Efgartigimod efficacy and safety in refractory myasthenia gravis: UK's first real-world experience.

Background: We report our experience of patients with generalised myasthenia gravis (gMG) treated with efgartigimod, an neonatal Fc receptor antagonist, under the Early Access to Medicine Scheme (EAMS) in the UK.

Methods: Data from all UK patients treated with efgartigimod under the EAMS July 2022 to July 2023 were collected retrospectively. Efgartigimod was administered as per the ADAPT protocol (consisting of a treatment cycle of four infusions at weekly intervals with further cycles given according to clinical need).

Severe subacute onset sensorimotor polyneuropathy associated with brentuximab vedotin treatment for refractory classical Hodgkin's lymphoma.

A man in his 30s with a history of refractory classical Hodgkin's lymphoma presented with subacute onset asymmetric sensory disturbance, and severe weakness affecting his right more than left hand. Symptom onset was 18 weeks after commencing brentuximab vedotin (BV) treatment, an anti-CD30 antibody drug-conjugate for relapsed/refractory classical Hodgkin's lymphoma. Symptoms progressed to his lower limbs with distal sensory loss, gait imbalance, bilateral foot drop and mild proximal leg weakness.

Measurement variability of blood-brain barrier permeability using dynamic contrast-enhanced magnetic resonance imaging.

Dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) is used to quantify the blood-brain barrier (BBB) permeability-surface area product. Serial measurements can indicate changes in BBB health, of interest to the study of normal physiology, neurological disease, and the effect of therapeutics. We performed a scan-rescan study to inform both sample size calculation for future studies and an appropriate reference change value for patient care.

Quantification of blood and CSF volume to predict outcome after aneurysmal subarachnoid hemorrhage.

This study aimed to describe the relationship between blood and CSF volumes in different compartments on baseline CT after aSAH, assess if they independently predict long-term outcome, and explore their interaction with age. CT scans from patients participating in a prospective multicenter randomized controlled trial of patients with aSAH were segmented for blood and CSF volumes. The primary outcomes were the mRS, and the Subarachnoid Hemorrhage Outcome Tool (SAHOT) at day 28 and 180.

Evaluation of Cerebrospinal Fluid α-Synuclein Seed Amplification Assay in Progressive Supranuclear Palsy and Corticobasal Syndrome.

Background: Seed amplification assay (SAA) testing has been developed as a biomarker for the diagnosis of α-synuclein-related neurodegenerative disorders.

Objective: The objective of this study was to assess the rate of α-synuclein SAA positivity in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) and to analyze clinical and pathological features of SAA-positive and -negative cases.

Methods: A total of 96 cerebrospinal fluid samples from clinically diagnosed PSP (n = 59) and CBS (n = 37) cases were analyzed using α-synuclein SAA.

Erythrocyte deformability correlates with systemic inflammation.

Recent evidence suggests that systemic conditions, particularly those associated with inflammation, can affect erythrocyte deformability in the absence of haematological conditions. In this exploratory study, we investigated the relationship between systemic inflammatory status and erythrocyte deformability (using osmotic gradient ektacytometry) in a heterogenous study population consisting of individuals with no medical concerns, chronic conditions, and acute illness, providing a wide range of systemic inflammation severity. 22 participants were included in a prospective observational study.

Person-centred integrated care for people living with Parkinson's, Huntington's and Multiple Sclerosis: A systematic review.

Introduction: People living with long-term neurological conditions (LTNCs) have complex needs that demand intensive care coordination between sectors. This review aimed to establish if integrated care improves outcomes for people, and what characterises successful interventions.

Methods: A systematic review of the literature was undertaken evaluating multisectoral integrated care interventions in people living with Parkinson's disease (PD), Multiple Sclerosis (MS) and Huntington's disease (HD).

Mild Systemic Inflammation Increases Erythrocyte Fragility.

There is growing evidence that inflammation impairs erythrocyte structure and function. We assessed the impact of mild systemic inflammation on erythrocyte fragility in three different settings. In order to investigate causation, erythrocyte osmotic fragility was measured in mice challenged with a live attenuated bacterial strain to induce low-grade systemic inflammation; a significant increase in erythrocyte osmotic fragility was observed.

Cysteamine toxicity presenting with acute encephalopathy and spastic tetraparesis.

Cystinosis is a lysosomal storage disorder usually presenting with renal disease in infancy. As soon as the diagnosis is made, cysteamine (a cystine-depleting medication), is started, significantly improving life expectancy. We describe a young woman taking lifelong cysteamine for nephropathic cystinosis, who became acutely encephalopathic with a spastic tetraparesis secondary to cysteamine toxicity, which was potentially worsened by copper deficiency.

Expertise in surgical neuro-oncology. Results of a survey by the EANS neuro-oncology section.

Introduction: Technical advances and the increasing role of interdisciplinary decision-making may warrant formal definitions of expertise in surgical neuro-oncology.

Research Question: The EANS Neuro-oncology Section felt that a survey detailing the European neurosurgical perspective on the concept of expertise in surgical neuro-oncology might be helpful.

Material And Methods: The EANS Neuro-oncology Section panel developed an online survey asking questions regarding criteria for expertise in neuro-oncological surgery and sent it to all individual EANS members.

Young-onset dementia with Lewy Bodies presenting with apathy and alexithymia.

A diagnosis of young-onset dementia can pose a significant challenge for the clinician. We present a young patient with a very unusual presentation of Dementia with Lewy Bodies. The lack of motor symptoms and his marked apathy delayed his diagnosis.

Systemic inflammation associates with and precedes cord atrophy in progressive multiple sclerosis.

In preclinical models of multiple sclerosis, systemic inflammation has an impact on the compartmentalized inflammatory process within the central nervous system and results in axonal loss. It remains to be shown whether this is the case in humans, specifically whether systemic inflammation contributes to spinal cord or brain atrophy in multiple sclerosis. Hence, an observational longitudinal study was conducted to delineate the relationship between systemic inflammation and atrophy using magnetic resonance imaging: the SIMS (Systemic Inflammation in Multiple Sclerosis) study.

A Systematic Review and Meta-Analysis of the Pathology Underlying Aneurysm Enhancement on Vessel Wall Imaging.

Intracranial aneurysms are common, but only a minority rupture and cause subarachnoid haemorrhage, presenting a dilemma regarding which to treat. Vessel wall imaging (VWI) is a contrast-enhanced magnetic resonance imaging (MRI) technique used to identify unstable aneurysms. The pathological basis of MR enhancement of aneurysms is the subject of debate.

Red Blood Cells in the Cerebrospinal Fluid Compartment After Subarachnoid Haemorrhage: Significance and Emerging Therapeutic Strategies.

Subarachnoid haemorrhage (SAH) is a subtype of stroke that predominantly impacts younger individuals. It is associated with high mortality rates and can cause long-term disabilities. This review examines the contribution of the initial blood load and the dynamics of clot clearance to the pathophysiology of SAH and the risk of adverse outcomes.

ADCOMS sensitivity versus baseline diagnosis and progression phenotypes.

Background: The Alzheimer's Disease COMposite Score (ADCOMS) is more sensitive in clinical trials than conventional measures when assessing pre-dementia. This study compares ADCOMS trajectories using clustered progression characteristics to better understand different patterns of decline.

Methods: Post-baseline ADCOMS values were analyzed for sensitivity using mean-to-standard deviation ratio (MSDR), partitioned by baseline diagnosis, comparing with the original scales upon which ADCOMS is based.

Delivering medical leadership training through the Healthcare Leadership Academy: a four year analysis.

Background: Formal leadership training is typically targeted at senior health professionals. The Healthcare Leadership Academy (HLA) was formed in 2016 to provide a leadership programme for students and early-career health professionals. This study analyses the effectiveness of the HLA scholarship programme as an intervention for improving interest in and preparing scholars for future leadership roles.

An assistive listening device improves hearing following aneurysmal subarachnoid haemorrhage.

Background And Purpose: Hearing impairment is common following aneurysmal subarachnoid haemorrhage (aSAH). Previous studies have demonstrated that auditory processing disorder (APD) is the primary underlying pathology. Assistive listening devices (ALDs) can be used to manage APD but have not been explored in aSAH.

Role of the repeat expansion size in predicting age of onset and severity in RFC1 disease.

RFC1 disease, caused by biallelic repeat expansion in RFC1, is clinically heterogeneous in terms of age of onset, disease progression and phenotype. We investigated the role of the repeat size in influencing clinical variables in RFC1 disease. We also assessed the presence and role of meiotic and somatic instability of the repeat.

Para-infectious brain injury in COVID-19 persists at follow-up despite attenuated cytokine and autoantibody responses.

To understand neurological complications of COVID-19 better both acutely and for recovery, we measured markers of brain injury, inflammatory mediators, and autoantibodies in 203 hospitalised participants; 111 with acute sera (1-11 days post-admission) and 92 convalescent sera (56 with COVID-19-associated neurological diagnoses). Here we show that compared to 60 uninfected controls, tTau, GFAP, NfL, and UCH-L1 are increased with COVID-19 infection at acute timepoints and NfL and GFAP are significantly higher in participants with neurological complications. Inflammatory mediators (IL-6, IL-12p40, HGF, M-CSF, CCL2, and IL-1RA) are associated with both altered consciousness and markers of brain injury.