Paraneoplastic neurological syndromes (PNSs) are group of disorders affecting one or multiple parts of the neuroaxis associated with underlying tumors. An antibody or autoantigen-specific cell-mediated immune response against neural antigen expressed in the tumor is the potential etiology for these rare but refractory disorders. In recent years, wide variety of neurological presentations and autoantibodies has been associated with paraneoplastic autoimmunity, leading to formulation of an updated expert consensus PNS diagnostic criteria. Recognition of these phenotypes and use of serological biomarkers may aid neurologists in early diagnosis of PNS cases encountered in the inpatient or outpatient practice. In this review article, we provide an overview of various clinical, radiological, and immunopathological characteristics of PNS. Furthermore, we discuss the updated PNS criteria and increasing recognition of neurological presentations resembling the PNS among patients receiving immune checkpoint inhibitors.
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http://dx.doi.org/10.1007/s10072-022-06083-y | DOI Listing |
Mult Scler
January 2025
Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA.
Background: Spinal cord (SC) atrophy is a key imaging biomarker of progressive multiple sclerosis (MS). Progressive MS is more common in men and postmenopausal women.
Objective: Investigate the impact of sex and menopause on SC measurements in persons with MS (pwMS).
Neurol Neuroimmunol Neuroinflamm
March 2025
MeLis Institute, SynatAc Team, Inserm U1314/ UMR CNRS5284, France.
Background And Objectives: Breast cancers (BCs) of patients with paraneoplastic neurologic syndromes and anti-Yo antibodies (Yo-PNS) overexpress human epidermal growth factor receptor 2 (HER2) and display genetic alterations and overexpression of the Yo-onconeural antigens. They are infiltrated by an unusual proportion of B cells. We investigated whether these features were also observed in patients with PNS and anti-Ri antibodies (Ri-PNS).
View Article and Find Full Text PDFJ Int Med Res
January 2025
Department of Gastroenterology & Hepatology, Dazhou Integrated TCM and Western Medicine Hospital: Dazhou Second People's Hospital, Dazhou, Sichuan Province, China.
Paraneoplastic chronic intestinal pseudo-obstruction (CIPO) is a rare manifestation associated with small cell lung carcinoma and other malignancies. In this report, we present a challenging case involving a 60-year-old man with small cell lung carcinoma who developed symptoms of intestinal pseudo-obstruction, including abdominal distention, pain, and obstipation. Despite receiving extensive treatment with intravenous gamma globulin and glucocorticoids, the patient's symptoms did not improve, ultimately resulting in irreversible nervous system damage.
View Article and Find Full Text PDFJ Neurol
January 2025
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Objectives: To report myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) epidemiology in two American regions using 2023 diagnostic criteria.
Patients And Methods: We compared age- and sex-adjusted incidence and prevalence of MOGAD per 2023 diagnostic criteria in Olmsted County (Minnesota [USA]) and Martinique (Caribbean [FR]) (01/01/2003-12/31/2018, prevalence day) using Poisson regression. Archived sera in 68-85% were available for MOG-IgG testing by live cell-based assay at Mayo Clinic.
Mov Disord Clin Pract
January 2025
The Edmond J. Safra Program in Parkinson's Disease, University Health Network and the University of Toronto, Toronto, Ontario, Canada.
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