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Skeletal abnormalities caused by a Connexin43 mutation in a mouse model for autosomal recessive craniometaphyseal dysplasia.
Bone Res
January 2025
Department of Endodontology, School of Dental Medicine, University of Connecticut Health, Farmington, CT, USA.
Craniometaphyseal dysplasia (CMD), a rare craniotubular disorder, occurs in an autosomal dominant (AD) or autosomal recessive (AR) form. CMD is characterized by hyperostosis of craniofacial bones and metaphyseal flaring of long bones. Many patients with CMD suffer from neurological symptoms.
View Article and Find Full Text PDFPOEMS Syndrome.
Presse Med
January 2025
Department of Hematology and Cellular Therapy, National Reference Center "AL Amyloidosis and Other Monoclonal Immunoglobulin Deposit Diseases, University Hospital of Limoges, Limoges, France.
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, Skin changes) is a syndrome that involves a monoclonal B-cell proliferation, most often plasmacytic, and a variable number of manifestations listed or not in the acronym. These manifestations include sclerotic bone lesions, plasmacytic Castleman disease, papillary edema, peripheral edema, ascites, thrombocytosis and/or polycythemia, venous and/or arterial thrombosis, and renal, pulmonary, and cardiac impairments . Diagnosis is often delayed due to the rarity of this entity and its clinical polymorphism, which can mimic other neurological disorders.
View Article and Find Full Text PDFProlonged neurologic symptoms following immune effector cell-associated neurotoxicity syndrome in patients with large B cell lymphoma treated with chimeric antigen receptor-modified T cell therapy.
Transplant Cell Ther
January 2025
Abramson Cancer Center, University of Pennsylvania, Philadelphia, Pennsylvania.
Background: While immune effector cell-associated neurotoxicity syndrome (ICANS) is a well-defined adverse effect associated with chimeric antigen receptor-modified T cell (CAR-T) therapy, some patients develop prolonged neurologic symptoms. Few studies have examined characteristics and outcomes of patients who develop such symptoms.
Objective: To provide an analysis of patients who developed ICANS in a single-center cohort of patients with large B-cell lymphoma (LBCL) who received commercial CAR-T and compare characteristics and outcomes between patients with vs.
Toluene Toxicity in the Brain: From Cellular Targets to Molecular Mechanisms.
Annu Rev Pharmacol Toxicol
January 2025
Department of Pharmacology, Addiction Science, and Toxicology, College of Medicine, The University of Tennessee Health Science Center, Memphis, Tennessee, USA; email:
Toluene intoxication constitutes a persistent public health problem worldwide. While most organs can be damaged, the brain is a primary target whether exposure is accidental, occupational, or recreational. Interventions to prevent/revert brain damage by toluene are curtailed by the scarce information on the molecular targets and mechanisms mediating toluene's brain toxicity and the common exposure to other neurotoxins and/or coexistence of neurological/psychiatric disorders.
View Article and Find Full Text PDFAll-Cause and Cause-Specific Mortality in Children With Congenital Zika Syndrome in Brazil.
JAMA Netw Open
January 2025
Center of Data and Knowledge Integration for Health, Gonçalo Moniz Institute, Oswaldo Cruz Foundation, Salvador, Bahia, Brazil.
Importance: Congenital Zika syndrome (CZS) can lead to a range of developmental and neurological issues, which increases the risk of early death. However, the all-cause and cause-specific mortality in children with CZS in the first 5 years of life remain unknown.
Objective: To compare the hazard of all-cause and cause-specific mortality before age 5 years among children with and without CZS in Brazil.
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