Valproate MHRA guidance: limitations and opportunities

Recent publication of the Medicines and Healthcare products Regulatory Agency (MHRA) in the United Kingdom has strengthened the regulatory measures for valproate medicines. It highlights the importance of making women of childbearing age with epilepsy aware of the teratogenic risks of valproate and encourages the withdrawal of it from those currently prescribed. While a significant directive, it raises concerns of not having considered the impact on special populations such as women with Intellectual Disability (ID). While it is important that women with ID are not excluded from such safety initiatives, due caution needs to be taken on a case by case basis preferably, to ensure their best interests are central to the decision making. Many women with moderate to profound ID cannot have informed consented sexual relationships not to mention cognitive incapability to make informed choices on medication suitability. These women are at potential risk of having their epilepsy control undermined due to the MHRA directives. Around 30% of people with moderate to profound ID have seizures of which 60% are considered treatment resistant. In this vulnerable population changes to medication without clear clinical and social insights could lead to increased harm levels. This paper enumerates the challenges of application of the new directive to these special populations and proposes a pathway based on individual cognitive ability to provide informed consent to facilitate the continuation or removal of valproate. It is important not to lose sight of individual circumstances and the importance of working collaboratively toward providing person center care

Confidence College – an online education tool for neurology patients

COVID-19 and its aftermath highlight the importance of patient self-care and involvement in monitoring and improving their health. Resources to guide this are essential. Our objective was to create a web-based patient education tool, to facilitate patient education and empowerment for people with epilepsy, multiple sclerosis and Parkinson’s disease, available without cost to patients, carers and clinicians. This project was conducted within community and secondary neurology services. Patients and their carers were involved in designing, reviewing and revising the tool, as equal partners with clinicians and digital engineers. A web-based design template was developed with graphics and links to enable patients to create personalised plans. Participants are patients, carers, clinicians (neurology consultants and specialist nurses), neurological charities, the London Neuroscience Clinical Network, NHS England and Shift.ms (a service design team with experience in creating digital services for individuals living with neurological conditions). Shift.ms conducted in-depth interviews. Clinicians used evidence from personal and PubMed databases. Shift.ms analysed and co-ordinated the responses, and designed the pilot tool. Confidence College provides a delivery model for patient education relating to multiple sclerosis, epilepsy and Parkinson’s disease. It requires follow-up evaluation regarding uptake. This web-based accessible patient empowerment tool has no limit on recurrent use, low maintenance costs and no additional costs in up-scaling the number of users. It is ideally suited for use during and after the COVID-19 pandemic

Development of the Epilepsy Risk Awareness scale (ERA scale) for people with epilepsy

Purpose: Quality of life in people with epilepsy depends on balancing protection from risks and avoiding unnecessary restrictions. The Epilepsy Risk Awareness Checklist (ERAC) was developed to summarise an individual’s safety, health care and quality of life and to facilitate communication between professionals. Although effective, the existing Checklist required quantification and shortening to increase its utility, particularly as a longitudinal tool for measuring and communicating changes over time. Methods: 5 clinical experts, 3 people with epilepsy and 5 carers assessed the importance of each item on the ERAC questionnaire in a two-round Delphi survey. The refined Epilepsy Risk Awareness scale (ERA scale) was piloted in 30 patients to obtain an overall and sub-scale score for personal safety, health care, and quality of life domains, and was compared with the validated Seizure Severity Scale and Epilepsy Self-Management Scale. Results: ERAC was shortened from 69 to 48 items to take 15–20 min for completion. Pilot results showed good internal consistency for the overall ERA scale, for the Personal Safety and Health Care subscales, but less for the Quality of Life subscale. There was strong association between ERA scale and the Epilepsy Self- Management Scale, but little relationship with Seizure Severity Scale scores, which focus on individual seizures. User ratings were high. Conclusions: The ERA scale has been shortened and quantified to provide an objective measure of the risks and safety profile in people with epilepsy. The scale will be further tested for intra-rater variability and utility

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension

BACKGROUND: We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG). METHODS: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH. RESULTS: SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided. CONCLUSIONS: This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH

Length of carotid stenosis predicts peri-procedural stroke or death and restenosis in patients randomized to endovascular treatment or endarterectomy.

BACKGROUND: The anatomy of carotid stenosis may influence the outcome of endovascular treatment or carotid endarterectomy. Whether anatomy favors one treatment over the other in terms of safety or efficacy has not been investigated in randomized trials. METHODS: In 414 patients with mostly symptomatic carotid stenosis randomized to endovascular treatment (angioplasty or stenting; n = 213) or carotid endarterectomy (n = 211) in the Carotid and Vertebral Artery Transluminal Angioplasty Study (CAVATAS), the degree and length of stenosis and plaque surface irregularity were assessed on baseline intraarterial angiography. Outcome measures were stroke or death occurring between randomization and 30 days after treatment, and ipsilateral stroke and restenosis ≥50% during follow-up. RESULTS: Carotid stenosis longer than 0.65 times the common carotid artery diameter was associated with increased risk of peri-procedural stroke or death after both endovascular treatment [odds ratio 2.79 (1.17-6.65), P = 0.02] and carotid endarterectomy [2.43 (1.03-5.73), P = 0.04], and with increased long-term risk of restenosis in endovascular treatment [hazard ratio 1.68 (1.12-2.53), P = 0.01]. The excess in restenosis after endovascular treatment compared with carotid endarterectomy was significantly greater in patients with long stenosis than with short stenosis at baseline (interaction P = 0.003). Results remained significant after multivariate adjustment. No associations were found for degree of stenosis and plaque surface. CONCLUSIONS: Increasing stenosis length is an independent risk factor for peri-procedural stroke or death in endovascular treatment and carotid endarterectomy, without favoring one treatment over the other. However, the excess restenosis rate after endovascular treatment compared with carotid endarterectomy increases with longer stenosis at baseline. Stenosis length merits further investigation in carotid revascularisation trials

Encephalitis associated to metabotropic glutamate receptor 5 (mGluR5) antibodies in cerebrospinal fluid

Copyright © 2018 Guevara, Farias, Silva-Rosas, Alarcon, Abudinen, Espinoza, Caro, Angus-Leppan and de Grazia. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. A 68-years-old Hispanic man, complained of night sweats, low grade fewer, unexplained weight loss, and memory problems over 3 months. Abdominal tomography showed multiple intra-abdominal adenopathy and biopsy confirmed classic Hodgkin's lymphoma. He commenced treatment with chemotherapy. Three months later, he had acute onset of inattention, auditory hallucinations and alterations of anterograde memory. The patient developed psychomoto

Familial limb pain and migraine: 8-year follow-up of four generations

Background Migraine limb pain may be under-recognized in adults and children. There is little information about familial forms of this disorder. Objectives To describe the clinical and inheritance patterns of familial migraine limb pain over four generations and to review the evidence for limb pain as a manifestation of migraine. Methods Prospective clinical and pedigree analysis with an 8-year follow-up of 27 family members. Results Eight members of the family had benign recurrent limb pain associated with headache in a dominant inheritance pattern. Limb pain occurred before, during or after the headache, with probable or definite migraine with aura, migraine without aura and lower-half headache. The limb pain fulfilled the International Headache Society criteria for aura in six patients and also occurred without headache in three. Four members of the family had recurrent abdominal pain and/or motion sickness in childhood. Conclusions This is the first report of dominant familial limb pain temporally associated with migraine headache, starting in adulthood or starting in childhood and continuing into adulthood. A search for a genetic marker is indicated. Limb pain should be included as a childhood periodic syndrome linked to migraine and recognized as part of the migraine spectrum in adults.The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: Fondecyt (Chile) Grant 1120339