Predicting seizure outcomes and functional outcomes after hemispherotomy: are we any better?

INTRODUCTION: Present study attempted to analyze seizure freedom and detailed functional outcomes after functional hemispherotomy and utility of hemispherotomy outcome prediction scale (HOPS) scores in predicting outcomes. METHODS: Patients who underwent functional hemispherotomy were analyzed for clinical presentation, neuroimaging, seizure outcomes, and functional outcomes. RESULTS: A total of 76 procedures were performed on 69 patients. Mean age at the surgery was 8 ± 6.1 years. Fourteen patients were < 2 years. Age of onset epilepsy of the cohort was 2.0 ± 3.3 years. All had severe catastrophic epilepsy with multiple daily seizures. All patients had motor deficits with 36 (52%) patients had contralateral dysfunctional hand. Perinatal stroke (49%) was most common substrate followed by cortical malformations (21.7%). Eight patients had contralateral imaging abnormalities. Fifty-nine (86.76%) patients remained seizure free (Engle 1a) at 41 + -20.9 months. HOPS scores were available for 53 patients and lowest seizure outcome was 71% for HOPS score of 4. Lower HOPS scores predicted better seizure outcomes. Cortical malformations operated earlier than 2 years predicted poor seizure outcomes (66.6%). Positive functional outcomes are recorded in 80% of patients with 78% reporting improvement from the pre-surgical level. Five (7.2%) patients underwent shunt surgery. One mortality recorded. CONCLUSIONS: Hemispherotomy has excellent seizure outcomes. Early surgery in cortical malformations appears to be predictor of poorer seizure outcomes. HOPS score is a good tool to predict the seizure outcomes. Hemispherotomy is perceived to improve the Cognitive and functional performance.

Temporal encephalocele: a rare but treatable cause of temporal lobe epilepsy

Objective: Although rare, temporal encephalocele is an important causative agent in surgically remediable drug-refractory epilepsy. The ideal treatment for temporal encephalocele remains unclear with a variety of resective surgeries recommended. Here, we analyse patient data on temporal encephalocele with a view to highlighting diagnostic clues and management strategies. Methods: Comprehensive databases at Deenanath Mangeshkar Hospital, Pune from January 2015 to June 2019 were reviewed for this observational study. Of 107 temporal lobe epilepsy surgery patients, nine individuals with temporal encephalocele were identified, who formed the study cohort. Their clinical, neuropsychological, EEG, imaging and long-term outcome data were analysed. Results: The study cohort consisted of seven males and two females with a mean age of 22 years. Epilepsy onset age varied from 4.5 to 19 years. Seven patients had focal non-motor seizures with impaired awareness, while two patients had focal motor seizures. Temporal encephalocele detection by MRI was reported in only two patients, and was missed in seven individuals. Three patients underwent standard anterior temporal lobectomy while the remaining six underwent resection of the temporal encephalocele with surrounding temporal pole. Eight patients showed Engel Class I outcome and one showed Class IIa outcome after a mean follow-up duration of 27 months (17-44 months). Histopathology confirmed gliosis in seven, hippocampal sclerosis type I in one and suspicious dyslamination with prominent gliosis in one patient. Six of eight patients reported an improvement in their psychological state (mood, anxiety and motivation) over time. Significance: A careful review of MRI in patients with temporal lobe epilepsy is necessary, followed by investigations for the presence of an encephalocele. When temporal lobe epilepsy is associated with encephalocele, tailored resection of the encephalocele and the surrounding temporal pole, sparing mesial temporal structures, demonstrates excellent long-term clinical and neuropsychological outcome.

Clinical profile and outcomes of epilepsy surgery in children from a tertiary epilepsy care center in India.

INTRODUCTION: The present study aims to describe epilepsy surgery outcomes in the pediatric population from a tertiary center in India. METHODOLOGY: Children less than 18 years who underwent epilepsy surgery between June 2015 and December 2019 for whom at least a 1-year follow-up was available, were retrospectively evaluated for clinical presentation, radiology, surgical intervention, and seizure outcomes. OBSERVATIONS: Out of a total of 355 epilepsy surgeries performed, 242 were in the pediatric group (140 males, 80 females). The mean age at surgery was 9.4 years ±4.8 years (range 4 months-18 years). The mean duration of epilepsy was 5.64 years ±3.91 (range 2 months-17 years). 126 patients experienced daily seizures, 45 weekly and 39 reported monthly seizures. Six had refractory status epilepticus. All the patients were on multiple anti-epileptic drugs (AEDs): the mean number of AEDs was 3.27 ± 0.98 (range 2-7 AEDs). Focal seizure was the most common seizure type seen in 72.27% of children (159/220). The most frequent etiology was focal cortical dysplasia (70), followed by bilateral parieto-temporo-occipital gliosis (48). All the patients underwent standard pre-surgical evaluation. Eleven patients needed stage 2 evaluation (intracranial EEG). The different surgeries performed were electrocorticography (ECOG) and navigation-guided resection (65), anterior temporal lobectomy and hippocampectomy (ATLAH) (48), functional hemispherotomy (39), callosotomy (28), disconnection surgeries (16), and multilobar resection (12). Twelve patients underwent more than one surgery. The patients were followed up between a minimum of 12 months and a maximum of 66 months (median 32 months; IQR 20 months). Engel class 1 outcome was observed in 81.38% in definitive surgeries. Outcomes of temporal lobe (TL) surgeries (92.3%) were better compared to hemispheric (87.17%) and extratemporal lobe (ETL) surgeries (75.32%). We encountered unexpected transient motor deficits in 2 patients and culture-proven meningitis in 8 patients. Post-surgery drug freedom (P-value 0.003) was the most important factor for better developmental, cognitive and behavioral outcomes. CONCLUSION: Epilepsy surgeries are safe and seizure outcomes are excellent in properly selected cases with thorough presurgical evaluation. Early referral to a tertiary epilepsy center is needed for timely intervention.

Role of epilepsy surgery in refractory status epilepticus in children.

INTRODUCTION: Status epilepticus (SE) is one of the most common medical emergencies, requiring urgent treatment; nearly 30 % patients develop refractory SE. The role of epilepsy surgery (ES) for refractory SE however remains unclear with empirical evidence limited to single case reports and small case series. The aim of the present study was to determine the clinical presentation, imaging characteristics and outcome of children with refractory SE who underwent emergency ES for refractory SE. MATERIAL AND METHOD: Patients who had SE, failed to respond to escalating medical treatment of SE with/ without pharmacological suppression therapy, and eventually underwent ES were included. RESULTS: There were ten children, 7 boys and 3 girls (range 6 months to 14 years). The age of onset of epilepsy varied from day 2 of life to 12.8 years. The duration of SE prior to surgery was 2-6 days (mean 3.7 days). Four patients had hemimegalencephaly, 3 had focal cortical dysplasia, 2 had Rasmussen's encephalitis, and one had hemispheric porencephalic cyst. The time interval between onset of seizures and ES ranged from 2 months to 8 years (mean 3.1 year). Seven patients underwent hemispherotomy, resection of dysplasia in two and temporo-parieto-occipital disconnection in one. Nine had Engel I outcome and Engel IIIa in one, at follow up of 12-44 months (mean 31 months). CONCLUSION: Emergency ES is an effective treatment option for termination of refractory SE in children with structural pathology, after failure of medical treatment. Patients with refractory SE with focal or hemispheric structural abnormality on MRI, and concordant semiology with/without concordant EEG can be surgical candidates with or without invasive monitoring.

Surgical outcomes for medically refractory epilepsy secondary to posterior cortex ulegyria as sequelae of perinatal insults.

BACKGROUND: To study surgical outcomes in pharmaco-resistant epilepsy associated with posterior cortex ulegyria secondary to perinatal insults. METHODS: A cohort was analysed for clinico-radiological charectaristics, surgical interventions and seizure outcomes. OBSERVATIONS: A total of 38 patients underwent surgery, divided as group A - curative surgeries (n = 20) and group B - palliative surgeries (n = 18). Mean age of onset of epilepsy in group A was 5.2 ± 3.4 years against 2.7 ± 2.4 years in group B (p < 0.01). Electroclinical Lennox Gastaut Syndrome was encountered in 9/20 patients in group A, against all 18 patients in group B. Disabling reflex epilepsy was seen in 10 (26 %) patients. Interictal electrophysiology localized in the posterior cortex in all patients in group A, but ictal onsets contributed in only 7/20 patients. Nine patients from group A had unilateral parieto-occipital ulegyria while bilateral in 11/20 patients, and 16/18 from group B. Group A patients underwent parieto-occipital resection (n = 10) and temporo-parieto-occipital disconnection (n = 10) while group B underwent complete corpus callosotomy (n = 18). In group A, Engel Ia outcome was achieved in 15/20 patients (75 %) at mean follow up of 23.5 ± 7.9 months. Group B patients experienced cessation of head drops in all 18 patients, with two-third reduction in seizure frequency at 29.2± 12.4 months of mean follow up. Reflex seizures responded completely in both groups. CONCLUSIONS: Epilepsy surgeries for posterior cortex ulegyria results in excellent seizure outcomes. Corpus callosotomy appears highly effective as a palliation for head drop as well as disabling reflex seizures in a well selected cohort.

A review of caregiver distress in epilepsy in India: Current issues and future directions for research.

India is home to a large number of individuals with epilepsy, with many of these patients having high care needs. Owing to limited infrastructural support and prevalent cultural attitudes, most of these individuals are cared for by their families. Such informal caregiving is often associated with poor physical and psychological outcomes, but the state of caregivers of people with epilepsy in India remains largely ignored. This review summarizes currently available research on distress among caregivers for people with epilepsy in India. A review of 20 studies published till July 2020 revealed significant burden in this population. Caregiving was reported to negatively impact one's physical and mental health, overall family functioning, and financial status. A range of seizure-related variables were found to be associated with greater perceived burden, with the impact of patient characteristics and psychosocial factors on burden being relatively unexplored. Much of the research identified was characterized by methodological limitations such as small sample sizes, exclusion of patients with comorbidities, and a failure to distinguish between carers of adult and pediatric populations, thus overshadowing the specific needs of each group. There is a need for larger, well-designed studies that focus on culture-specific psychological and social factors in the Indian context of caregiving for people with epilepsy.

Failed Hemispherotomy: Insights from Our Early Experience in 40 Patients.

OBJECTIVE: To study the factors responsible for failure of hemispherotomy and outcomes of revision surgery. The effect of the surgeon's learning curve on failures was also analyzed. METHODS: Forty consecutive patients, who underwent functional hemispherotomy through a 4-year period, from the inception of the single-surgeon epilepsy surgery program, were analyzed. RESULTS: A total of 47 functional hemispherotomies were performed in the study period in 40 patients (7 revision surgeries on 6 patients). Mean age of the cohort was 9.45 ± 14.84 years and it included 7 infants (<2 years). Of the 9 patients (23.5%) who failed the first procedure, 6 qualified for revision surgery, all of whom belonged to the cohort of the first 15 patients treated during the first 2 years of the program. Hemimegalencephaly was the most common disease (n = 4). Ipsilateral temporal stem (n = 3), frontobasal connections (n = 2), splenium of corpus callosum (n = 2), and posterior insula (n = 2) were residual undisconnected substrates identified for revision on imaging. The substrates for failure were obvious in 5/6 patients and resulted from incomplete disconnection, implying surgical inadequacy. At the mean follow-up of 30 ± 13.17 months (range, 13-55 months), 35 of 40 patients (87.5%) remained seizure free (Engel class Ia), including 4/6 patients who underwent redo surgery. Revision did not benefit the remaining 2 patients (Engel class III). There was no mortality. CONCLUSIONS: Surgical revision is more common in hemimegalencephaly and in the early days of a surgical program. Affirmative neuroimaging improves the outcomes of subsequent revision surgery.

Long-Term Outcomes in Children Diagnosed with Autism Spectrum Disorders in India.

We investigated long-term outcomes in children with diagnosis of autism spectrum disorders based on Childhood Autism Rating Scale (CARS score). Information about outcomes such as speech, friendships and activities of daily living (ADLs) was collected through telephone-based interviews. Gilliam Autism Rating Scale-2 and Vineland Social Maturity Scale were used to assess level of functioning at follow-up. Parents of 80 [67 males, mean age 12 (3) years] children participated in the interview, 23 attended follow-up assessment. Sixty-four (80%) were verbal, 34 (42.5%) had need-based speech, 20 (25%) had friends and 37 (46%) had achieved age-appropriate ADLs. Median total follow-up period was 10 years. Lower disease severity, parent participation and higher maternal education were associated with better outcomes.