Syncope vs. Seizure: Ictal Bradycardia and Ictal Asystole.

Background: Ictal arrhythmia is a rare condition that causes arrhythmic manifestations induced by epileptic seizures, including asystole or bradycardia. Ictal asystole (IA) is a very rare condition found in patients undergoing video-encephalography (EEG) monitoring. It is often related to temporal lobe epilepsy and can cause syncope, which can lead to injury or even death. Case Presentation. Two patients with epilepsy showed symptoms of syncope. Both patients underwent 4-day ambulatory EEG tests and were diagnosed with IA. Following the tests, the patients were implanted with a permanent pacemaker, and one of them underwent a temporal lobectomy. As a result of these procedures, the patients experienced a reduction in episodes of symptomatic syncope. Conclusion: Patients with ictal asystole and symptomatic ictal bradycardia are at increased risk of falls due to seizures. Although there are no specific guidelines for managing this condition, antiseizure medications, epilepsy surgery, and cardiac pacemaker implantation have been effective treatments.

Comparative effectiveness of digital mental healthcare models for adults with epilepsy: A study protocol of a randomized controlled trial.

OBJECTIVE: Mental health complaints are prevalent among people with epilepsy, yet there are major barriers that prevent access to psychological care, including high out-of-pocket costs and a lack of accessible specialized services. The purpose of the current study is to examine the comparative efficacy, acceptability, cost-effectiveness, and long-term outcomes of a digital psychological intervention when delivered under two models of care (i.e., guided vs. unguided) in supporting the mental health and functioning of adults with epilepsy. METHOD: Approximately 375 participants across Australia will be enrolled. Eligible participants will have a confirmed diagnosis of epilepsy, experience difficulties with their emotional health, be at least 18 years of age, and live in Australia. Participants will be randomized (2:2:1) to receive the Wellbeing Neuro Course, a 10-week internet-delivered program, with (i.e., guided) or without guidance by a mental health clinician (i.e., unguided), or be allocated to a treatment-as-usual waiting-list control group. Participants will complete online questionnaires at pre-, post-treatment, and 3- and 12-month follow-up and consent to have their data linked to their medical records to capture healthcare system resource use and costs. ANALYSIS: Primary outcome measures will be symptoms of depression and anxiety. A cost-utility analysis will be undertaken using the Australian healthcare system perspective and according to current economic evaluation guidelines. Resource use and costs to the healthcare system during the study period will be captured via data linkage to relevant administrative datasets in Australia. SIGNIFICANCE: The results of this trial will provide important data concerning the relative outcomes of these different models of care and will inform the integration of digital psychological interventions translation into healthcare services. ETHICS AND DISSEMINATION: The Human Research Ethics Committee of Macquarie University approved the proposed study (Reference No: 520231325151475). The results will be disseminated through peer-reviewed publication(s). ANZCTR TRIAL REGISTRATION NUMBER: ACTRN12623001327673. PLAIN LANGUAGE SUMMARY: This study seeks to find out if a 10-week online psychological treatment can improve the mental health and well-being of Australian adults with epilepsy. Around 375 participants will be randomly assigned to different groups: one will receive treatment with guidance from mental health clinician (guided group), one without guidance (unguided group), and one starting later (waiting control group). All participants will fill out the same outcome measures online. The main goal of this research is to compare these groups and assess how well the treatment works in improving mental health outcomes.

Déjà vécu with recollective confabulation: an unusual presentation of Alzheimer's disease.

A man in his 80s presented with gradual onset of a persistent and delusion-like perception that novel encounters are repetitions of previous experiences. Within 2 years of symptom onset, he had impaired verbal memory and executive dysfunction on neuropsychological assessment. Cerebrospinal fluid core Alzheimer's disease (AD) biomarkers analysis supported probable AD. Generalised and left temporal atrophy was seen on MRI of the brain. Neurological fludeoxyglucose-positron emission tomography (PET)/CT showed left temporal and bilateral frontal lobe hypometabolism. His presenting symptom is known as déjà vécu with recollective confabulation, a rare phenomenon associated with AD and other neurodegenerative disorders. While several potential mechanisms have been previously proposed, the fludeoxyglucose-PET/CT hypometabolism in the temporal and frontal lobes in this case suggests dual deficits in recognition memory and metacognition may be culprit mechanisms. Although uncommon, déjà vécu with recollective confabulation is a fascinating phenomenon that can provide a unique insight into memory and delusional processes in dementia.

How frequently is anxiety and depression identified and treated in hospital and community samples of adults with epilepsy?

While it is often stated that psychiatric co-morbidity in PWE is under-recognized and under-treated, little research has directly examined this assertion. The aims of this study were to understand the rates of confirmed diagnosis and treatment of depression and anxiety in people with epilepsy (PWE). Two samples were recruited: a hospital sample of 106 adult outpatients with epilepsy who underwent a structured psychiatric diagnostic interview and a community sample of 273 PWE who completed validated measures of depression and anxiety symptoms online. In the hospital sample, fewer participants who met criteria for an anxiety disorder had received a prior diagnosis compared to those with a depressive disorder (36% vs 67%). In the community sample, the rates of known diagnosis were comparable (65% vs. 69%). Approximately, one-third of PWE with an anxiety disorder (or clinically significant symptoms) were receiving current treatment compared to approximately half of those with depression. These findings confirm the high rates of psychiatric co-morbidity in PWE and indicate that a large proportion of anxiety diagnoses, in particular, are undetected and not receiving either pharmacological or psychological support. Future work is needed to improve the detection and management of psychiatric co-morbidity in PWE, especially for anxiety disorders.

Cannabinoids for drug-resistant seizures in a critically ill patient-Case report and literature review.

WHAT IS KNOWN AND OBJECTIVE: Drug-resistant seizures are life-threatening and contribute to sustained hospitalization. We present the case of a critically ill 28-year-old male with Lennox-Gastaut syndrome who had approximately 30 seizures/day in the intensive care unit. CASE DESCRIPTION: Patient required mechanical ventilation and pharmacologically induced thiopentone coma. He was commenced on cannabidiol and subsequently extubated. He remained seizure-free thereafter on a combination of cannabidiol and anti-epileptic medication that predated his critical illness. WHAT IS NEW AND CONCLUSION: Our case report provides a unique perspective on the role of cannabidiol in achieving remission from drug-resistant seizures in critically ill patients.

Design and validation of two measures to detect anxiety disorders in epilepsy: The Epilepsy Anxiety Survey Instrument and its brief counterpart.

OBJECTIVE: The study objective was to develop and validate the first epilepsy-specific anxiety survey instrument (Epilepsy Anxiety Survey Instrument [EASI]) alongside a briefer screening instrument to detect anxiety disorders in routine clinical practice (brEASI). METHODS: The instruments were developed utilizing a mixed-methods approach in four related studies. Pilot items were developed following qualitative interviews with people with epilepsy (PWE; Study 1) and consultation with multidisciplinary experts in anxiety and epilepsy (Study 2). PWE (n = 314) then completed pilot items alongside existing measures of anxiety and depression (Study 3). Factor analysis was conducted to refine the scale and select well-performing items for a briefer diagnostic screener (brEASI). The brEASI was validated against a gold standard diagnostic interview in 106 PWE recruited from an outpatient epilepsy service (Study 4). Receiver operating characteristic analysis was conducted to determine the brEASI's diagnostic performance. RESULTS: Twenty-six pilot items were generated based on the findings of Studies 1 and 2. Analyses in Study 3 resulted in an 18-item EASI, and eight well-performing items were selected for the brEASI. The area under the curve (AUC) of brEASI was excellent (AUC = 0.89, 95% confidence interval = 0.82-0.94). At a cutoff of 7, it demonstrated a sensitivity of 76% and specificity of 84% for identifying Diagnostic and Statistical Manual of Mental Disorders, 5th edition anxiety disorders. SIGNIFICANCE: The EASI and brEASI represent the first valid and reliable epilepsy-specific anxiety instruments. The EASI has been designed to comprehensively assess anxiety in PWE, whereas the brEASI may be used within busy neurology settings to provide rapid information to aid diagnoses of anxiety disorders. Given the significant prevalence and burden of anxiety in PWE, these tools are important potential solutions to improve the understanding and detection of anxiety in epilepsy.

NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome.

Opsoclonus-myoclonus syndrome (OMS) is a brainstem/cerebellar syndrome producing disabling multi-directional saccadic oscillations with oscillopsia, with or without somatic myoclonus and cerebellar ataxia (Wong et al., 2001; Armangué et al., 2016). OMS is presumed to have an autoimmune basis and patients with it are tested for antineuronal antibodies and have imaging to locate any tumors. Here we report a unusual case of a young woman who had NMDAR antibody (NMDAR-ab) positive, teratoma-related, isolated OMS without encephalopathy. Removal of her ovarian teratoma, and immunotherapy with steroids, intravenous immunoglobulin (IVIg), plasma exchange (PLEX), and ultimately with B-cell depletion with rituximab resulted in total recovery after 3 months. Patients with teratoma-related OMS very rarely have NMDAR-ab which suggests that it is not the NMDAR-ab per se that causes the OMS.

A qualitative examination and theoretical model of anxiety in adults with epilepsy.

OBJECTIVE: There is an elevated prevalence of anxiety disorders among people with epilepsy, and the comorbidity of anxiety in epilepsy is associated with adverse medical and psychosocial outcomes. Despite its importance, little is known about what psychosocial or epilepsy factors may be associated with the development of anxiety. The aim of this qualitative study was to determine what factors may explain why some people with epilepsy develop anxiety disorders and others do not. METHODS: Adults with epilepsy were recruited from an outpatient epilepsy service. Semistructured interviews were conducted with 26 participants, 15 of whom reported clinically significant levels of anxiety. Grounded theory analysis was used to develop a theoretical model of anxiety development in the context of epilepsy. RESULTS: Qualitative analyses revealed a number of processes that appeared to account for the development of anxiety in the context of epilepsy. These included inflated estimates regarding epilepsy-specific risks and excessive attempts to avoid these risks. Such excessive avoidance often resulted in greater interference with participants' role functioning, thus risking ongoing quality of life. A number of premorbid and contextual factors also appear to be implicated in the development of anxiety. CONCLUSION: This investigation provides a comprehensive account for the development of anxiety in epilepsy, which is consistent with existing theories of anxiety development and maintenance. Importantly, this model provides a foundation for future research and appropriate treatment strategies to address anxiety in people with epilepsy.

Evaluation of a Decision Aid for Women with Epilepsy Who Are Considering Pregnancy: A Randomized Controlled Trial.

BACKGROUND: For many women with epilepsy (WWE), decision making about pregnancy is complicated by considerations such as the potential teratogenicity of antiepileptic drugs, offspring risk of epilepsy, seizure occurrence during pregnancy, and the challenges of parenting amidst poorly controlled seizures. OBJECTIVE: This proof-of-concept, randomized controlled trial aimed to evaluate a decision aid (DA) developed to help WWE decide if they should start or enlarge their families. METHODS: Seventy-nine WWE of childbearing age were recruited from Epilepsy Action Australia between October and November 2013 and randomized to receive the intervention (the DA) or not, and to complete a set of questionnaires pre- and post- intervention. The DA, delivered as a PDF booklet, provided balanced evidence-based information about options, risks and benefits, including probabilities; as well as steps for clarifying values and considering options within one's personal situation. RESULTS: Compared with the control group, the DA group had statistically significant improvements in knowledge about pregnancy and epilepsy (Cohen's d = 1.24; 95%CI = 0.77 to 1.83) and reduced decisional conflict (Cohen's d = 0.59; 95%CI = 0.21 to 0.99). Changes in decision self-efficacy, certainty of choice, patient-practitioner communication abilities and value congruence with choice were comparable between the DA and control group. Importantly, women's decisions about motherhood were not biased towards either direction, and there were no adverse effects on depression or anxiety. All women who received the DA indicated they would recommend it to other WWE. CONCLUSIONS: The DA has the potential to serve as a useful support tool for WWE who are considering motherhood. Future research is needed to test the DA in clinical settings with guidance from a health professional. The trial was registered with the Australian New Zealand Clinical Trials Registry (ID ACTRN12613001082796).

Pregnancy-related knowledge and information needs of women with epilepsy: a systematic review.

For women with epilepsy (WWE), pregnancy is complicated by considerations such as the potential teratogenicity of antiepileptic drugs (AEDs) versus the risks of having seizures during pregnancy. However, qualitative research suggests that many WWE remain uninformed about the risks associated with epilepsy and pregnancy and may, therefore, be making uninformed decisions about their families. The objectives of this review were to determine the level of patient knowledge, their informational needs, and whether these needs concerning pregnancy and childbirth issues are met among WWE. Electronic databases searched were PsycINFO, MEDLINE, Embase, CINAHL, and Web of Science. Studies were included if they used quantitative methods to survey WWE aged 16years or older about their knowledge, access to information, or informational needs specifically regarding epilepsy and pregnancy. Twelve studies were identified and assessed for research standards using the Quality Index. Overall Quality Index score was only 7.1 out of 14, indicating significant design limitations of many included studies, including highly selective sampling methods and the use of unvalidated outcome measures. There was a paucity of studies investigating specific areas of women's knowledge and information needs. Overall, WWE reported adequate awareness, but limited knowledge, of key issues regarding pregnancy and childbirth. Across studies, many women reported not receiving information about these issues. Evidence suggested that many WWE wanted to receive more information - particularly about the risks of AEDs for their offspring - well in advance of choosing an AED or planning pregnancy. Women aged under 35years wanted the most information. Preconception counseling received by many WWE appears insufficient, risking uninformed decision-making about pregnancy. Further research is needed to investigate the barriers that WWE face in accessing, receiving, and retaining appropriate information.

The phenotypic spectrum of dystonia in Mohr-Tranebjaerg syndrome.

Mohr-Tranebjaerg syndrome (MTS) is an X-linked recessive disorder characterized by deafness and dystonia. However the phenotypic expression of dystonia has not been systematically defined. We report clinical, neurophysiological, and ophthalmological data on 6 subjects from 3 Australian kindreds, including 2 with novel mutations, together with a systematic review of the literature, in order to define the phenotypic expression of dystonia. Profound hearing impairment in affected males develops by infancy and precedes the development of dystonia, which varies in time of onset from the first to the sixth decades, with a peak in the second and third decades. Dystonia in MTS tends to be focal, segmental, or multifocal in distribution at onset, with a predilection for the upper body, variably involving the head, neck, and upper limbs. The majority of patients have progression or generalization of their dystonia regardless of age of onset. Within our 3 kindreds, we observed relative intrafamilial homogeneity but interfamilial variation. The median time to the development of moderate-severely disabling dystonia in these subjects was 11 years. Associated features included progressive cognitive decline, pyramidal signs, and in 1 patient, gait freezing and postural instability. Optic atrophy and cortical visual impairment were both observed. We report for the first time a female patient who developed multiple disabling neurological complications of MTS. Our findings more clearly define and expand the phenotype of both the dystonia and other neurological features of MTS and have implications for the diagnosis and management of this condition.

Acute psychiatric illness in a young woman: an unusual form of encephalitis.

A 21-year-old woman was admitted to hospital with a diagnosis of acute psychotic mania, but developed, over approximately 6 weeks, seizures, delirium, catatonia, movement disorder and autonomic dysfunction. She was found to have antibodies to N-methyl-D-aspartate (NMDA) NR1-NR2 receptors in both serum and cerebrospinal fluid, consistent with anti-NMDA-receptor encephalitis, a severe, potentially lethal but treatment-responsive encephalitis often associated with ovarian tumour. With aggressive immunotherapy and bilateral oophorectomy, she recovered over a period of 14 months from her initial presentation. No ovarian tumour was identified.