilateral cerebellar hemispheres (0.885 vs 1.031; P = .009) were significantly lower in patients who had unchanged/improved hemiparesis postoperatively compared with patients who had worsened hemiparesis. Relative risk of worsening hemiparesis was significantly higher in patients with a cerebral peduncle ratio < 0.7 (relative risk, 4.3; P = .03) or a cerebellar ratio < 1.0 (relative risk, 6.4; P = .006). CONCLUSION Although patients who undergo Hemispherectomy are heterogeneous, we report a method of predicting postoperative hemiparesis using only standard volumetric magnetic resonance imaging. This information could be used in preoperative discussions with patients and families to help better understand that chance of retaining baseline motor function. ABBREVIATIONS CST, corticospinal tractfMRI, functional magnetic resonance imagingTMS, transcranial magnetic stimulation.

  • longitudinal seizure outcome and prognostic predictors after Hemispherectomy in 170 children
    2013
    Co-Authors: Ahsan N. V. Moosa, Ahmad Marashly, Gary Cosmo, Deepak Lachhwani, Lara Jehi, Elaine Wyllie, Prakash Kotagal, Ajay Gupta, William Bingaman
    Abstract:

    Objective: Data on longitudinal seizure outcome after Hemispherectomy in children are limited. This study explores the postHemispherectomy longitudinal seizure outcome and its predictors. Methods: We reviewed 186 consecutive children who underwent Hemispherectomy between 1997 and 2009 at our center. Clinical, EEG, imaging, and surgical data were collected. Seizure outcome data were collected via a structured questionnaire by contacting families (n = 125) or from the medical records at last follow-up (n = 58). Results: Of 186 patients, 3 were lost to follow-up; 13 seizure-free patients with new-onset nonepileptic spells were excluded. Perioperative complications were not collected. There was no mortality. At a mean follow-up of 5.3 years (±3.3 years), 112 of 170 children (66%) were seizure-free (Engel class 1a). In 58 patients with seizure recurrence, 8 had late remission and 16 had >90% reduction. Overall, at last follow-up, 136 patients (80%) were either seizure-free or had major improvement. Using survival analysis, the estimated probability of seizure freedom after Hemispherectomy was 78% (95% confidence interval [CI] = 75%–81%) at 6 months, 76% (95% CI = 73%–79%) at 1 year, 71% (95% CI = 68%–74%) at 2 years, and 63% (95% CI = 59%–67%) at 5 years. On multivariate analysis, bilateral PET abnormalities (risk ratio = 2.53, 95% CI = 1.02–5.85) and acute postoperative seizures (risk ratio = 7.03, 95% CI = 3.07–15.9) independently predicted seizure recurrence. Conclusions: The long-term seizure-free rates after Hemispherectomy remained stable at 63% at 5 years and beyond. This study will assist in better candidate selection for Hemispherectomy, presurgical counseling, and early identification of surgical failures.

  • Long-term functional outcomes and their predictors after Hemispherectomy in 115 children
    2013
    Co-Authors: Ahsan N. V. Moosa, Ahmad Marashly, Gary Cosmo, Deepak Lachhwani, Lara Jehi, Elaine Wyllie, Prakash Kotagal, William Bingaman
    Abstract:

    SUMMARY Purpose: To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had Hemispherectomy. Functional outcome measures studied were the following: ambula-tion ability, visual symptoms, spoken language, reading skills, and behavioral problems. Methods: We reviewed 186 consecutive children who underwent Hemispherectomy between 1997 and 2009 at our center. Preoperative clinical, electroencephalography (EEG), imaging, and surgical data were collected. One hundred twenty-five families completed a structured questionnaire to assess the functional status and seizure outcome. Prognostic predictors were examined using a multivariate regression analysis. Key Findings: At a mean follow-up of 6.05 years after Hemispherectomy, 70 patients (56%) were seizure-free and 45 (36%) had seizure recurrence; 10 patients (8%) were free of their preoperative seizures but had new-onset nonepileptic spells and were excluded from further analysis. Of 115, at follow-up (mean age at follow-up 12.7 years, range 2–28 years), 96 patients (83%) walked independently, 10 (8.7%) walked with assistance, and 9 (7.8%) were unable to walk. New visual symptoms that were not present preoperatively were reported only in 28 patients (24%). Eighty patients (70%) had satisfactory spoken language skills but only 44 (42%) of the 105 children older than 6 years had satis-factory reading skills. Significant behavioral problems were reported in 30 patients (27%). Only five (6.2%) of the 81 chil-dren aged between 6 and 18 years attended mainstream school without assistance; 48 (59%) were in mainstream school with assistance and the rest were in special school for disabled or home cared. Five (21%) of the 24 patients older than 18 years of age were gainfully employed. Multivariate logistic regression analysis identified the following factors as independently associated with poor functional outcome. (1) Seizure recurrence negatively affected all functional domains—ambulation ability, spoken language and reading skills, and behavior (p < 0.05). (2) Abnormalities in the unoperated hemisphere on magnetic resonance imaging (MRI) (p < 0.05) and preexisting quadriparesis (p < 0.01) correlated with poor motor outcome. (3) Multilobar MRI abnormalities in the contralateral hemisphere (odds ratio [OR] = 13.9, p = 0.001) and young age (indeterminate preoperative language status) at Hemispherectomy (OR = 11.1, p = 0.01) also correlated with poor language outcome. (4) Younger age at epilepsy onset correlated with poor reading skills (p = 0.01) but not with spoken language skills. Significance: This study highlights the long-term functional status of patients after Hemispherectomy. The majority of patients were ambulant independently; however, impairments in reading and spoken language were frequent. Seizure recurrence after Hemispherectomy and contralateral hemisphere abnormalities on MRI were the major predictors of poor outcome in ambulation, spoken language, and reading abilities. This study will assist in presurgical counseling using simple understandable functional outcome measures and may help in planning early interventions after Hemispherectomy to improve functional outcome.

Harry T Chugani - One of the best experts on this subject based on the ideXlab platform.

  • anatomical Hemispherectomy revisited outcome blood loss hydrocephalus and absence of chronic hemosiderosis
    2019
    Co-Authors: Sandeep Sood, Harry T Chugani, Mohammed Ilyas, Neena I Marupudi, Eishi Asano, Ashok Kumar, Aimee F Luat, Sheena Saleem
    Abstract:

    To evaluate microsurgical trans-sylvian trans-ventricular anatomical Hemispherectomy with regard to seizure outcome, risk of hydrocephalus, blood loss, and risk of chronic hemosiderosis in patients with intractable seizures selected for surgery using current preoperative assessment techniques. Out of 86 patients who underwent Hemispherectomy between February 2000 and April 2019, by a single surgeon, at a tertiary care referral center, 77 patients (ages 0.2–20 years; 40 females) who had an anatomical Hemispherectomy were analyzed. Five of these were ‘palliative’ surgeries. One-stage anatomical Hemispherectomy was performed in 55 children, two-stage anatomical Hemispherectomy after extraoperative intracranial monitoring in 16, and six hemispherectomies were done following failed previous resection. Mean follow-up duration was 5.7 years (range 1–16.84 years). Forty-six patients had postoperative MRI scans. Ninety percent of children with non-palliative Hemispherectomy achieved ILAE Class-1 outcome. Twenty-seven patients were no longer taking anticonvulsant medications. Surgical failures (n = 4) included one patient with previous meningoencephalitis, one with anti-GAD antibody encephalitis, one with idiopathic neonatal thalamic hemorrhage, and one with extensive tuberous sclerosis. There were no failures among patients with malformations of cortical development. Estimated average blood loss during surgery was 387 ml. Ten (21%) children developed hydrocephalus and required a shunt following one-stage Hemispherectomy, whereas 10 (50%) patients developed hydrocephalus among those who had extraoperative intracranial monitoring. Only 20% of the shunts malfunctioned in the first year. Early malfunctions were related to the valve and later to fracture disconnection of the shunt. One patent had a traumatic subdural hematoma. None of the patients developed clinical signs of chronic ‘superficial cerebral hemosiderosis’ nor was there evidence of radiologically persistent chronic hemosiderosis in patients who had postoperative MRI imaging. Surgical results of anatomical Hemispherectomy are excellent in carefully selected cases. Post-operative complications of hydrocephalus and intraoperative blood loss are comparable to those reported for hemispheric disconnective surgery (hemispherotomy). The rate of shunt malfunction was less than that reported for patients with hydrocephalus of other etiologies Absence of chronic ‘superficial hemosiderosis’, even on long-term follow-up, suggests that anatomical Hemispherectomy should be revisited as a viable option in patients with intractable seizures and altered anatomy such as in malformations of cortical development, a group that has a reported high rate of seizure recurrence related to incomplete disconnection following hemispheric disconnective surgery.

  • anatomical Hemispherectomy revisited outcome blood loss hydrocephalus and absence of chronic hemosiderosis
    2019
    Co-Authors: Sandeep Sood, Mohammed Ilyas, Neena I Marupudi, Eishi Asano, Aimee F Luat, Sheena Saleem, Ajay Kumar, Harry T Chugani
    Abstract:

    Purpose To evaluate microsurgical trans-sylvian trans-ventricular anatomical Hemispherectomy with regard to seizure outcome, risk of hydrocephalus, blood loss, and risk of chronic hemosiderosis in patients with intractable seizures selected for surgery using current preoperative assessment techniques.

  • role of external ventriculostomy in the management of fever after Hemispherectomy
    2008
    Co-Authors: Sandeep Sood, Eishi Asano, Harry T Chugani
    Abstract:

    Object Fever is a common occurrence after cerebral Hemispherectomy in children and prolongs the hospital stay. The authors determined whether an external ventriculostomy might reduce the incidence of fever following a Hemispherectomy. Methods The postoperative courses of 27 patients who had undergone cerebral Hemispherectomy for intractable seizures were retrospectively analyzed. Results Thirteen children underwent an external ventriculostomy, and only 1 had an elevated axillary body temperature of ≥ 39°C during the postoperative period. Among 14 patients who did not undergo an external ventriculostomy, 7 had a postHemispherectomy fever of ≥ 39°C. Patients who underwent an external ventriculostomy had a lower risk of postoperative fever compared with those who did not undergo the procedure (8 vs 50%, respectively; p = 0.03, Fisher exact test). None of the patients had an infection accounting for the cause of the fever. The hospital stay for patients who had undergone postoperative external ventriculostomy...

  • diffusion tensor imaging of the corticospinal tract following cerebral Hemispherectomy
    2006
    Co-Authors: Hiroyuki Wakamoto, Thomas J Eluvathingal, Malek I Makki, Csaba Juhasz, Harry T Chugani
    Abstract:

    Following cerebral Hemispherectomy, the corticospinal tract is believed to undergo reorganizational changes, which can induce enhanced function of the contralateral motor pathway and mediate partial recovery of motor function. The aim of this study was to use diffusion tensor imaging to investigate the effects of Hemispherectomy on the corticospinal tract, with particular attention to the corticospinal tract contralateral to the resection. Diffusion tensor imaging would presumably detect microstructural abnormalities through quantitative measurements of the fiber tract integrity and orientation. Four patients with anatomic Hemispherectomy and three patients with subtotal Hemispherectomy were examined and compared with age-matched normal controls. Apparent diffusion coefficient and fractional anisotropy values were measured in regions along the corticospinal tract: internal capsule, cerebral peduncle, rostral pons, midpons, and caudal pons. None of the patients with anatomic Hemispherectomy or subtotal Hemispherectomy showed significant changes in either apparent diffusion coefficient or fractional anisotropy values in the corticospinal tract contralateral to the resected hemisphere, whereas increased apparent diffusion coefficient and decreased fractional anisotropy were observed in the ipsilateral rostral pons, midpons, and caudal pons of all patients with anatomic Hemispherectomy, as well as in the ipsilateral cerebral peduncle of one patient with subtotal Hemispherectomy. Increased apparent diffusion coefficient values were also noted in the ipsilateral internal capsule of the same patient. This study revealed no evidence of significant reinforcement of the contralateral corticospinal tract in patients with Hemispherectomy, at least from diffusion tensor imaging measurements, but suggests that wallerian degeneration most likely occurs in the ipsilateral motor pathway.

  • pediatric rasmussen encephalitis social communication language pet and pathology before and after Hemispherectomy
    1996
    Co-Authors: Rochelle Caplan, Harry T Chugani, Susan Curtiss, Harry V Vinters
    Abstract:

    This prospective case study examined social communication (i.e., formal thought disorder, cohesion), language, positron emission tomography glucose utilization, and neuropathology in four children with Rasmussen encephalitis who achieved seizure control following right Hemispherectomy. Prior to Hemispherectomy, all four children had illogical thinking, loose associations, cohesive deficits, and impaired performance on formal language tests. Their postoperative improvement in social communication and language appeared to be related to age of onset, duration of illness, and postsurgical reversibility of hypometabolism in the nonresected prefrontal cortex. These changes were not associated with increase in IQ scores. The variability in the type and extent of pathologic change across subjects reflected the severity and duration of the illness. The study's findings imply that early surgical intervention might have mitigated certain aspects of the social communication and linguistic deficits found in these children.

Elaine Wyllie - One of the best experts on this subject based on the ideXlab platform.

  • Hemispherectomy in adults and adolescents seizure and functional outcomes in 47 patients
    2019
    Co-Authors: Robert A Mcgovern, Ahsan N. V. Moosa, Lara Jehi, Elaine Wyllie, Ajay Gupta, Jorge Gonzalezmartinez, Robyn M Busch, Lisa Ferguson, Imad Najm, William Bingaman
    Abstract:

    Objective To examine longitudinal seizure and functional outcomes after Hemispherectomy in adults and adolescents. Methods We reviewed 47 consecutive patients older than 16 years who underwent Hemispherectomy between 1996 and 2016 at our center. Clinical, electroencephalographic (EEG), imaging, neuropsychological, surgical, and functional status data were analyzed. Results Thirty-six patients were 18 years or older at surgery; 11 were aged between 16 and 18 years. Brain injury leading to hemispheric epilepsy occurred before 10 years of age in 41 (87%) patients. At a mean follow-up of 5.3 postoperative years (median = 2.9 years), 36 (77%) had Engel class I outcome. Longitudinal outcome analysis showed 84% seizure freedom (Engel IA) at 6 months, 76% at 2 years, and 76% at 5 years and beyond, with stable longitudinal outcomes up to 12 years from surgery. Multivariate analysis demonstrated that acute postoperative seizures and contralateral interictal spikes at 6-month follow-up EEG were associated with seizure recurrence. Patients who could walk unaided preoperatively and had no cerebral peduncle atrophy on brain magnetic resonance imaging were more likely to experience worsening of motor function postoperatively. Otherwise, postoperative ambulatory status and hand function were unchanged. Of the 19 patients who completed neuropsychological testing, 17 demonstrated stable or improved postoperative outcomes. Significance Hemispherectomy in adults is a safe and effective procedure, with seizure freedom rates and functional outcome similar to those observed in children.

  • longitudinal seizure outcome and prognostic predictors after Hemispherectomy in 170 children
    2013
    Co-Authors: Ahsan N. V. Moosa, Ahmad Marashly, Gary Cosmo, Deepak Lachhwani, Lara Jehi, Elaine Wyllie, Prakash Kotagal, Ajay Gupta, William Bingaman
    Abstract:

    Objective: Data on longitudinal seizure outcome after Hemispherectomy in children are limited. This study explores the postHemispherectomy longitudinal seizure outcome and its predictors. Methods: We reviewed 186 consecutive children who underwent Hemispherectomy between 1997 and 2009 at our center. Clinical, EEG, imaging, and surgical data were collected. Seizure outcome data were collected via a structured questionnaire by contacting families (n = 125) or from the medical records at last follow-up (n = 58). Results: Of 186 patients, 3 were lost to follow-up; 13 seizure-free patients with new-onset nonepileptic spells were excluded. Perioperative complications were not collected. There was no mortality. At a mean follow-up of 5.3 years (±3.3 years), 112 of 170 children (66%) were seizure-free (Engel class 1a). In 58 patients with seizure recurrence, 8 had late remission and 16 had >90% reduction. Overall, at last follow-up, 136 patients (80%) were either seizure-free or had major improvement. Using survival analysis, the estimated probability of seizure freedom after Hemispherectomy was 78% (95% confidence interval [CI] = 75%–81%) at 6 months, 76% (95% CI = 73%–79%) at 1 year, 71% (95% CI = 68%–74%) at 2 years, and 63% (95% CI = 59%–67%) at 5 years. On multivariate analysis, bilateral PET abnormalities (risk ratio = 2.53, 95% CI = 1.02–5.85) and acute postoperative seizures (risk ratio = 7.03, 95% CI = 3.07–15.9) independently predicted seizure recurrence. Conclusions: The long-term seizure-free rates after Hemispherectomy remained stable at 63% at 5 years and beyond. This study will assist in better candidate selection for Hemispherectomy, presurgical counseling, and early identification of surgical failures.

  • Long-term functional outcomes and their predictors after Hemispherectomy in 115 children
    2013
    Co-Authors: Ahsan N. V. Moosa, Ahmad Marashly, Gary Cosmo, Deepak Lachhwani, Lara Jehi, Elaine Wyllie, Prakash Kotagal, William Bingaman
    Abstract:

    SUMMARY Purpose: To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had Hemispherectomy. Functional outcome measures studied were the following: ambula-tion ability, visual symptoms, spoken language, reading skills, and behavioral problems. Methods: We reviewed 186 consecutive children who underwent Hemispherectomy between 1997 and 2009 at our center. Preoperative clinical, electroencephalography (EEG), imaging, and surgical data were collected. One hundred twenty-five families completed a structured questionnaire to assess the functional status and seizure outcome. Prognostic predictors were examined using a multivariate regression analysis. Key Findings: At a mean follow-up of 6.05 years after Hemispherectomy, 70 patients (56%) were seizure-free and 45 (36%) had seizure recurrence; 10 patients (8%) were free of their preoperative seizures but had new-onset nonepileptic spells and were excluded from further analysis. Of 115, at follow-up (mean age at follow-up 12.7 years, range 2–28 years), 96 patients (83%) walked independently, 10 (8.7%) walked with assistance, and 9 (7.8%) were unable to walk. New visual symptoms that were not present preoperatively were reported only in 28 patients (24%). Eighty patients (70%) had satisfactory spoken language skills but only 44 (42%) of the 105 children older than 6 years had satis-factory reading skills. Significant behavioral problems were reported in 30 patients (27%). Only five (6.2%) of the 81 chil-dren aged between 6 and 18 years attended mainstream school without assistance; 48 (59%) were in mainstream school with assistance and the rest were in special school for disabled or home cared. Five (21%) of the 24 patients older than 18 years of age were gainfully employed. Multivariate logistic regression analysis identified the following factors as independently associated with poor functional outcome. (1) Seizure recurrence negatively affected all functional domains—ambulation ability, spoken language and reading skills, and behavior (p < 0.05). (2) Abnormalities in the unoperated hemisphere on magnetic resonance imaging (MRI) (p < 0.05) and preexisting quadriparesis (p < 0.01) correlated with poor motor outcome. (3) Multilobar MRI abnormalities in the contralateral hemisphere (odds ratio [OR] = 13.9, p = 0.001) and young age (indeterminate preoperative language status) at Hemispherectomy (OR = 11.1, p = 0.01) also correlated with poor language outcome. (4) Younger age at epilepsy onset correlated with poor reading skills (p = 0.01) but not with spoken language skills. Significance: This study highlights the long-term functional status of patients after Hemispherectomy. The majority of patients were ambulant independently; however, impairments in reading and spoken language were frequent. Seizure recurrence after Hemispherectomy and contralateral hemisphere abnormalities on MRI were the major predictors of poor outcome in ambulation, spoken language, and reading abilities. This study will assist in presurgical counseling using simple understandable functional outcome measures and may help in planning early interventions after Hemispherectomy to improve functional outcome.

  • reoperative Hemispherectomy for intractable epilepsy a report of 36 patients
    2012
    Co-Authors: Sumeet Vadera, Deepak Lachhwani, Ahsan N. V. Moosa, Lara Jehi, Elaine Wyllie, Prakash Kotagal, Ajay Gupta, William Bingaman
    Abstract:

    BACKGROUND: In patients with medically intractable epilepsy and diffuse unilateral hemispheric disease, functional or disconnective Hemispherectomy is a widely accepted and successful treatment option. If recurrent seizures develop after disconnective Hemispherectomy, management options become more complex and include conversion to anatomic Hemispherectomy. OBJECTIVE: To present the outcomes of all patients undergoing reoperative Hemispherectomy in 1 institution by 1 surgeon since 1998. METHODS: The medical records, operative reports, and imaging studies for 36 patients undergoing reoperative Hemispherectomy for continuing medically intractable epilepsy from 1998 to 2011 at Cleveland Clinic were reviewed. Patient characteristics, cause of seizure, imaging findings, surgery-related complications, and long-term seizure outcomes were evaluated. RESULTS: Patients presented with a variety of seizure origins, including Rasmussen encephalitis, perinatal infarction, cortical dysplasia, and hemimegalencephaly. Overall, 19% of patients were seizure free after conversion to anatomic Hemispherectomy, and 45% reported a decrease in seizure frequency by ≥ 90%. An additional 36% reported no improvement. Generalized ictal electroencephalography tended to confer a poorer prognosis, as did cortical dysplasia as the underlying diagnosis. CONCLUSION: The possibility that residual epileptogenic tissue in the operated hemisphere remains connected should be considered after failed functional Hemispherectomy because our data suggest that improvement in seizure frequency is possible after reoperative Hemispherectomy, although the chance of obtaining seizure freedom is relatively low. The decision to proceed with reoperative Hemispherectomy should be made after proper discussion with the patient and family and informed consent is given.

  • contralateral mri abnormalities in candidates for Hemispherectomy for refractory epilepsy
    2010
    Co-Authors: Tove Hallbook, Deepak Lachhwani, Prakash Kotagal, William Bingaman, Paul Ruggieri, Chirla Adina, Ayaj Gupta, Elaine Wyllie
    Abstract:

    Summary Purpose:  To assess the impact of contralateral magnetic resonance imaging (MRI) findings on seizure outcome after Hemispherectomy for refractory epilepsy. Methods:  We retrospectively reviewed 110 children, 0.4–18 (median 5.9) years of age, who underwent Hemispherectomy for severe refractory epilepsy at Cleveland Clinic Children’s Hospital. In children with contralateral (as well as ipsilateral) MRI findings appreciated preoperatively, the decision to proceed to surgery was based on other features concordant with the side with the most severe MRI abnormality, including ipsilateral epileptiform discharges, lateralizing seizure semiology, and side of hemiparesis. Results:  We retrospectively observed contralateral MRI abnormalities (predominantly small hemisphere, white matter loss or abnormal signal, or sulcation abnormalities) in 81 patients (74%), including 31 of 43 (72%) with malformations of cortical development (MCD), 31 of 42 (73%) with perinatal injury from infarction or hypoxia, and 15 of 25 (60%) with Rasmussen’s encephalitis, Sturge-Weber syndrome, or posttraumatic encephalomalacia. Among 84 children (76%) with lesions that were congenital or acquired pre- or perinatally, 67 (83%) had contralateral MRI abnormalities (p = 0.02). Contralateral findings were subjectively judged to be mild or moderate in 70 (86%). At follow-up 12–84 (median 24) months after surgery, 79% of patients with contralateral MRI abnormalities were seizure-free compared to 83% of patients without contralateral MRI findings, with no differences based on etiology group or type or severity of contralateral MRI abnormality. Discussion:  MRI abnormalities, usually mild to moderate in severity, were seen in the contralateral hemisphere in the majority of children who underwent Hemispherectomy for refractory epilepsy due to various etiologies, especially those that were congenital or early acquired. The contralateral MRI findings, always much less prominent than those in the ipsilateral hemisphere, did not correlate with seizure outcome and may not contraindicate Hemispherectomy in otherwise favorable candidates.

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  • Intrinsic functional organization of putative language networks in the brain following left cerebral Hemispherectomy
    2017
    Co-Authors: Anna Ivanova, Eran Zaidel, Noriko Salamon, Susan Bookheimer, Lucina Q. Uddin, Stella De Bode
    Abstract:

    In rare cases of severe and intractable epilepsy, cerebral Hemispherectomy is performed to arrest seizure activity and improve quality of life. The remaining hemisphere is often capable of supporting many cognitive functions post-surgery, although the outcome depends on the underlying etiology, hemisphere removed, and age of resection. The mechanisms underlying this massive reorganization are at present unknown. Here we examined intrinsic functional connectivity of putative language brain networks in four children after left cerebral Hemispherectomy using resting-state functional magnetic resonance imaging (rsfMRI). We compared these functional systems to intrinsic language networks in 15 neurotypical controls using region-of-interest (ROI)-based functional connectivity analyses. In three out of four Hemispherectomy patients, the ROI placed in the right inferior gyrus revealed a functional network that strongly resembled the right-hemisphere intrinsic language network observed in controls. This network typically comprised inferior frontal gyrus, superior temporal sulcus, and premotor regions. Quantitative ROI-to-ROI analyses revealed that functional connectivity between major nodes of the language network was significantly altered in all 4 examined patients. Overall, our data demonstrate that the pattern of functional connectivity within language networks is at least partially preserved in the intact right hemisphere of patients who underwent left Hemispherectomy.

  • complex syntax in the isolated right hemisphere receptive grammatical abilities after cerebral Hemispherectomy
    2015
    Co-Authors: Stella De Bode, Gary W Mathern, Lieselotte Smets, Stanley Dubinsky
    Abstract:

    article i nfo Objectives: In this study, we explored the syntactic competence of the right hemisphere (RH) after left cerebral Hemispherectomy, on the premise that it (syntactic competence) is known to be one of the most strongly left- lateralized language functions. As basic syntactic development for individuals in this subject pool has already been extensively explored, we focused instead on the investigation of complex syntactic constructions that are normally acquired later in childhood, i.e., between 7 and 9 years of age. Methods: Grammatical competence in 10 participants who had undergone left cerebral Hemispherectomy was compared to that of a group of normally developing children, with the two groups matched by the size of their vocabulary. The two tests we used for this research were created by the 1st language acquisition linguists and weredesignedtotestsetsofconstructionscategorizedanddifferentiatedbytheorderinwhichtheyarenormally acquired and by the type of grammatical competence that they involve. Results: We found that both groups followed the same developmental sequence of syntactic development with five (50%) postsurgical participants (all with prenatal etiologies) reaching nearly mature command of sentence grammar. Seizures negatively impacted performance on all tests. Conclusions: The isolated RH has the potential to support the complex grammatical categories that emerge rela- tively late in the normal acquisition of English by native speakers. Successful performance may be related to the timing of the initial insult and seizure control following Hemispherectomy.

  • literacy after cerebral Hemispherectomy can the isolated right hemisphere read
    2015
    Co-Authors: Stella De Bode, Gary W Mathern, Marine Chanturidze, Stanley Dubinsky
    Abstract:

    Abstract Objectives Cerebral Hemispherectomy, a surgical procedure undergone to control intractable seizures, is becoming a standard procedure with more cases identified and treated early in life [33]. While the effect of the dominant hemisphere resection on spoken language has been extensively researched, little is known about reading abilities in individuals after left-sided resection. Left-lateralized phonological abilities are the key components of reading, i.e., grapheme–phoneme conversion skills [1]. These skills are critical for the acquisition of word-specific orthographic knowledge and have been shown to predict reading levels in average readers as well as in readers with mild cognitive disability [26]. Furthermore, impaired phonological processing has been implicated as the cognitive basis in struggling readers. Here, we explored the reading skills in participants who have undergone left cerebral Hemispherectomy. Methods Seven individuals who have undergone left cerebral Hemispherectomy to control intractable seizures associated with perinatal infarct have been recruited for this study. We examined if components of phonological processing that are shown to reliably separate average readers from struggling readers, i.e., phonological awareness, verbal memory, speed of retrieval, and size of vocabulary, show the same relationship to reading levels when they are mediated by the right hemisphere [2]. Results We found that about 60% of our group developed both word reading and paragraph reading in the average range. Phonological processing measured by both phonological awareness and nonword reading was unexpectedly spared in the majority of participants. Phonological awareness levels strongly correlated with word reading. Verbal memory, a component of phonological processing skills, together with receptive vocabulary size, positively correlated with reading levels similar to those reported in average readers. Receptive vocabulary, a bilateral function, was preserved to a certain degree similar to that of strongly left-lateralized phonological skills [3]. Later seizure onset was associated with better reading levels. Conclusions When cerebral Hemispherectomy is performed to control seizures associated with very early (in utero) insult, it has been found that the remaining right hemisphere is still able to support reading and phonological processing skills that are normally mediated by the left hemisphere. Our results also suggest the existence of variability in individuals after Hemispherectomy, even within groups having the same etiology and similar timing of insult.

  • cerebral Hemispherectomy sensory scores before and after intensive mobility training
    2012
    Co-Authors: Stella De Bode, Stacy L Fritz, Gary W Mathern
    Abstract:

    Purpose: It is unclear whether sensory modalities can be modified by rehabilitation and if sensory functions vary on the affected side many years after cerebral Hemispherectomy. This pilot, proof-of-concept study assessed light touch and proprioception before and after 10 days of intensive mobility training in individuals after Hemispherectomy. Methods: Light touch and proprioception of the upper and lower extremity was measured using the Fugl-Meyer sensory subtest on the paretic side in 18 individuals with Hemispherectomy before and after mobility training. Sensory scores and differences related to mobility training were compared with clinical variables. Results: Patients were 7.1 ± 5.7 years from time of surgery to sensory assessment and mobility training. Light touch scores were 81 ± 22% and proprioception values were 64 ± 23% of normal (p = 0.0022). Light touch did not correlate with proprioception scores, and differences comparing after with before mobility training did not correlate. In multivariate analysis, younger age at seizure onset correlated with better light touch scores, and older age at onset correlated with improvements in light touch scores with mobility training. By comparison, proprioception scores were better in individuals with perinatal infarcts compared with Rasmussen encephalitis and Sturge-Weber. Post-training, proprioception scores were better in Sturge-Weber cases. Conclusion: Light touch was less affected than proprioception on the paretic side after cerebral Hemispherectomy. Improvements with mobility training correlated with older age at seizure onset and etiology. These findings suggest that many years after epilepsy surgery sensory functions are not static supporting the notion of existing developmental neuroplasticity of the remaining cerebral cortex along with brain stem and spinal cord pathways.

  • locomotor training remodels fmri sensorimotor cortical activations in children after cerebral Hemispherectomy
    2007
    Co-Authors: Stella De Bode, Gary W Mathern, Susan Y Bookheimer, Bruce H Dobkin
    Abstract:

    Purpose. This study examined whether locomotor training, which included body weight—supported treadmill therapy, improved walking and induced cortical representational adaptations using functional magnetic resonance imaging in the remaining sensorimotor network after cerebral Hemispherectomy. Methods. Hemispherectomy patients (n = 12) underwent 2 weeks of gait training for at least 30 hours each. They were tested pre- and posttraining with the Fugl-Meyer Motor Assessment, unassisted single-limb stance time, and usual and fastest walking speeds. Three patients performed voluntary ankle movements as the functional magnetic resonance imaging activation task pre- and posttraining. Control subjects included 5 healthy children tested 2 weeks apart, 2 of whom trained on the treadmill, and 2 Hemispherectomy patients who received upper extremity rehabilitation and no gait therapy. Results. Although patients reported improvements with gait training, behavioral outcomes did not significantly change. Training was ass...