Surgical candidates in children with epileptic spasms can be selected without invasive monitoring: A report of 70 cases.

OBJECTIVE: Prior surgical series in children with drug-resistant epileptic spasms have reported use of intracranial EEG monitoring in up to two-third of patients. We report outcome after epilepsy surgery for drug-resistant epileptic spasms in a cohort of children without the use of intracranial EEG monitoring in any of the patients. METHODS: Medical records of all consecutive children aged 5 years or under who had epilepsy surgery for epileptic spasms at Cleveland Clinic between 2000 and 2018 were reviewed. Post-operative seizure outcome and predictors of prognosis of seizure outcome were analyzed. RESULTS: Seventy children with active epileptic spasms underwent surgical resections during the study period. Mean age at seizure onset was 6.8 (+9.31) months and median age at surgery was 18.5 months. An epileptogenic lesion was identified on brain MRI in all patients; 17 (24%) had bilateral abnormalities. Etiologies included malformations of cortical development (58%), perinatal infarct/encephalomalacia (39%), and tumor (3%). None of the patients had intracranial EEG. Surgical procedures included hemispherectomy (44%), lobectomy/ lesionectomy (33%), and multilobar resections (23%). Twelve children needed repeat surgery; six (50%) became seizure free after the second surgery. At six months follow-up, 73% (51/70) were seizure-free since surgery. At a mean follow-up of 4.7 years, 60% (42/70) had Engel 1 outcome. In those with seizure recurrence, 17 (60%) reported improvement. Shorter epilepsy duration (p = 0.05) and lobar or sub-lobar epileptogenic lesions (p = 0.02) predicted favorable seizure outcome at 6 months after surgery. For long term outcome, patients with bilateral abnormalities on MRI (p = 0.001), and multilobar extent on MRI (p = 0.02) were at higher risk for recurrence. SIGNIFICANCE: Children with drug-resistant epileptic spasms secondary to an epileptogenic lesion detected on MRI could be selected for epilepsy surgery without undergoing intracranial EEG monitoring. A surgical selection paradigm without intracranial monitoring may allow early surgery without the risks of invasive monitoring.

Perisylvian vulnerability to postencephalitic epilepsy.

OBJECTIVE: Postencephalitic epilepsy is often resistant to antiseizure medications, leading to evaluation for epilepsy surgery. Characterizing its localization carries implications for optimal surgical approach. We aimed to determine whether a prior history of encephalitis is associated with specific epileptogenic networks among patients with drug resistant epilepsy undergoing stereotactic EEG (SEEG). METHODS: We conducted a retrospective cohort study of drug resistant epilepsy, with and without a prior history of encephalitis. We analyzed SEEG recordings to identify patterns of seizure onset and organization. Seventeen patients with a history of encephalitis (of infectious etiology in two subjects) were identified from a database of patients undergoing SEEG and were compared to seventeen drug-resistant epilepsy controls without a history of encephalitis matched for confounding variables including pre-implantation hypotheses, epilepsy duration, age, and sex. RESULTS: Independent bilateral seizures were noted in 65% of the postencephalitic epilepsy cohort. We identified four SEEG-ictal patterns in patients with a prior history of encephalitis: (1) anteromesial temporal onset (24%), (2) anteromesial temporal onset with early spread to the perisylvian region (29%), (3) perisylvian (59%) and (4) synchronized anteromesial temporal and perisylvian (29%) onsets. Patterns 3 and 4, with perisylvian involvement at onset, were unique to the encephalitis group (p = 0.0003 and 0.04 respectively) and exhibited a "patchwork" organization. None of the encephalitis patients vs 5/7 matched controls had Engel I outcome (p = 0.0048). CONCLUSIONS: Postencephalitic epilepsies involve anteromesial temporal and perisylvian networks, often in a bilateral independent manner. Unique ictal patterns involving the perisylvian regions was identified in the encephalitis group, but not in the matched control group. SIGNIFICANCE: These findings may reflect a selective vulnerability of the perisylvian regions to epilepsy resulting from encephalitis, significantly mitigating the chances of success with SEEG-guided temporal resections.

Vasospasm Following Hemispherectomy: A Case Report of a Novel Complication.

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.

Antiepileptic Drug Treatment of Epilepsy in Children.

PURPOSE OF REVIEW: The treatment of epilepsy in children is highly individualized at each and every major step in the management. This review examines various factors that modify the treatment from the point of initiation of therapy to the decision to stop an antiepileptic drug (AED). RECENT FINDINGS: AED therapy leads to seizure freedom in about 70% of all children with epilepsy. AED initiation could be delayed until a second seizure in most children and may be avoided altogether in many children with self-limited childhood focal epilepsies. Three key factors influence the choice of AED: seizure type(s), efficacy of the drug for the seizure type, and the side effect profile of the drug(s). For epileptic spasms, steroids and vigabatrin are the most effective treatment options. For absence seizures, ethosuximide and valproic acid are superior to lamotrigine. For focal seizures, many newer AEDs have favorable side effect profiles with efficacy comparable to older-generation drugs. For generalized epilepsies, valproic acid remains the most effective drug for a broad range of seizure types. Genetic and metabolic etiologies may guide unique treatment choices in some children. After 2 years or more of seizure freedom, if the recurrence risk after AED withdrawal is acceptable, slow weaning of AEDs should be done over the span of 6 weeks or longer. After discontinuation, about 70% of patients remain seizure free, and of those with recurrence, the majority achieve seizure control with restarting an AED. When treatment with two or more AEDs fails, other treatment opportunities for drug-resistant epilepsy, including epilepsy surgery, vagal nerve stimulation, and dietary therapies should be considered. SUMMARY: Carefully selected medical therapy guided by seizure type and AED characteristics is effective in more than two-thirds of children with epilepsy.

Robot-assisted stereoelectroencephalography in children.

OBJECTIVEThe goal in the study was to describe the clinical outcomes associated with robot-assisted stereoelectroencephalography (SEEG) in children.METHODSThe authors performed a retrospective, single-center study in consecutive children with medically refractory epilepsy who were undergoing robot-assisted SEEG. Kaplan-Meier survival analysis was used to calculate the probability of seizure freedom. Both univariate and multivariate methods were used to analyze the preoperative and operative factors associated with seizure freedom.RESULTSFifty-seven children underwent a total of 64 robot-assisted procedures. The patients' mean age was 12 years, an average of 6.4 antiepileptic drugs (AEDs) per patient had failed prior to implantation, and in 56% of the patients the disease was considered nonlesional. On average, children had 12.4 electrodes placed per implantation, with an implantation time of 9.6 minutes per electrode and a 10-day postoperative stay. SEEG analysis yielded a definable epileptogenic zone in 51 (89%) patients; 42 (74%) patients underwent surgery, half of whom were seizure free at last follow-up, 19.6 months from resection. In a multivariate generalized linear model, resective surgery, older age, and shorter SEEG-related hospital length of stay were associated with seizure freedom. In a Cox proportional hazards model including only the children who underwent resective surgery, older age was the only significant factor associated with seizure freedom. Complications related to bleeding were the major contributors to morbidity. One patient (1.5%) had a symptomatic hemorrhage resulting in a permanent neurological deficit.CONCLUSIONSThe authors report one of the largest pediatric-specific SEEG series demonstrating that the modern surgical management of medically refractory epilepsy in children can lead to seizure freedom in many patients, while also highlighting the challenges posed by this difficult patient population.

Electroclinical features of seizures associated with autoimmune encephalitis.

PURPOSE: We sought to characterize the electroclinical features of seizures associated with autoimmune encephalitis and their relevance to outcome. METHODS: 19 patients with seizures and autoimmune encephalitis were identified from a database of 100 patients (2008-2017) with autoimmune neurological disorders. Clinical and electroclinical characteristics were collected. Persistent seizures at last follow-up were then correlated with electroclinical features. RESULTS: Status epilepticus (53%) and early intractability to AEDs (median time to second AED 9.5 days) marked the onset of refractory seizures (median number of AEDs 3). Seizure semiology (abdominal (16%), psychic (42%), olfactory (6%) auras), interictal temporal epileptiform discharges (42%), and ictal onset in the temporal region (63%) mirrored radiologic involvement of the medial temporal regions (on MRI in 74% and/or FDG-PET in 75%). In addition, multimodal auras, with somatosensory (26%), autonomic (26%), gustatory (11%), and visual (16%), features were seen in 82% of patients with focal aware seizures, invoking broader involvement of the perisylvian regions. A change in seizure semiology and EEG findings was often seen. Electroclinical features were similar regardless of antibody type, with the exception of the association of faciobrachial dystonic seizures with LGI1 antibodies. Eight patients had medically intractable seizures at last follow-up and were more likely than patients with seizure remission to have generalized tonic-clonic seizures and temporal lobe involvement on the basis of semiological features, interictal EEG and MRI changes. CONCLUSIONS: Seizures associated with autoimmune encephalitis exhibit common electroclinical features which show dynamic evolution over time. We propose a role for the temporo-perisylvian regions in their generation.

Quantitative positron emission tomography-guided magnetic resonance imaging postprocessing in magnetic resonance imaging-negative epilepsies.

OBJECTIVE: Detection of focal cortical dysplasia (FCD) is of paramount importance in epilepsy presurgical evaluation. Our study aims at utilizing quantitative positron emission tomography (QPET) analysis to complement magnetic resonance imaging (MRI) postprocessing by a morphometric analysis program (MAP) to facilitate automated identification of subtle FCD. METHODS: We retrospectively included a consecutive cohort of surgical patients who had a negative preoperative MRI by radiology report. MAP was performed on T1-weighted volumetric sequence and QPET was performed on PET/computed tomographic data, both with comparison to scanner-specific normal databases. Concordance between MAP and QPET was assessed at a lobar level, and the significance of concordant QPET-MAP+ abnormalities was confirmed by postresective seizure outcome and histopathology. QPET thresholds of standard deviations (SDs) of -1, -2, -3, and -4 were evaluated to identify the optimal threshold for QPET-MAP analysis. RESULTS: A total of 104 patients were included. When QPET thresholds of SD = -1, -2, and -3 were used, complete resection of the QPET-MAP+ region was significantly associated with seizure-free outcome when compared with the partial resection group (P = 0.023, P < 0.001, P = 0.006) or the no resection group (P = 0.002, P < 0.001, P = 0.001). The SD threshold of -2 showed the best combination of positive rate (55%), sensitivity (0.68), specificity (0.88), positive predictive value (0.88), and negative predictive value (0.69). Surgical pathology of the resected QPET-MAP+ areas revealed mainly FCD type I. Multiple QPET-MAP+ regions were present in 12% of the patients at SD = -2. SIGNIFICANCE: Our study demonstrates a practical and effective approach to combine quantitative analyses of functional (QPET) and structural (MAP) imaging data to improve identification of subtle epileptic abnormalities. This approach can be readily adopted by epilepsy centers to improve postresective seizure outcomes for patients without apparent lesions on MRI.

Extreme delta - With or without brushes: A potential surrogate marker of disease activity in anti-NMDA-receptor encephalitis.

OBJECTIVE: Anti-NMDA receptor encephalitis (NMDARE) may not respond to first line immunotherapy. Biomarkers to track disease course and guide escalation of immunotherapy are needed. We describe the evolution of EEG in four patients with NMDARE requiring prolonged intensive care. METHODS: Within a database of 121 patients with immune-mediated neurological disorders, ten with NMDARE were retrospectively identified. Four patients did not respond to first line immunotherapy. Continuous EEG was reviewed and correlated with clinical status and treatment. RESULTS: Intermittent polymorphic delta slowing was present in all patients. Generalized rhythmic delta occupied increasing proportion of the EEG as disease progressed, at times with superimposed beta. The institution of second line immunotherapy was followed by progressive decrease in rhythmic delta, predating clinical improvement. In one patient who did not respond to second line immunotherapy, rhythmic delta continued to occupy a majority of the recording. The extreme delta pattern was not seen in a comparison cohort of patients with autoimmune encephalitis without anti-NMDA-R antibodies. CONCLUSIONS: Extreme delta, with or without brushes, increases with progression of NMDARE, responds to escalation of immunotherapy, predating clinical improvement, and is likely specific to NMDA-R antibodies. SIGNIFICANCE: Extreme delta may be a surrogate marker of disease activity in NMDARE refractory to first line immunotherapy.

Cognitive Outcome After Epilepsy Surgery in Children.

Cognitive dysfunction in children with epilepsy is primarily contributed by etiology, seizures, frequency of interictal epileptiform discharges, and adverse effects of antiepileptic drugs. The direct effect of epilepsy surgery on cognitive outcome depends on two key factors: the function that is present in the epileptogenic zone to be removed, and the dysfunction outside the epileptogenic zone caused by epilepsy. Studies on cognitive outcome in children after various types of epilepsy surgery estimate "no significant change" in about 70% of children, improvement in cognition in 10%-15%, and decline in 10%-15%. In young children with epileptic encephalopathy, the reversible dysfunction outside the epileptogenic zone is larger and hence carry better chances of improved outcome after successful surgery. If the epileptogenic zone harbors significant cognitive function (memory, language, or other function), then a decline in function may occur with its resection. Understanding the pathophysiological basis for the cognitive changes after epilepsy surgery assists in counseling patients and families before surgery.

Does facial attractiveness influence perception of epilepsy diagnosis? An insight into stigma in epilepsy.

BACKGROUND: Using a group of young healthy individuals and patients with multiple sclerosis (pMS), we aimed to investigate whether the physical attractiveness judgment affects perception of epilepsy. We tested hypothesis that subjects, in the absence of relevant clues, would catch upon the facial attractiveness when asked to speculate which person suffers epilepsy and select less attractive choices. METHOD: Two photo-arrays (7 photos for each gender) selected from the Chicago Face Database (180 neutral faces of Caucasian volunteers with unknown medical status) were shown to study participants. Photos were evenly distributed along a continuum of attractiveness that was estimated by independent raters in prestudy stage. In each photo-array, three photos had rating 1-3 (unattractive), one photo had rating 4 (neutral), and three photos had rating 5-7 (attractive). High-quality printed photo-arrays were presented to test subjects, and they were asked to select one person from each photo-array "who has epilepsy". Finally, all subjects were asked to complete questionnaire of self-esteem and 19-item Scale of stereotypes toward people with epilepsy. RESULTS: In total, 71 students of psychology, anthropology, or andragogy (mean age: 21.6±1.7years; female: 85.9%) and 70 pMS (mean age: 37.9±8years; female: 71.4%) were tested. Majority of students or pMS had no previous personal experience with individuals with epilepsy (63.4%; 47.1%, p=0.052). Male photo was selected as epileptic in the following proportions: students - 84.5% unattractive, 8.5% neutral, and 7% attractive; pMS - 62.9% unattractive, 8.6% neutral, and 28.6% attractive (p=0.003). Female photo was selected as epileptic in the following proportions: students - 38% unattractive, 52.1% neutral, and 9.9% attractive; pMS - 32.9% unattractive, 34.3% neutral, and 32.9% attractive (0.003). Both groups showed very low potential for stigmatization: significantly lower in pMS in 10 items. Patients with multiple sclerosis showed significantly higher self-esteem than students (p=0.007). CONCLUSION: Facial attractiveness influences the perception of diagnosis of epilepsy. Both students and pMS were less willing to attribute epilepsy to attractive person of both genders.

Occipito-Frontal Sharp Waves-An Under-Recognized Electroencephalogram Pattern in Self-Limited Idiopathic Childhood Focal Epilepsy.

We report four children who highlight the potentially under-recognized electroencephalogram pattern of bilaterally synchronous occipito-frontal sharp waves, which may occur in children with Panayiotopoulos type self-limited childhood epilepsy but may be easily confused with the patterns of symptomatic generalized epilepsy. Our patients were young, healthy children who had infrequent, predominantly nocturnal, fairly prolonged seizures characterized by altered consciousness, vomiting, and autonomic features, or in one case nocturnal secondary generalized tonic-clonic convulsion. Their electroencephalograms showed stereotyped abundant sleep-activated sharp waves with maximum negativity in bilateral occipital and frontal greater than centro-temporo-parietal regions. On a "double-banana" bipolar montage, the sharp waves had upward deflections in frontal electrodes, downward deflections at occipital electrodes, and a positive phase reversal in the middle of each chain. The lower-amplitude occipital discharges slightly preceded the frontal discharges, consistent with posterior-to-anterior propagation likely originating from mesial occipital regions. In the proper clinical context, recognizing the electroencephalogram pattern of bilateral occipito-frontal sharp waves affords confidence in a favorable prognosis, presents the option to possibly defer daily treatment with antiepileptic medication, and shifts the emphasis to rescue medication for isolated seizures.

Re-review of MRI with post-processing in nonlesional patients in whom epilepsy surgery has failed.

Management of MRI-negative patients with intractable focal epilepsy after failed surgery is particularly challenging. In this study, we aim to investigate whether MRI post-processing could identify relevant targets for the re-evaluation of MRI-negative patients who failed the initial resective surgery. We examined a consecutive series of 56 MRI-negative patients who underwent resective surgery and had recurring seizures at 1-year follow-up. T1-weighted volumetric sequence from the pre-surgical MRI was used for voxel-based MRI post-processing which was implemented in a morphometric analysis program (MAP). MAP was positive in 15 of the 56 patients included in this study. In 5 patients, the MAP+ regions were fully resected. In 10 patients, the MAP+ regions were not or partially resected: two out of the 10 patients had a second surgery including the unresected MAP+ region, and both became seizure-free; the remaining 8 patients did not undergo further surgery, but the unresected MAP+ regions were concordant with more than one noninvasive modality in 7. In the 8 patients who had unresected MAP+ regions and intracranial-EEG before the previous surgery, the unresected MAP+ regions were concordant with ictal onset in 6. Our data suggest that scrutiny of the presurgical MRI guided by MRI post-processing may reveal relevant targets for reoperation in nonlesional epilepsies. MAP findings, when concordant with the patient's other noninvasive data, should be considered when planning invasive evaluation/reoperation for this most challenging group of patients.

Outcome after epilepsy surgery for cortical dysplasia in children.

BACKGROUND AND PURPOSE: Epilepsy surgery for medically refractory epilepsy secondary to cortical dysplasia in children poses special challenges. We aim to review the current available literature on the outcome after epilepsy surgery for cortical dysplasia in children and critically evaluate the prognostic predictors of outcome. METHODS: A comprehensive review of the literature was performed focusing on the outcome after epilepsy surgery for cortical dysplasia in children. Two large recent meta-analyses that included children and adults and several pediatric series of cortical dysplasia in children were reviewed. RESULTS AND CONCLUSIONS: The overall seizure freedom rates range from 40 to 73 %, at about 2 years after surgery; most studies report 50-55% success rate. Complete resection of the epileptogenic lesion/zone remains the most important variable predictive of postoperative seizure freedom. Features unique to cortical dysplasia that limits our ability to ensure complete resection of the epileptogenic zone are reviewed.

Long-term functional outcomes and their predictors after hemispherectomy in 115 children.

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: ambulation 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 satisfactory reading skills. Significant behavioral problems were reported in 30 patients (27%). Only five (6.2%) of the 81 children 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.

Focal epileptogenic lesions.

The spectrum of focal epileptogenic lesions and their clinical manifestations in children differ substantially from those seen in adults. In adults, mesial temporal sclerosis is the common lesion in surgical series; but in children, MTS is uncommon, and when it does occur, it exists frequently as dual pathology. The most common lesions in pediatric epilepsy surgery candidates are malformations of cortical development, developmental tumors, or encephalomalacia from infarction, hypoxia, trauma, or infection. Careful analysis of the lesion characteristics on brain MRI is sufficiently predictive of pathology in most cases. Histopathological evaluation remains the gold standard for diagnosis of mass lesions. The electroclinical phenotype of epilepsy in adults is largely determined by the anatomical location of the lesion and its connectivity. In children, in addition to the location of the lesion, the age at onset of the lesion and the age at onset of epilepsy have a major impact on the electroclinical phenotype. Children with congenital or early acquired lesions may manifest with generalized features on EEG and seizure semiology. Experience from various centers has demonstrated that a subset of these children benefit from epilepsy surgery despite a generalized epilepsy phenotype. All children with medically refractory epilepsy and a focal lesion should undergo evaluation for potential epilepsy surgery irrespective of the EEG findings and seizure semiology.

Neonatal stroke and progressive leukoencephalopathy in a child with an ACTA2 mutation.

Mutations in the smooth muscle-specific isoform of α-actin (ACTA2) cause vascular smooth muscle dysfunction leading to aortic aneurysm and moyamoya syndrome. A unique R179H mutation in ACTA2 has been reported to result in widespread smooth muscle dysfunction affecting vascular and extravascular smooth muscles. We report a 7-year-old girl with an ACTA2 R179H mutation manifesting with neonatal seizures due to multifocal infarcts, asymmetric motor deficits, global developmental delay, spasticity, congenital bilateral mydriasis, and a large patent ductus arteriosus. Serial magnetic resonance imaging (MRI) of the brain over 7 years showed diffuse supratentorial white matter abnormalities consistent with a progressive leukoencephalopathy. Magnetic resonance angiography of the cerebral vessels showed stenosis in the terminal portion of the bilateral internal carotid arteries with fusiform dilation of the proximal segment. Neonatal onset of neurologic symptoms in ACTA2 mutations has not been previously reported. R179H mutation in ACTA2 represents the severe end of the disease spectrum.

Longitudinal seizure outcome and prognostic predictors after hemispherectomy in 170 children.

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.