Manganese overload as a co-factor of neurological symptoms in a patient with sclerosing cholangitis due to Langerhans cell histiocytosis.

Not available.

Improving Examination Skills in Neuromuscular Disorders Through an Educational Video.

Background: The acquisition of practical skills is a key objective of medical education. Improving knowledge and skills is essential for early diagnosis of patients suffering from neuromuscular (NM) diseases.

Approach: Multimedia tools have proved to be useful and effective for learning clinical skills.

Real life data of ONC201 (dordaviprone) in pediatric and adult H3K27-altered recurrent diffuse midline glioma: Results of an international academia-driven compassionate use program.

Introduction: H3K27-altered diffuse midline gliomas (DMG) have limited therapeutic options and a very poor prognosis. Encouraging responses were observed in early clinical trials with ONC201. As ONC201 was unavailable in Europe, a compassionate use program supported by the French Authorities was launched for patients at progression after standard of care radiotherapy.

PARP inhibitors in gliomas: Mechanisms of action, current trends and future perspectives.

Gliomas are the most common primary malignant brain tumours in adults. Despite decades of research into novel therapeutic approaches, the prognosis remains poor. PARP1-2 are critical for DNA repair, cell survival and genomic stability and PARP inhibition (PARPi) may be a promising therapeutic approach for gliomas.

Comparison of Dual Monoclonal Antibody Therapies for COVID-19 Evolution: A Multicentric Retrospective Study.

Background: Neutralizing antibodies targeting the SARS-CoV-2 Spike protein reduce COVID-19-related risk of hospitalization, particularly in high-risk individuals. The COCOPREV-R study aimed to evaluate and compare clinical outcomes in high-risk SARS-CoV-2 patients treated with dual monoclonal antibody therapies and to identify associated virological factors.

Methods: The COCOPREV-R study retrospectively collected real-world data from high-risk patients receiving Bamlanivimab/Etesevimab or Casirivimab/Imdevimab dual monoclonal antibody therapies (22 February 2021 to 15 June 2021).

Central nervous system tumors in adolescents and young adults: A Society for Neuro-Oncology Consensus Review on diagnosis, management, and future directions.

Adolescents and young adults (AYAs; ages 15-39 years) are a vulnerable population facing challenges in oncological care, including access to specialized care, transition of care, unique tumor biology, and poor representation in clinical trials. Brain tumors are the second most common tumor type in AYA, with malignant brain tumors being the most common cause of cancer-related death. The 2021 WHO Classification for central nervous system (CNS) Tumors highlights the importance of integrated molecular characterization with histologic diagnosis in several tumors relevant to the AYA population.

Reappraisal of prognostic factors in CNS WHO grade 3 oligodendrogliomas IDH-mutant and 1p/19q co-deleted: lessons from the French POLA cohort.

Background: In POLA cohort, three pathological groups of CNS WHO grade 3 oligodendroglioma IDH-mutant and 1p/19q co-deleted have been described: group 1 (high mitotic count only), group 2 (microvascular proliferation MVP and no necrosis), and group 3 (MVP and necrosis).

Methods: 494 patients from the POLA cohort, with a median follow up of 96 months were included. To identify the impact of the pathological groups and contrast enhancement in group 1 on overall survival (OS) or progression free survival (PFS), survival curves were obtained (Kaplan-Meier method) and compared (log-rank test).

Radiomics-driven personalized radiotherapy for primary and recurrent tumors: A general review with a focus on reirradiation.

Purpose: This review systematically investigates the role of radiomics in radiotherapy, with a particular emphasis on the use of quantitative imaging biomarkers for predicting clinical outcomes, assessing toxicity, and optimizing treatment planning. While the review encompasses various applications of radiomics in radiotherapy, it particularly highlights its potential for guiding reirradiation of recurrent cancers.

Methods: A systematic review was conducted based on a Medline search with the search engine PubMed using the keywords "radiomics or radiomic" and "radiotherapy or reirradiation".

Updated EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection - Update 1.

The standard of care for adult patients with gliomas, glioneuronal and neuronal tumors consists of combinations of surgery, radiotherapy, and chemotherapy. For many systemic cancers, targeted treatments are a major part of the standard treatment, however, the predictive significance of most of the targets for treatment in systemic cancer are less well established in central nervous system (CNS) tumors . In 2023 the EANO Guideline Committee presented evidence based recommendations for rational testing of molecular targets for targeted treatments.

Systemic treatments in recurrent or metastatic salivary gland cancer: a systematic review.

Background: Salivary gland cancers are infrequent and pose a challenge owing to their histological diversity and varied clinical behavior, making the selection of optimal systemic treatments for advanced or recurrent stages difficult. This systematic review aims to assess overall survival outcomes and systemic treatment responses across four types of salivary cancers.

Methods: A PubMed and Google Scholar search identified studies involving initially advanced or relapsed cases undergoing systemic treatment.

Survival Outcomes Associated With First-Line Procarbazine, CCNU, and Vincristine or Temozolomide in Combination With Radiotherapy in IDH-Mutant 1p/19q-Codeleted Grade 3 Oligodendroglioma.

Purpose: Patients with IDH-mutant 1p/19q-codeleted grade 3 oligodendroglioma (O3) benefit from adding alkylating agent chemotherapy to radiotherapy (RT). However, the optimal chemotherapy regimen between procarbazine, 1-(2-Chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), and vincristine (PCV) and temozolomide (TMZ) remains unclear given the lack of randomized trial data comparing both regimens.

Methods: The objective was to assess the overall survival (OS) and progression-free survival (PFS) associated with first-line PCV/RT versus TMZ/RT in patients newly diagnosed with O3.

Poorer clinical outcomes after THA in patients with a spinal scoliotic deformity: a case-control study of 268 patients assessed with PROMS.

Introduction: Spinal deformities can lead to specific complications after total hip arthroplasty (THA), such as functional leg length discrepancy due to a fixed pelvic obliquity, as well as an increased risk of prosthetic instability due to a lack of adaptive pelvic mobility, but these issues were not investigated in large comparative series. Therefore a retrospective case-control study was done aiming: 1) to analyze the impact of a preoperative scoliotic deformity on the functional outcomes of patients who underwent THA with a minimum 1-year follow-up, 2) to measure the prevalence of scoliosis in both the case and control groups 3) to screen other factors that may be correlated with poorer clinical outcomes in patients who underwent THA, including age, gender, Body Mass index (BMI), American Society of Anesthesiologists (ASA) score, primary THA etiology and postoperative complication occurrence.

Hypothesis: The presence of scoliosis would have a negative impact on THA outcomes as assessed by PROMs.

Achievements of international rare cancers networks and consortia in the neuro-oncology field.

Purpose Of Review: In this review, we investigated the role of European oncological networks on management and care of patients with central nervous system (CNS) malignancies.

Recent Findings: Within this universe of tumors, malignancies of the central nervous system (CNS) malignancies represent a challenge because of several reasons such as biological complexity, the need of dedicated experienced physicians (surgeons, pathologists, radiologists and neuro-oncologists) and tertiary healthcare providers. Limits to the development of effective and innovative care are represented by the rarity of these tumors and their extreme heterogeneity in terms of clinical presentation, course of the disease, genetic assessments and site of presentation.

[Lipoprotein-associated phospholipase A (Lp-PLA): Relevant biomarker and therapeutic target?].

Over the last fifteen years, numerous studies have sought to decipher the role of lipoprotein-associated phospholipase A (Lp-PLA) in vascular inflammation-related diseases, notably atherosclerosis. Despite the disappointing results of clinical trials using the Lp-PLA inhibitor darapladib, new pathophysiological, epidemiological and genetic data have enabled the development of new inhibitors. Recent studies also show that Lp-PLA is involved in vascular inflammation-related diseases other than atherosclerosis (ischemic stroke, Alzheimer's disease and vascular dementia, diabetes, cancers…), and inhibition of Lp-PLA could have beneficial therapeutic in these diseases.

Innate immune response in acute critical illness: a narrative review.

Background: Activation of innate immunity is a first line of host defense during acute critical illness (ACI) that aims to contain injury and avoid tissue damages. Aberrant activation of innate immunity may also participate in the occurrence of organ failures during critical illness. This review aims to provide a narrative overview of recent advances in the field of innate immunity in critical illness, and to consider future potential therapeutic strategies.