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.

Epilepsia partialis continua and unilateral cortical-subcortical FLAIR-hyperintense lesion in Rasmussen's encephalitis: Is it diagnostic?

OBJECTIVE: Rasmussen encephalitis (RE) is a focal encephalitis, characterized by epilepsia partialis continua (EPC) with or without seizures and progressive unilateral deficits. Imaging characteristics of RE have been rarely described in detail in relation to EPC. So, the study aimed to explore if any relationship exists between the imaging characteristics and the presence or evolution of EPC in patients with RE. METHODS: This retrospective study included 11 patients with RE fulfilling the European consensus statement on RE followed between 2015 and 2020. RESULTS: The mean age for onset of seizures was 12 years (range 2.5-24 years). Seven patients had limb EPCs, two had face EPCs, face, and limb EPC in one, and lingual EPC in one patient. The first MRI was done within 1 day to 1 month of the onset of seizures. It was normal in two patients and showed only cortical atrophy, focal or hemispheric in four patients, caudate atrophy in two, and cortical or subcortical hyperintensity (HI) in six patients. Follow-up MRI, within 3 weeks to 6 months of the onset of EPC (mean 1.6 months) showed paramedian frontal HI with limb EPC in six patients. Insular HI in four patients; two had facial EPCs while lingual EPC and limb EPC with facial EPC was observed in one patient each. SIGNIFICANCE: Fluid-attenuated inversion recovery (FLAIR) HI and focal cortical atrophy on MRI is the most common finding in the early course of RE. T2 and FLAIR hyperintensity in the paramedian frontal or insular cortex may antedate the onset of EPC or may occur simultaneously with EPC.

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.

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.

Gyratory seizures as a presentation of temporal encephalocele.

Gyratory seizures (GS) are rare and have been reported in focal (frontal and temporal) as well as generalized epilepsies. The exact neuroanatomical substrate of gyration during a seizure is not clearly understood, but is thought to be due to the involvement of the basal ganglia. The direction of gyration occurring without head version is thought to have ipsilateral cerebral origins, while gyrational seizures preceded by forced head version are suggestive of seizure onset contralateral to the direction of gyration or the presence or absence of forced head version is thought to have important implications for lateralization of seizure origins from the direction of gyration. This case describes gyratory seizures in a young boy with a temporal encephalocele. Report of MRI brain initially indicated no abnormalities, but careful review revealed a left temporal encephalocele. PET scan showed left temporal hypometabolism. The patient underwent a left anterior temporal resection with amygdalohippocampectomy (ATL + AH) and is seizure-free for 18 months. Temporal encephalocele is the most commonly iatrogenic but may also be spontaneous, post-traumatic, or in relation to chronic otitis media. This case suggests that gyratory seizures may be a unique presentation of temporal encephalocele and this possibility warrants investigation in patients with medically refractory epilepsy.

Innovative Use of Tympanometry and Scintigraphy in Preoperative Assessment of the Patients with Chronic Otitis Media.

Normal middle ear volume indicates the well ventilated middle ear and subsequently the normal function of the Eustachian tube. We carried out preoperative assessment of the middle ear volume by tympanometry and scintigraphic evaluation of Eustachian tube patency in patients with unilateral otitis media. We correlated the middle ear volume and Eustachian tube patency in these patients. Prospective. A total of 58 patients with unilateral otitis media were studied. All patients underwent Eustachian tube scintigraphy. We categorized the patients as Group A with patent Eustachian tube and Group B with blocked Eustachian tube (ET). We assessed the equivalent middle ear volume (VeqME) in all patients and correlated it with ET patency. We also correlated the degree of hearing loss and intraoperative middle ear pathology in two groups. There is strong linear correlation between the ET patency and VeqME of the patients. Degree of hearing loss and the middle ear pathology is also found to be severe in patients with blocked ET and low VeqME. Preoperative assessment of patients with chronic otitis media should include the objective evaluation of middle ear volume and ET patency, as it is the reliable predictor of middle ear pathology.

Adult onset Still's disease: Role of scintigraphy.

Adult onset Still's disease (AOSD) often poses a diagnostic and therapeutic challenge and clinical guidelines are lacking. It is often diagnosed after a considerable delay; bone scintigraphy imaging may offer new imaging techniques for early diagnosis and successful therapy in follow-up examinations. We describe the liver and bone scan findings in a 21-year old female patient with AOSD, who had been having fever of high grade, recurrent and intermittent in character, associated with severe sore throat and joint pains. She had been extensively investigated for possible infectious disease. She had received antibiotics and antimalarial drugs without any response.

Triple ectopic thyroid: A rare entity.

Ectopic thyroid tissue is an uncommon congenital aberration. It is extremely rare to have three ectopic foci at three different sites. The thyroid scan has been used successfully to diagnose ectopic thyroid tissue. We report a case of ectopic thyroid tissue at base of tongue, another at the level of hyoid and third one as aberrant tissue at suprahyoid location in a 16 year old female who presented with swelling in front of neck. This patient was clinically diagnosed as thyroglossal cyst and was being planned for surgery. Preoperative thyroid scan helped in establishing diagnosis of ectopic thyroid which was the only functioning thyroid tissue. Thus, it prevented unnecessary surgery. Therefore it is suggested that thyroid scan and USG/CT scan must be done as routine work up in neck swellings pre operatively to avoid unnecessary surgeries.

TC MDP bone scan in evaluation of painful scoliosis.

A 18-year-old male presented with low back ache. The patient was investigated and was diagnosed to have painful scoliosis. X-ray and other examinations could not reveal any diagnosis. The patient was referred to undergo bone scan on clinical suspicion of osteoid osteoma and to rule out stress fracture if any. Planar bone scan was performed, which showed a lesion in L3 vertebra and was further evaluated with SPECT (Single photon emission computed tomography) study to characterize the lesion. On SPECT examination, the classical features of osteoid osteoma, the double density sign (11), was noted in the pars interarticularis region. These findings were confirmed by a CT scan, which showed a sclerotic lesion in pars interarticularis of L3 vertebra. The patient was posted for operation and was relieved of symptoms in the postoperative follow-up.