Electrodiagnostic subtyping in Guillain-Barré syndrome patients in the International Guillain-Barré Outcome Study.

Background And Purpose: Various electrodiagnostic criteria have been developed in Guillain-Barré syndrome (GBS). Their performance in a broad representation of GBS patients has not been evaluated. Motor conduction data from the International GBS Outcome Study (IGOS) cohort were used to compare two widely used criterion sets and relate these to diagnostic amyotrophic lateral sclerosis criteria.

Digenic Leigh syndrome on the background of the m.11778G>A Leber hereditary optic neuropathy variant.

Leigh syndrome spectrum (LSS) is a primary mitochondrial disorder defined neuropathologically by a subacute necrotizing encephalomyelopathy and characterized by bilateral basal ganglia and/or brainstem lesions. LSS is associated with variants in several mitochondrial DNA genes and more than 100 nuclear genes, most often related to mitochondrial complex I (CI) dysfunction. Rarely, LSS has been reported in association with primary Leber hereditary optic neuropathy (LHON) variants of the mitochondrial DNA, coding for CI subunits (m.

Proposal of early CT morphological criteria for response of liver metastases to systemic treatments in gastroenteropancreatic neuroendocrine tumors: Alternatives to RECIST.

RECIST 1.1 criteria are commonly used with computed tomography (CT) to evaluate the efficacy of systemic treatments in patients with neuroendocrine tumors (NETs) and liver metastases (LMs), but their relevance is questioned in this setting. We aimed to explore alternative criteria using different numbers of measured LMs and thresholds of size and density variation.

Second surgery after vertical paramedian hemispherotomy for epilepsy recurrence.

Background: Vertical Paramedian Hemispherotomy (VPH) is considered an effective surgical treatment for drug-resistant epilepsy with 80% of patients experiencing seizure freedom or worthwhile improvement. Identifying persistent connective tracts is challenging in failed VPH.

Methods: We reviewed our series of consecutive patients undergoing VPH for hemispheric drug-resistant epilepsy and included cases with recurrent epileptic seizures undergoing second surgery with at least 6 months of postoperative follow-up.

An International Perspective on Preceding Infections in Guillain-Barré Syndrome: The IGOS-1000 Cohort.

Background And Objectives: Infections play a key role in the development of Guillain-Barré syndrome (GBS) and have been associated with specific clinical features and disease severity. The clinical variation of GBS across geographical regions has been suggested to be related to differences in the distribution of preceding infections, but this has not been studied on a large scale.

Methods: We analyzed the first 1,000 patients included in the International GBS Outcome Study with available biosamples (n = 768) for the presence of a recent infection with , hepatitis E virus, , cytomegalovirus, and Epstein-Barr virus.

Duodenal CD8+ T resident memory cell apoptosis contributes to gut barrier dysfunction and microbial translocation in early alcohol-associated liver disease in humans.

Background: Intestinal T cells are key in gut barrier function. Their role in early stages of alcohol-associated liver disease (ALD) remain unknown.

Aim: To explore the links between intestinal T cells, microbial translocation and ALD METHODS: Patients with alcohol use disorder (AUD) following a rehabilitation programme were compared to subjects with non-alcoholic fatty liver disease (NAFLD) and healthy controls.

MRI-guided DBS of STN under general anesthesia for Parkinson's disease: results and microlesion effect analysis.

Background: The efficacy of the subthalamic nucleus (STN) stimulation for Parkinson's disease has been widely established. The microlesion effect (MLE) due to deep brain stimulation (DBS) electrode implantation has been reputed to be a good predictor for long-term efficacy of the procedure but its analysis in asleep implantation is still unclear. We thus analyzed MLE rate in our strategy of targeting the STN on MRI under general anesthesia and its correlation with our long-term results.

Electrodiagnostic subtyping in Guillain-Barré syndrome: Use of criteria in practice based on a survey study in IGOS.

Electrodiagnostic (EDx) studies are helpful in diagnosing and subtyping of Guillain-Barré syndrome (GBS). Published criteria for differentiation into GBS subtypes focus on cutoff values, but other items receive less attention, although they may influence EDx subtyping: (a) extensiveness of EDx testing, (b) nerve-specific considerations, (c) distal compound muscle action potential (CMAP)-amplitude requirements, (d) criteria for conduction block and temporal dispersion. The aims of this study were to investigate how these aspects were approached by neuromuscular EDx experts in practice and how this was done in previously published EDx criteria for GBS.

European Academy of Neurology guidance for developing and reporting clinical practice guidelines on rare neurological diseases.

Background And Purpose: Rare diseases affect up to 29 million people in the European Union, and almost 50% of them affect the nervous system or muscles. Delays in diagnosis and treatment onset and insufficient treatment choices are common. Clinical practice guidelines (CPGs) may improve the diagnosis and treatment of patients and optimize care pathways, delivering the best scientific evidence to all clinicians treating these patients.

Electrodiagnosis of Guillain-Barre syndrome in the International GBS Outcome Study: Differences in methods and reference values.

Objective: To describe the heterogeneity of electrodiagnostic (EDx) studies in Guillain-Barré syndrome (GBS) patients collected as part of the International GBS Outcome Study (IGOS).

Methods: Prospectively collected clinical and EDx data were available in 957 IGOS patients from 115 centers. Only the first EDx study was included in the current analysis.

Enhancing Safety in Epilepsy Surgery (EASINESS): Study Protocol for a Retrospective, Multicenter, Open Registry.

Optimizing patient safety and quality improvement is increasingly important in surgery. Benchmarks and clinical quality registries are being developed to assess the best achievable results for several surgical procedures and reduce unwarranted variation between different centers. However, there is no clinical database from international centers for establishing standardized reference values of patients undergoing surgery for mesial temporal lobe epilepsy.

ECHDC1 knockout mice accumulate ethyl-branched lipids and excrete abnormal intermediates of branched-chain fatty acid metabolism.

The cytosolic enzyme ethylmalonyl-CoA decarboxylase (ECHDC1) decarboxylates ethyl- or methyl-malonyl-CoA, two side products of acetyl-CoA carboxylase. These CoA derivatives can be used to synthesize a subset of branched-chain fatty acids (FAs). We previously found that ECHDC1 limits the synthesis of these abnormal FAs in cell lines, but its effects in vivo are unknown.

Joint Belgian recommendation on screening for DPD-deficiency in patients treated with 5-FU, capecitabine (and tegafur).

Objectives: Fluoropyrimidines such as 5-Fluorouracil (5-FU), capecitabine and tegafur are drugs that are often used in the treatment of maliginancies. The enzyme dihydropyrimidine dehydrogenase (DPD) is the first and rate limiting enzyme of 5-FU catabolism. Genetic variations within the DPYD gene (encoding for DPD protein) can lead to reduced or absent DPD activity.

Irradiation level related to intraoperative imaging device in paediatric elastic stable intramedullary nailing: preliminary prospective study on 51 patients using PCXMC software.

Purpose: Radiation-induced cancers due to imaging devices concern above all the growing child, however, to date, intraoperative irradiation doses are not well-documented in children. The goal of the study was to evaluate the intraoperative doses received by patients operated with the use of a C-arm in traumatology, as well as the lifetime attributable risk of cancer death (LAR) related to the irradiation of the imaging device.

Methods: From 1 April 2017 to 31 March 2019, we started a multicentre study and prospectively recruited all consecutive children who needed elastic stable intramedullary nailing (ESIN) for long-bone fracture.

Anatomical study of the descending genicular artery and implications for image-guided interventions for knee pain.

Introduction: The descending genicular artery (DGA) has recently been mentioned as accompanying some nerves in the medial aspect of the knee joint. This could be clinically relevant as the arteries could serve as landmarks for accurate nerve capture during ultrasound-guided nerve blockade or ablation. The aim of this cadaveric study was to investigate the anatomical distribution of the DGA, assess the nerves running alongside its branches, and discuss the implications for regional anesthesia and knee pain interventions.

Tonic, Burst, High-Density, and 10-kHz High-Frequency Spinal Cord Stimulation: Efficiency and Patients' Preferences in a Failed Back Surgery Syndrome Predominant Population. Review of Literature.

Background: Spinal cord stimulation (SCS) is a neuromodulation process to treat neuropathic pain, initially developed on tonic paresthesia-based stimulation. In the last decade, 3 major paresthesia-free SCSs have emerged. Several studies show their superiority over tonic stimulation.

The Pulmonary Autograft After the Ross Operation: Results of 25-Year Follow-Up in a Pediatric Cohort.

Background: Progressive autograft dilation and need for later reoperation remain major concerns of the Ross procedure. The study investigates the clinical outcome after the Ross operation, including a longitudinal analysis of autograft dimensions over 25 years.

Methods: From November 1991 to April 2019, 137 patients underwent a Ross procedure at the University Hospitals of UCL (Université catholique de Louvain)-Brussels and Ghent.

The role of the radiation oncologist in quality and patient safety: A proposal of indicators and metrics.

This manuscript represents a collaboration from an international group of quality and safety expert radiation oncologists. It is a position/review paper with the specific aim of defining the role of the radiation oncologist in quality and safety management. This manuscript is unique in that we recommend specific quality assurance/control tasks and correlated quality and indicators and safety measures that are the responsibility of the radiation oncologist.

Time to Diagnosis in Cushing's Syndrome: A Meta-Analysis Based on 5367 Patients.

Context: Signs and symptoms of Cushing's syndrome (CS) overlap with common diseases, such as the metabolic syndrome, obesity, osteoporosis, and depression. Therefore, it can take years to finally diagnose CS, although early diagnosis is important for prevention of complications.

Objective: The aim of this study was to assess the time span between first symptoms and diagnosis of CS in different populations to identify factors associated with an early diagnosis.

Current treatment practice of Guillain-Barré syndrome.

Objective: To define the current treatment practice of Guillain-Barré syndrome (GBS).

Methods: The study was based on prospective observational data from the first 1,300 patients included in the International GBS Outcome Study. We described the treatment practice of GBS in general, and for (1) severe forms (unable to walk independently), (2) no recovery after initial treatment, (3) treatment-related fluctuations, (4) mild forms (able to walk independently), and (5) variant forms including Miller Fisher syndrome, taking patient characteristics and hospital type into account.