Cargando…

Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment

Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associa...

Descripción completa

Detalles Bibliográficos
Autores principales: Marsili, Luca, Marcucci, Samuel, LaPorta, Joseph, Chirra, Martina, Espay, Alberto J., Colosimo, Carlo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10216177/
https://www.ncbi.nlm.nih.gov/pubmed/37239077
http://dx.doi.org/10.3390/biomedicines11051406
_version_ 1785048235810750464
author Marsili, Luca
Marcucci, Samuel
LaPorta, Joseph
Chirra, Martina
Espay, Alberto J.
Colosimo, Carlo
author_facet Marsili, Luca
Marcucci, Samuel
LaPorta, Joseph
Chirra, Martina
Espay, Alberto J.
Colosimo, Carlo
author_sort Marsili, Luca
collection PubMed
description Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associated with antibodies against neural surface antigens, known as “intermediate- or low-risk” antibodies, are less frequently associated with cancer. In this narrative review, we will focus on PNS of the central nervous system (CNS). Clinicians should have a high index of suspicion with acute/subacute encephalopathies to achieve a prompt diagnosis and treatment. PNS of the CNS exhibit a range of overlapping “high-risk” clinical syndromes, including but not limited to latent and overt rapidly progressive cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome, paraneoplastic (and limbic) encephalitis/encephalomyelitis, and stiff-person spectrum disorders. Some of these phenotypes may also arise from recent anti-cancer treatments, namely immune-checkpoint inhibitors and CAR T-cell therapies, as a consequence of boosting of the immune system against cancer cells. Here, we highlight the clinical features of PNS of the CNS, their associated tumors and antibodies, and the diagnostic and therapeutic strategies. The potential and the advance of this review consists on a broad description on how the field of PNS of the CNS is constantly expanding with newly discovered antibodies and syndromes. Standardized diagnostic criteria and disease biomarkers are fundamental to quickly recognize PNS to allow prompt treatment initiation, thus improving the long-term outcome of these conditions.
format Online
Article
Text
id pubmed-10216177
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-102161772023-05-27 Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment Marsili, Luca Marcucci, Samuel LaPorta, Joseph Chirra, Martina Espay, Alberto J. Colosimo, Carlo Biomedicines Review Paraneoplastic neurological syndromes (PNS) include any symptomatic and non-metastatic neurological manifestations associated with a neoplasm. PNS associated with antibodies against intracellular antigens, known as “high-risk” antibodies, show frequent association with underlying cancer. PNS associated with antibodies against neural surface antigens, known as “intermediate- or low-risk” antibodies, are less frequently associated with cancer. In this narrative review, we will focus on PNS of the central nervous system (CNS). Clinicians should have a high index of suspicion with acute/subacute encephalopathies to achieve a prompt diagnosis and treatment. PNS of the CNS exhibit a range of overlapping “high-risk” clinical syndromes, including but not limited to latent and overt rapidly progressive cerebellar syndrome, opsoclonus-myoclonus-ataxia syndrome, paraneoplastic (and limbic) encephalitis/encephalomyelitis, and stiff-person spectrum disorders. Some of these phenotypes may also arise from recent anti-cancer treatments, namely immune-checkpoint inhibitors and CAR T-cell therapies, as a consequence of boosting of the immune system against cancer cells. Here, we highlight the clinical features of PNS of the CNS, their associated tumors and antibodies, and the diagnostic and therapeutic strategies. The potential and the advance of this review consists on a broad description on how the field of PNS of the CNS is constantly expanding with newly discovered antibodies and syndromes. Standardized diagnostic criteria and disease biomarkers are fundamental to quickly recognize PNS to allow prompt treatment initiation, thus improving the long-term outcome of these conditions. MDPI 2023-05-09 /pmc/articles/PMC10216177/ /pubmed/37239077 http://dx.doi.org/10.3390/biomedicines11051406 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Marsili, Luca
Marcucci, Samuel
LaPorta, Joseph
Chirra, Martina
Espay, Alberto J.
Colosimo, Carlo
Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment
title Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment
title_full Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment
title_fullStr Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment
title_full_unstemmed Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment
title_short Paraneoplastic Neurological Syndromes of the Central Nervous System: Pathophysiology, Diagnosis, and Treatment
title_sort paraneoplastic neurological syndromes of the central nervous system: pathophysiology, diagnosis, and treatment
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10216177/
https://www.ncbi.nlm.nih.gov/pubmed/37239077
http://dx.doi.org/10.3390/biomedicines11051406
work_keys_str_mv AT marsililuca paraneoplasticneurologicalsyndromesofthecentralnervoussystempathophysiologydiagnosisandtreatment
AT marcuccisamuel paraneoplasticneurologicalsyndromesofthecentralnervoussystempathophysiologydiagnosisandtreatment
AT laportajoseph paraneoplasticneurologicalsyndromesofthecentralnervoussystempathophysiologydiagnosisandtreatment
AT chirramartina paraneoplasticneurologicalsyndromesofthecentralnervoussystempathophysiologydiagnosisandtreatment
AT espayalbertoj paraneoplasticneurologicalsyndromesofthecentralnervoussystempathophysiologydiagnosisandtreatment
AT colosimocarlo paraneoplasticneurologicalsyndromesofthecentralnervoussystempathophysiologydiagnosisandtreatment