Epilepsy and Behavior Case Reports最新文献

Seizure types have been described that do not conform to traditional classification schemes. We present another unusual type characterized by focal onset with secondary generalization, that is followed immediately by continued focal activity that generalizes again without an intervening break. Better understanding of these seizure types may allow improved targeted therapies and help shed light on the mechanistic underpinnings of epilepsy.

Duplication of the methyl-CpG-binding protein 2 gene (MECP2) is a rare condition that results in epilepsy in half of the cases. Although this condition has been well characterized in the literature, there is a lack of research on MECP2 duplication-related epilepsy and its management.

We present the case of an eleven-year old male with MECP2 duplication and epilepsy, who was resistant to polytherapy. The patient responded well to valproic acid (VPA) initially and upon re-challenge. This case report provides evidence for the use of VPA as an initial monotherapy for treatment of drug-resistant MECP2 duplication-related epilepsy.

A 21-year-old male with an SCN1A mutation died of cerebral herniation 3 h after a seizure occurring during physical activity. Cases of fatal cerebral edema in patients with SCN1A mutations after fever and status epilepticus have been recently reported raising the question whether sodium channel dysfunction may contribute to cerebral edema and thereby contribute to the increased premature mortality in Dravet Syndrome. We report on our patient and discuss whether the combination of hyperthermia and ion channel dysfunction may not only trigger seizures but also a fatal pathophysiological cascade of cerebral edema and herniation leading to cardiorespiratory collapse.

Lacosamide is one of the third-generation antiseizure drugs that block voltage-gated sodium channels by enhancing slow inactivation. The most common adverse effects of lacosamide include dizziness, headache, nausea, vomiting, diplopia, fatigue, and sedation. Less common side effects include memory impairment, weight gain, rash, and atrioventricular block. In this article, we describe a patient with Lennox–Gastaut syndrome who developed excessive laughing as a rare side effect of lacosamide with complete resolution after discontinuation of the medication. The present case illustrates that excessive laughing may occur as an adverse effect of lacosamide.

Introduction

Drug-resistant epilepsy (DRE) occurs in 20–30% of all patients who develop epilepsy and can occur from diverse causes. Cyclosporine-A (CSA) is an immunosuppressive drug utilized to prevent graft-versus-host disease (GvHD) in transplant patients and is known to cause neurotoxicity, including seizures. In some cases, however, patients can develop DRE. Only a limited number of cases have been reported in which DRE has developed after CSA exposure — all in children. Here we present a rare case of an adult developing DRE after post-transplant CSA neurotoxicity. In addition, we provide a comprehensive review and analysis of all reported cases in the literature.

Case report

A 29-year-old man with Non-Hodgkin's Lymphoma underwent an allogenic hematopoietic stem cell transplant and experienced a CSA-induced seizure at 7.5 months' post-transplant. The patient was discontinued on CSA and began a low dose tacrolimus regimen. At 33 months' post-transplant, he had seizure recurrence and developed DRE. Imaging revealed right mesial temporal sclerosis (MTS) and video EEG localized ictal activity to the right anterior temporal lobe. He was successfully treated with a right anterior temporal lobectomy and amygdalohippocampectomy.

Literature review

Seven peer-reviewed studies described 15 patients who underwent transplantation with post-transplant CSA administration and subsequently developed DRE following an initial CSA-induced seizure. All 15 patients were children suggesting that young age is a risk factor for DRE after CSA-induced seizures. Initial CSA-induced seizures occurred at an average of 1.6 ± 1.1 months after transplant and seizure recurrence 9.2 ± 8.0 months after transplant. All reported CSA routes of administration (n = 6) were intravenous and 7 of 9 (78%) reported CSA blood levels above the therapeutic range. The incidence of MTS (40%) in these 15 patients was significantly higher than the incidence in the general DRE population (24%) and was most effectively treated via epilepsy surgery.

Conclusions

The use of cyclosporine for GvHD prophylaxis and treatment following transplantation may cause seizures and be associated with DRE. Although discontinuation and dose decrease of CSA often reverse adverse neurological events, initial CSA-induced seizures may be associated with MTS that and subsequent greater risk of DRE development.

This study compared temporal lobe epilepsy (TLE) patients with amygdala lesion (AL) without hippocampal sclerosis (HS) (TLE-AL) with patients with TLE and HS without AL (TLE-HS). Both subtypes of TLE arose from the right hemisphere.

The TLE-AL group exhibited a lower Working Memory Index (WMI) on the Wechsler Adult Intelligence Scale, third edition (WAIS-III), indicating that the amygdala in the right hemisphere is involved in memory-related function. [18F]fluorodeoxyglucose positron emission topography (FDG-PET) showed glucose hypometabolism limited to the right uncus for the TLE-AL group.

The results suggest the importance of considering cognitive functions in the non-dominant hemisphere to prevent impairment after surgery.

A 52-year-old female with a longstanding history of drug-resistant epilepsy that included focal impaired awareness seizure presented at end of service of her vagus nerve stimulator (VNS) generator. She had undergone a generator replacement in 2010 without complication. However, her latest replacement was accompanied by multiple bouts of asystole. We discuss the case, possible causes of the asystole, and its relevance to the future of VNS generator replacement and epilepsy treatment.

We report a case of focal status epilepticus (SE) associated with peri-ictal water drinking (PIWD) behavior in a nine-year-old left-handed boy with epilepsy. We reviewed prior cases of epileptic peri-ictal water drinking. Only one adult patient with status epilepticus and PIWD has been reported previously. This is the first reported case of PIWD SE in a pediatric patient with frontal lobe epilepsy. We found PIWD to have no lateralizing value.