Role of Single Photon Emission Computed Tomography in Epilepsy

Molecular imaging with ictal single photon emission computed tomography (SPECT) is an established functional imaging modality for the presurgical evaluation of patients with refractory partial onset seizures. SPECT coregistered on to the MRI has greater sensitivity to identify the ictal onset zone. Ictal SPECT should always be interpreted in the context of other presurgical investigations. Ictal SPECT is sensitive method for the lateralization of TLE, but ictal SPECT is more sensitive when MRI is normal. Ictal SPECT and interictal PET are complementary to each other in lateralizing the side in patients with TLE and normal MRI. In extratemporal epilepsy, ictal SPECT will guide the placement of surface grid and depth electrodes

Individualised prediction of drug resistance and seizure recurrence after medication withdrawal in people with juvenile myoclonic epilepsy: A systematic review and individual participant data meta-analysis

Summary Background A third of people with juvenile myoclonic epilepsy (JME) are drug-resistant. Three-quarters have a seizure relapse when attempting to withdraw anti-seizure medication (ASM) after achieving seizure-freedom. It is currently impossible to predict who is likely to become drug-resistant and safely withdraw treatment. We aimed to identify predictors of drug resistance and seizure recurrence to allow for individualised prediction of treatment outcomes in people with JME. Methods We performed an individual participant data (IPD) meta-analysis based on a systematic search in EMBASE and PubMed – last updated on March 11, 2021 – including prospective and retrospective observational studies reporting on treatment outcomes of people diagnosed with JME and available seizure outcome data after a minimum one-year follow-up. We invited authors to share standardised IPD to identify predictors of drug resistance using multivariable logistic regression. We excluded pseudo-resistant individuals. A subset who attempted to withdraw ASM was included in a multivariable proportional hazards analysis on seizure recurrence after ASM withdrawal. The study was registered at the Open Science Framework (OSF; https://osf.io/b9zjc/). Findings  368) was predicted by an earlier age at the start of withdrawal, shorter seizure-free interval and more currently used ASMs, resulting in an average internal-external cross-validation concordance-statistic of 0·70 (95%CI 0·68–0·73). Interpretation We were able to predict and validate clinically relevant personalised treatment outcomes for people with JME. Individualised predictions are accessible as nomograms and web-based tools. Funding MING fonds

Legal challenges in neurological practice

Clinical neuroscience has made tremendous advances over the last century. Neurology as a discipline is still considered challenging and at times risky due to the natural history and progressive course of few of the neurological diseases. Encouragingly, the patient and their caregivers are now increasingly willing to be actively involved in making decisions. The patients' relationship with the doctor is a reflection of the society. A society that is orienting itself toward “rating” and “feedback” has made this doctor–patient relationship, a consumer–service provider relationship. This perhaps is due to commercialization of health that usually accompanies globalization. Moreover, a rapid influx of information from potential erroneous sources such as the Internet has also made patient and caregivers not being hesitant to taking legal course in the case of adverse events during treatment or simply because of dissatisfaction. The purpose of the legal process initiated by patients with neurological ailments is more often to compensate for the income lost, physical and psychological anguish that accompanies disease and its treatment, and to fund treatment or rehabilitation requirements. However, it is not clearly established if monetary benefits acquired lead to better opportunities for recovery of the patient. The consumer protection act and commercialization of medical services may well have an adverse effect on the doctor and patient relationship. Hence, there is a great need for all medical professionals to mutually complement and update each other. This review examines legal (litigation) processes with special interest on medicolegal system in patients with neurological ailments and the challenges faced by the neurologist during day-to-day clinical practice

Extrahepatic portal vein obstruction with parkinsonism and symmetric hyperintense basal ganglia on T1 weighted MRI

Abnormal high signal in the globus pallidus on T1 weighted magnetic resonance imaging (MRI) of the brain has been well described in patients with chronic liver disease. It may be related to liver dysfunction or portal-systemic shunting. We report a case of extra hepatic portal vein obstruction with portal hypertension and esophageal varices that presented with extra pyramidal features. T1 weighted MRI brain scans showed increased symmetrical signal intensities in the basal ganglia. Normal hepatic function in this patient emphasizes the role of portal- systemic communications in the development of these hyperintensities, which may be due to deposition of paramagnetic substances like manganese in the basal ganglia

Clinical characteristics and long-term outcome of surgery for hypothalamic hamartoma in children with refractory epilepsy

Context: Hypothalamic hamartomas (HH) are ectopic masses of neuronal and glial tissue most commonly presenting with medically refractory gelastic seizures with evolution to other seizure types. They are also associated with cognitive and behavioral problems to varying extent. Surgery has been found to improve quality of life in more than 50% of patients. Aim: To evaluate the clinical characteristics and long-term outcome of surgery in children with HH and refractory epilepsy. Materials and Methods: A retrospective analysis of presurgical, surgical, and postsurgical data of six children who underwent surgery for HH and had at least 3 years follow-up was performed. Results: Six children (male: female = 5:1) aged 3-16 years (at the time of surgery) underwent surgical resection of HH for refractory epilepsy. At last follow-up (range 3-9 years), three children were in Engel′s class I, two in Class II, and one in class III outcome. Significant improvement in behavior, quality of life was noted in four children; while the change in intelligence quotient (IQ) was marginal. Conclusions: Medically refractory epilepsy associated with behavioral and cognitive dysfunction is the most common presentation of HH. Open surgical resection is safe with favorable outcome of epilepsy in 50% with significant improvement in behavior and marginal change in cognitive functions

Neuroplasticity in hemispheric syndrome: An interesting case report

Functional hemispherectomy is an accepted treatment in hemispherical intractable epilepsy syndromes. We report a patient who had functional hemispherectomy for intractable seizures secondary to right hemispheric cortical dysplasia. Preoperatively, the patient had mild left hemiparesis and functional magnetic resonance imaging (fMRI) showed bilateral motor function lateralization to normal left hemisphere. The patient remains seizure free at 1-year follow-up, with no deterioration of motor power on left side. This report reviews physiology of neural plasticity for motor function lateralization and also reliability of fMRI in determining the functional shift

Outcome of epilepsy surgery in children after evaluation with non-invasive protocol

Objective : To assess outcome of epilepsy surgery in children with medically refractory partial epilepsy evaluated with non-invasive protocol and to determine the predictors of outcome. Patients and Methods : Retrospective analysis of presurgical, surgical, and postsurgical data was performed in 87 children who had at least 1 year post surgery follow-up. Outcome was assessed according to Engel's outcome classification. Stepwise regression followed by logistic regression analysis was employed in data analysis. Results : Mean follow-up was 32 (12-58) months and 44 (50.6%) were males. The age of onset of epilepsy was below the age of 2 years in 24 (30.8%). Resective surgery was done in 78 children. The commonest surgery performed was a temporal resection (88.9%) in adolescents and an extra-temporal resection ( 60.6%) in children. The commonest pathology was hippocampal sclerosis (HS) in adolescents and developmental, tumoral lesions, and gliosis in children. At last follow-up, 50 (64.1%) were seizure free and Engel's favourable outcome was noted in 59 (75.6%). After stepwise regression analysis, variables found to be significant (P < 0.05) and predicting a favourable outcome were lesion on MRI, normal IQ, and partial seizures without secondary generalization. Bilateral spikes on interictal EEG and acute postoperative seizures were predictors of poor outcome. A regression model was developed; the sensitivity, specificity, accuracy, and area under ROC curve were 82%, 91%, 88.5%, and 0.97%, respectively. Conclusion : Favourable outcome after epilepsy surgery can be obtained in children with temporal lobe epilepsy with HS and lesion-related epilepsies in developing countries with limited resources, after evaluation with a non-invasive protocol