Refractory Left Temporal Lobe Epilepsy Research Articles

S06. A case of linear scleroderma en coup de sabre and epilepsy: Manage the sabre to control the seizures

Introduction Linear scleroderma en coup de sabre (ECS) is a rare form of localized scleroderma (0.4–2.7/100,000 people) ( Peterson et al., 1997 ) that has characteristic cutaneous involvement of the forehead and scalp ( Holland et al., 2006 ) and is named in reference to its “stroke of a sabre”-like appearance. It typically has onset in the pediatric population and has a higher association with extracutaneous manifestations than other forms of localized scleroderma. Focal epilepsy is the most commonly reported extracutaneous manifestation of ECS, seen in 42% of reported cases ( Doolittle et al., 2015 ). In one study, 7 of 7 patients with ECS associated epilepsy had abnormal brain imaging ( Amaral et al., 2013 ). Methods Ms. J is a 24 year old right handed woman with history of ECS diagnosed in 2007 involving the left forehead, refractory left temporal lobe epilepsy with onset in 2008, and a left anterior temporal lobe lesion that was resected in 2008 with reported pathology of arteriovenous malformation (AVM). Ms. J has undergone extensive evaluation by physicians in the departments of neurology and rheumatology at the Mayo Clinic since 2013, including two epilepsy monitoring unit (EMU) evaluations, ictal-SPECT scan, serial brain MRI and head and neck MRA studies, serological testing, and CSF testing. She has been treated with various antiepileptic medications, and methotrexate and mycophenolate mofetil have been used to manage her ECS. Results Preoperative imaging of the left anterior temporal lobe lesion was retrospectively reviewed by the Mayo Clinic neuroradiology department and is not suggestive of AVM; request for pathology slides from the outside facility has been unsuccessful. Serial imaging since the resection shows new T2 hyperintensity, microhemorrages on susceptibility sequences, and scattered enhancement in the mid and posterior left temporal region, identical in appearance to the resected anterior temporal lobe lesion. Ms. J enjoyed 2 extended periods of seizure freedom, 2008 – May, 2011, and November, 2013 – November, 2016. Her seizure control correlated with treatment of her ECS: she reliably took methotrexate from 2008 – February, 2011 and mycophenolate mofetil from September, 2013 – August, 2016. She restarted mycophenolate mofetil in Jaunary, 2017 and has been seizure free since July (except for an EMU admission October, 2017 on reduced antiepileptic medications). She has been treated with a combination of lamotrigine, levetiracetam, and oxcarbazepine, currently all three, and was maintained on antiepileptic medications since 2008. Conclusion This case illustrates the importance to recognize ECS as a cause of focal epilepsy and brain imaging lesions, and suggests a need for ECS management for seizure control. Proper diagnosis is needed to avoid ineffective brain surgery and to prompt appropriate ECS treatment.

Evaluating the Effectiveness of Medication Plus Neurofeedback on Decrement of Severity of Epileptic Symptoms in Two Patients with Refractory Left Temporal Lobe Epilepsy in Isfahan

Introduction: Mesial temporal lobe epilepsy is one of the most common complex partial syndromes which its common treatment is medication. The present study aims at comparing the efficacy of drug therapy and drug therapy combined with neurofeedback on decrement of severity of epileptic symptoms in patients with refractory left temporal lobe epilepsy in Isfahan city. Materials & methods: This is a single case study, with MBD design and multiple baselines, in which two patients with refractory left temporal lobe epilepsy were recruited from Masih Epilepsy Centre in Isfahan city. The subjects were entered to MBD design after the baseline phase. They received 30 sessions of neurofeedback individually, and two weeks after the intervention, they were reassessed in the follow up phase for three consequent weeks. Scale of Temporal Lobe Epilepsy Symptoms was used to collect the data. Findings: The results showed that according to the visual analysis of the data, neurofeedback intervention was effective for both of the patients, with PND 100% for the first patient, and PND 90% for the other. The decrement of severity of epileptic symptoms was maintained constant three weeks following the intervention. Discussion & conclusions: The findings suggest that neurofeedback is effective on decrement of severity of epileptic symptoms in patients with refractory temporal lobe epilepsy in Isfahan city.

Test-retest reliability of cortical language mapping in a patient with refractory focal epilepsy: 11 years later.

Electrical stimulation mapping (ESM) is considered the gold standard for identification of essential language cortex and is especially important in patients for whom classic language landmarks are less useful because of reorganization in response to epileptogenic or neoplastic cortex. However, little is known regarding the reliability of the procedure, particularly over extended time intervals. The authors present the case of a young man with refractory left temporal lobe epilepsy in the setting of a low-grade left temporal tumor who had undergone intraoperative language mapping at age 14 years and repeat mapping at age 25. Results from both the initial ESM and the repeat ESM 11 years later revealed a positive auditory description naming site in the same location on the superior temporal gyrus, at the anterior aspect of the tumor. This case provides support for the reliability of ESM and underscores intraindividual reliability in the location of language cortex over a prolonged period.

Triple pathological findings in a surgically amenable patient with mesial temporal lobe epilepsy.

Mesial temporal sclerosis (MTS) is a well-recognized cause of intractable epilepsy; however, coexistence with focal cortical dysplasia (FCD) is less common. Middle fossa epidermoid cysts are rare and may involve the temporal lobe. Most epidermoids are clinically silent, slow-growing, and seldom associated with overt symptomatology, including seizures. We describe a patient with multiple comorbidities including left MTS and a large epidermoid cyst involving the left quadrigeminal plate cistern compressing upon the cerebellar vermis and tail of the left hippocampus, resulting in refractory left temporal lobe epilepsy. The patient underwent left anterior temporal lobectomy. The surgical pathology demonstrated a third pathological finding of left temporal FCD type Ia. The patient has been seizure-free since the surgery. This case provides additional information with regard to the understanding of epileptogenicity and surgical planning in patients with MTS and epidermoid cysts.

Weighing the value of memory loss in the surgical evaluation of left temporal lobe epilepsy: a decision analysis.

Anterior temporal lobectomy is curative for many patients with disabling medically refractory temporal lobe epilepsy, but carries an inherent risk of disabling verbal memory loss. Although accurate prediction of iatrogenic memory loss is becoming increasingly possible, it remains unclear how much weight such predictions should have in surgical decision making. Here we aim to create a framework that facilitates a systematic and integrated assessment of the relative risks and benefits of surgery versus medical management for patients with left temporal lobe epilepsy. We constructed a Markov decision model to evaluate the probabilistic outcomes and associated health utilities associated with choosing to undergo a left anterior temporal lobectomy versus continuing with medical management for patients with medically refractory left temporal lobe epilepsy. Three base-cases were considered, representing a spectrum of surgical candidates encountered in practice, with varying degrees of epilepsy-related disability and potential for decreased quality of life in response to post-surgical verbal memory deficits. For patients with moderately severe seizures and moderate risk of verbal memory loss, medical management was the preferred decision, with increased quality-adjusted life expectancy. However, the preferred choice was sensitive to clinically meaningful changes in several parameters, including quality of life impact of verbal memory decline, quality of life with seizures, mortality rate with medical management, probability of remission following surgery, and probability of remission with medical management. Our decision model suggests that for patients with left temporal lobe epilepsy, quantitative assessment of risk and benefit should guide recommendation of therapy. In particular, risk for and potential impact of verbal memory decline should be carefully weighed against the degree of disability conferred by continued seizures on a patient-by-patient basis.