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.
Background:
Retinal artery occlusion (RAO) is a stroke equivalent that causes significant morbidity. There has been growing emphasis on urgent in-hospital evaluation of these patients, both to facilitate potential thrombolytic therapy and expedite workup; however, little is known regarding its effect on systems of care. We thus examined presenting characteristics and management of hospitalized RAO patients using the Greater Cincinnati Northern Kentucky Stroke Study (GCNKSS).
Methods:
The GCNKSS is a population-based study of stroke in a 5-county region with a population of 1.3 million representative of the USA in terms of Black race, income, and education. All cases of RAO among Black individuals from July 2019-December 2020 and among White individuals from 2020 were chart abstracted using ICD codes. All cases underwent physician adjudication. Demographic and clinical data were recorded.
Results:
We identified 57 hospitalizations with acute RAO among 55 patients. Characteristics of their hospitalization and demographics are shown (Table). Notably, 19% (11/57) of patients presented in a thrombolytic window of ≤3.5 and average time from symptom onset to evaluation was 17 hours. Most patients initially interacted with a subspecialist (53%) and presented to the ED in delayed fashion. One patient received thrombolytic therapy, four (7%) patients underwent carotid revascularization, and no patients had newly identified atrial fibrillation, cardiac thrombus, or endocarditis.
Discussion:
Our population-based study found only a minority of patients presented within a thrombolytic window, suggesting that systems of care need to promote more rapid evaluation of these patients. Very few patients received select intervention, but the high impact of carotid revascularization may warrant urgent evaluation of even low risk patients. Further study of long-term outcomes in this patient population is called for.
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