To prospectively assess the feasibility of establishing low cost epilepsy surgery programs in resource poor settings. Method: We started epilepsy surgery centers in Tier 2 and Tier 3 cities in India in private hospitals. This model is based on the identifying and operating ideal epilepsy surgery candidates on the basis of clinical history, interictal and ictal video-EEG data, and 1.5 T MRI without other investigations and without regular involvement of other specialists. Trained epileptologists formed the fulcrum of this program who identified ideal candidates, offered them counseling, and read video-EEG and MRI. We also spread epilepsy awareness among locals and physicians and established focused epilepsy clinics. The expenses were subsidized for deserving patients and policies were devised to keep video-EEG duration and staff requirement to minimum. Difficult epilepsy surgery cases were referred to established centers. Initial surgeries were performed by invited epilepsy surgeons and subsequently by local neurosurgeons. Results: A total of 125 epilepsy surgeries were performed at three centers since 2012. This included 81(64.8%) temporal lobe resections, 26 (20.8%) extratemporal focal resections, and 13 (10.4%) hemispherotomies. Of the 93 patients with more than 1 year of postoperative followup, 86 (92.5%) had Engel class IA outcome. There were minor complications in 5% patients. Average cost of presurgical evaluation and surgery was Rs. 92,707 (USD 1,324). Conclusions: It is possible to establish successful epilepsy surgery programs in resource poor setting with reasonable costs. This low cost model can be replicated in other parts of world to reduce the surgical treatment gap.
Purpose Children with refractory focal to bilateral tonic-clonic seizures, despite normal high-resolution imaging, are often not subjected to genetic tests due to the costs involved and instead undergo multimodality presurgical evaluation targeted at delineating a focal onset. The objective of this study was to ascertain genotype-phenotype correlations in this group of patients. Method An online hospital database search was conducted for children who presented in 2019 with drug-resistant epilepsy dominated by nonlateralizing focal-onset/rapid generalized (bilateral) tonic-clonic seizures (GTCS), subjected to presurgical evaluation and subsequent genetic testing due to absence of a clear focus hypothesis. Results Phenotypic homogeneity was apparent in 3 children who had onset in infancy with drug-resistant GTCS (predominantly unprovoked and occasionally fever provoked) and subsequent delayed development. 3-Tesla magnetic resonance imaging (MRI) scans were negative and video EEG documented a homogeneous pattern of multifocal and/or generalized epileptiform discharges with phenomenology favoring probable focal-onset/generalized-onset bilateral tonic-clonic seizures. All 3 tested positive for SCN1A gene variants (heterozygous missense substitution variants in 2 children, one of which was novel and a novel duplication in one that led to frameshift and premature truncation of the protein), suggestive of SCN1A-mediated epilepsy. This electroclinical profile constituted 3 out of 25 patients with SCN1A-epilepsy phenotypes at our center. Conclusions These cases suggest that children with early-onset drug-resistant “generalized” epilepsy are likely to have a genetic basis although the presentation may not be typical of Dravet syndrome. Hence, genetic testing for SCN1A variants is recommended in children with drug-resistant MRI negative focal-onset/generalized-onset bilateral tonic-clonic seizures before subjecting them to exhaustive presurgical workup and to guide appropriate treatment and prognostication.
: Understanding the exact etiology of stroke in the young has great relevance to its prevention and management. Recent development in the surgical and interventional therapy of extra cranial and intracranial vascular diseases have made it necessary to correctly identify the causative as well as contributory factors of stroke.This cross sectional observational study was done on patients admitted in the stroke unit of Geetanjali Medical College, Udaipur of aged 15 to 50 years with stroke and who have underwent at least one standard vessel imaging procedure within 2 weeks of stroke onset. Out of 310 patients, majority of the strokes occurred in the setting of a rheumatic valvular heart disease (63.6%), with mitral valve disease being the most frequent association. Almost 80% of patients had normal ECG whereas 20% of the patients had an ECG abnormality.70% of patients had normal ECHO and the most frequent etiologic abnormality detected was valvular heart disease. Scan and MRI was done in 180 patients. The most commonly performed angiographic procedure was CT angiogram. TOF MRA was the next most commonly performed angiographic procedure. The highest yield for picking up abnormalities was noted in the CT Angiography. The yield of CT angiography was 35.4% and 37.7% for extracranial and intracranial vasculature respectively.Arterial dissections are fairly common in young population and should be considered in appropriate clinical setting and all patients should have a good and timely angiographic assessment to rule out dissections. Thus, this study highlights the importance of risk factor evaluation, and control, complete etiological evaluation including angiographic assessment in the diagnosis, management and planning of secondary prevention strategies in young patients with stroke.
Photoparoxysmal response (PPR) is commonly associated with idiopathic generalised epilepsies. Most of the focal events induced by intermittent photic stimulation (IPS) are reported to be of occipital origin. Only six temporal lobe epilepsy patients have been reported in the literature with focal PPR at extraoccipital sites. We report a four-year-old girl with possible encephalitis who presented initially with epilepsia partialis continua of limbs on the right side. Interictally, she had left centro-parietal periodic lateralized epileptiform discharges (PLEDs). She responded to medical treatment and was free of seizures and motor and cognitive deficits at six months follow-up. Repeat EEG at follow-up showed left centro-parietal spikes accentuated by IPS. This is the first report of an extraoccipital, extratemporal focus showing PPR. The possible mechanism of PPR from this fronto-parietal epileptogenic focus is discussed.
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