Palliation for catastrophic nonlocalizing epilepsy: a retrospective case series of complete corpus callosotomy at a single institution.

OBJECTIVE: In this study, the authors describe their 10-year single-institution experience with single-step complete corpus callosotomy (CCC) for seizure management in pediatric and adult patients with catastrophic, medically refractory, nonlocalizing epilepsy at Advent Health Orlando. METHODS: The authors conducted a retrospective observational study of patients aged 6 months to 49 years who underwent clinically indicated CCC for drug-resistant nonlocalizing epilepsy at Advent Health Orlando between July 2011 and July 2021. Follow-up ranged from 12 months to 10 years. RESULTS: Of the 101 patients (57% of whom were male) who met eligibility criteria, 81 were pediatric patients and 20 were ≥ 18 years. All patients had seizures that appeared poorly lateralized on both electroencephalograms and clinical semiological studies. Of 54 patients with drop seizures before CCC, 29 (54%) achieved stable freedom from drop seizures after CCC. Of the 101 patients, 14 (13.9%) experienced stable resolution of all types of clinical seizures (International League Against Epilepsy classes 1 and 2). The most common postoperative neurological complication was a transient disconnection syndrome, observed in 50% of patients; of those patients, 73% experienced syndrome resolution within 2 months after surgery, and all resolved by the 2-year follow-up. Formal neuropsychological test results were stable in 13 patients assessed after CCC. CONCLUSIONS: CCC is an effective and well-tolerated palliative surgical technique. In this study, drop attacks were reduced after CCC but could recur for the first time as late as 44 months after surgery. Other seizure types were also reduced postoperatively but could recur for the first time as late as 28 months after surgery. Nearly 14% of patients achieved stable and complete freedom from seizures after CCC. Re-evaluation after CCC can reveal lateralized seizure onset in some patients.

Antiseizure Medication-Induced Alopecia: A Literature Review.

Background: Adverse effects of antiseizure medications (ASMs) remain one of the major causes of non-adherence. Cosmetic side effects (CSEs) are among the most commonly reported side effects of ASMs. In this context, alopecia is one of the CSEs that has a high intolerance rate leading to poor therapeutical compliance. Methods: We performed a literature review concerning alopecia as a secondary effect of ASMs. Results: There are 1656 individuals reported with ASM-induced alopecia. Valproate (983), lamotrigine (355), and carbamazepine (225) have been extensively reported. Other ASMs associated with alopecia were cenobamate (18), levetiracetam (14), topiramate (13), lacosamide (7), vigabatrin (6), phenobarbital (5), gabapentin (5), phenytoin (4), pregabalin (4), eslicarbazepine (3), brivaracetam (2), clobazam (2), perampanel (2), trimethadione (2), rufinamide (2), zonisamide (2), primidone (1), and tiagabine (1). There were no reports of oxcarbazepine and felbamate with drug-induced alopecia. Hair loss seen with ASMs was diffuse and non-scarring. Telogen effluvium was the most common cause of alopecia. A characteristic feature was the reversibility of alopecia after ASM dose adjustment. Conclusions: Alopecia should be considered one important adverse effect of ASMs. Patients reporting hair loss with ASM therapy should be further investigated, and specialist consultation is recommended.

Comparison of care accessibility, costs, and quality with face-to-face and telehealth epilepsy clinic visits.

During the COVID-19 pandemic, restrictions on reimbursement for telehealth visits were lifted and this visit type was suddenly available to patients around the United States of America. Telehealth visits offer potential cost savings for patients and families, which may vary by region of the world studied. Also, aggressiveness of the care patients receive may differ, and patients or families may be more likely to choose one visit type over another based on seizure control. This is a prospective face-to-face clinic versus telehealth clinic visit comparison study involving patients with seizures, their legal guardians, and caretakers who attend clinic. We compared travel distance, work-related factors, childcare, satisfaction of care, changes in seizure medication or diagnostics tests ordered, and willingness to cancel appointments to better understand the behavioral patterns of patients, caretakers, and providers. Our results indicate that many patients and families still prefer in-person interactions with their medical providers. Patient and family satisfaction levels were equal with both visit types. No significant difference was seen in medical management between face-to-face and telehealth visits. Also, prior seizure control did not dictate the type of visit chosen. Telehealth participants were significantly more willing to cancel appointments if asked to switch to face-to-face then face-to-face participants asked to complete telehealth visits. Surprisingly, we found that patients and families choosing telehealth were not statistically more likely to be employed or take less time off work. Also, distance from home to office was not significantly shorter for participants choosing face-to-face visits. Offering a combination of telehealth and face-to-face visits appears to be the optimal strategy in caring for patients with controlled and uncontrolled seizure disorders to ensure adherence with clinic visits and satisfaction with care. Our study suggests that providers are equally willing to adjust medications or order additional diagnostic testing regardless of visit type. Patients and families may be less likely to cancel telehealth visits than face-to-face visits; this finding may translate to improved seizure control and long-term decreased cost of care.

Telehealth perceptions in patients with epilepsy and providers during the COVID-19 pandemic.

Coronavirus disease 2019 (COVID-19) has required novel solutions for issues that arise with social distancing. Telehealth has become one of those solutions in many clinics around the U.S. As we look beyond, the pandemic telehealth can be utilized as an important tool for clinics in the future. Patient satisfaction will most likely affect reimbursement, while provider perception will affect implementation. We see this as a valuable tool to many epilepsy clinics. The goal of our survey was to determine the perceptions and satisfaction of patients with intractable epilepsy and providers with telehealth during the COVID-19 pandemic; we surveyed patient and providers. We evaluated the first 111 patients who participated in our telehealth visits. We conducted telephone surveys with the first 68 patients who agreed to participate. We also conducted surveys by email with our providers who participated in these telehealth visits. We found that 66% of patients and 67% of providers would use a telehealth visit in the future if given the option. Review of our patients' and providers' comments provides valuable insights for building a long-term successful intractable epilepsy telehealth clinic.

Successful Intrathecal Rituximab Administration in Refractory Nonteratoma Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Case Report.

N-methyl-D-aspartate receptor (NMDA-R) antibody encephalitis is an immune-mediated disorder characterized by the presence of anti-NMDA antibody in serum and cerebrospinal fluid, with a characteristic combination of psychological and neurological signs and symptoms. The scientific knowledge pertaining to the management of anti-NMDA-R encephalitis is growing. It is important that neuroscience nurses be aware of treatments as well as the newest novel treatment options available. Early aggressive intervention is imperative to recovery. The first line of treatment often includes high-dose steroids, intravenous immunoglobulin, and therapeutic plasma exchange. Second-line therapy for refractory NMDA-R encephalitis includes intravenous rituximab and cyclophosphamide. Even with these treatments, up to 25% of patients may be left with severe deficits or have a fatal outcome. It is well known that penetration of monoclonal anti-CD20 antibody therapy (rituximab) into the cerebrospinal fluid is 0.1% of that in the serum. Therefore, efficacy of rituximab in the treatment of NMDA encephalitis may be improved by intrathecal administration in selected cases with a poor response to intravenous rituximab. We present a case of anti-NMDA-R encephalitis that was refractory to first- and second-line therapies, who responded to intrathecal rituximab, to highlight a novel treatment that may be able to prevent long-term disability and improve clinical outcomes.

Management of obstructive sleep apnea in a developmentally delayed pediatric patient with aggressive behavior and Pierre Robin sequence.

REPORT OF CASE: This is a case of a 15-year-old nonverbal, developmentally delayed boy with history of Fragile X syndrome (FXS), autistic spectrum disorder, Pierre Robin sequence (PRS), subglottic stenosis, and ongoing obstructive sleep apnea (OSA), who required aggressive surgical intervention. OSA related symptoms included loud snoring, apneas, awakenings with gasping breaths, enuresis, and daytime sleepiness. The patient also had a history of behavioral problems including aggressive behavior, which led to his family and his health care providers experiencing challenges caring for him. For example, the patient's behavior made examining him very difficult and prevented ENT exam. Likewise, OSA was difficult to manage due to the patient's inability to cooperate with treatment. The OSA treatment course for this patient is briefly described below.

Adult convulsive status epilepticus in the developing country of Honduras.

Epidemiologic data on convulsive status epilepticus (CSE) is needed to develop preventative strategies. Epilepsy is one of the known risk factors for CSE. A systematic review of epidemiologic studies on status epilepticus (SE) completed in the United States and Europe reports that people with epilepsy account for less than 50% of cases of SE in all age groups. Less is known about the epidemiology of SE in developing countries including those in Central America. A high incidence of epilepsy, widespread non-adherence to anti-epileptic drugs (AED), and common use of complementary and alternative medicines have been shown in all ages in the developing country of Honduras, Central America. In 2003, an epidemiologic study of CSE in Honduran children demonstrated it is common and exhibits a long duration until onset of treatment. The etiologies, treatment, and outcomes of CSE in Honduran adults have not been thoroughly studied. This study is a consecutive case series of 31 adult patients presenting with CSE to the adult medicine emergency department of the tertiary care "Hospital Escuela" in the capital city Tegucigalpa, Honduras. The majority (77.4%) of patients had a prior history of epilepsy. Non-adherence to AED was the most common etiology of CSE (75.0%). The mortality rate in this pilot CSE study was 14.8%, which is similar to studies from industrialized countries where mortality from status epilepticus ranged from 7.6 to 22% for all age groups. However, this rate is concerning given that CSE from epilepsy and AED non-adherence generally carries a good prognosis. Improving AED adherence in this population appears to be the most effective approach in decreasing the rate, and possibly the mortality of Honduran adult CSE.

Increased intracranial pressure in a case of pediatric multiple sclerosis.

A 15-year-old girl presented to our emergency department with dizziness, anorexia, nausea, and malaise. Clinical examination and magnetic resonance imaging studies showed characteristic features of multiple sclerosis. Surprisingly, a diagnostic lumbar puncture showed significant intracranial hypertension in addition to numerous oligoclonal bands, elevated immunoglobulin G index and immunoglobulin G/albumin ratio in the cerebrospinal fluid. It is proposed that a large burden of active demyelinating disease may cause increased intracranial pressure, thus providing an additional sound rationale for prompt therapeutic administration of intravenous high-dose steroids.