Critical incidents, nocturnal supervision, and caregiver knowledge on SUDEP in patients with Dravet syndrome: A prospective multicenter study in Germany

Abstract

Objective

The aim was to investigate the monitoring, interventions, and occurrence of critical, potentially life-threatening incidents in patients with Dravet syndrome (DS) and caregivers’ knowledge about sudden unexpected death in epilepsy (SUDEP).

Methods

This multicenter, cross-sectional study of patients with DS and their caregivers in Germany consisted of a questionnaire and prospective diary querying the disease characteristics and demographic data of patients and caregivers.

Results

Our analysis included 108 questionnaires and 82 diaries. Patients with DS were 49.1% male (n = 53), with a mean age of ...

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Long‐term treatment with ganaxolone for seizures associated with cyclin‐dependent kinase‐like 5 deficiency disorder: 2‐year open‐label extension follow‐up

Abstract

Objective

In the placebo-controlled, double-blind phase of the Marigold study (NCT03572933), ganaxolone significantly reduced major motor seizure frequency (MMSF) in patients with cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD). We report 2-year safety and clinical outcomes data from the open-label extension (OLE) phase of Marigold.

Methods

Patients with CDD who completed the double-blind phase were eligible to continue in the OLE. Efficacy assessments included MMSF reduction from pre-randomization baseline, responder rates, and Clinical Global Impression of Improvement scale (CGI-I) scores, including assessment of ...

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Climate change: attitudes and concerns of, and learnings from, people with neurological conditions, carers and healthcare professionals

Abstract

Objective

Concern about climate change amongst the general public is acknowledged by surveys. The healthcare sector must play its part in reducing greenhouse gas emissions and adapting to a changing climate, which will require the support of its stakeholders including those with epilepsy who may be especially vulnerable. It is important to understand this community’s attitudes and concerns about climate change and societal responses.

Methods

A survey was made available to over 100,000 people amongst a section of the neurological community (patients, carers ...

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Valproate use associated with frontal and cerebellar grey matter volume reductions: a voxel‐based morphometry study

Abstract

Recent morphometric MRI studies suggested the possibility that valproate (VPA) use is associated with parieto-occipital cortical thinning in patients with heterogeneous epilepsy syndromes. In this study, we examined the effect of VPA on the brain volume using a large number of homogenous patients with idiopathic generalized epilepsy. Voxel-based morphometry was used to compare regional grey matter (GM) volume between 112 patients currently taking VPA (VPA+ group), 81 patients not currently taking VPA (VPA– group), and 120 healthy subjects (control group). ...

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Female Sex Steroids and Epilepsy Part 2: A Practical and Human Focus on Catamenial Epilepsy

Abstract

Catamenial epilepsy is the best described and most researched sex-steroid specific seizure exacerbation. Yet despite this there are no current evidence based treatments nor an accepted diagnostic tool. The best tool we can currently have is tracking seizures over menstrual cycles however the reality of tracking seizures and menstrual cycles is fraught with challenges. In Part 1 (citation to be added on publication) of this two-part review we outlined the often complex and reciprocal relationship between seizures and sex steroids. ...

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Epilepsy with generalized tonic–clonic seizures alone: Electroclinical features and prognostic patterns

Abstract

Objective

Epilepsy with generalized tonic–clonic seizures alone (GTCA) is a common but poorly characterized idiopathic generalized epilepsy (IGE) syndrome. Hence, we investigated electroclinical features, seizure outcome, and antiseizure medication (ASM) withdrawal in a large cohort of GTCA patients.

Methods

In this multicenter retrospective study, GTCA patients defined according to the diagnostic criteria of the International League Against Epilepsy (2022) were included. We investigated prognostic patterns, drug resistance at the last visit, and ASM withdrawal, along with their prognostic factors.

Results

We included 247 patients with ...

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Staged treatment response in Status epilepticus ‐ Lessons from the SENSE registry

Abstract

Objectives

While in epilepsy patients the likelihood of becoming seizure-free decreases substantially with each unsuccessful treatment, to our knowledge this has been poorly investigated in status epilepticus (SE). We aimed to evaluate the proportion of SE cessation and functional outcome after successive treatment steps.

Methods

We conducted a post-hoc analysis of a prospective, observational, multicenter cohort (SENSE), in which 1049 incident adult SE episodes were prospectively recorded at 9 European centers. We analyzed 996 SE episodes without coma-induction before the third treatment step. ...

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