Paraneoplastic neurological syndromes: a single institution 10-year case series
Given its rare incidence, there are few epidemiological case series on paraneoplastic neurologic syndromes (PNS).
We present a 10-year series compiled in the Section of Neuro-Oncology, Yale Cancer Center between 2002 and 2012.
Twenty-five cases met the PNS Euro-network criteria for definitive PNS. Most (64%; 16/25) had no known neoplasm. Cerebrospinal fluid pleocytosis declined logarithmically over time. Neuroimaging abnormalities were seen in 88% of cases (15/17), but with delayed onset. Therapeutic benefit correlated strongly to pre-treatment modified Rankin Scale (mRS) (p < 0.01), but not with time elapsed between syndrome onset to treatment (p = 0.8), first immunotherapy modality (corticosteroids: n = 10; IVIG: n = 10; PLEX: n = 3; p = 0.37), or number of immunotherapy modalities provided (p = 0.17). PNS-related mortality was high (24%; 6/25). Nonetheless, 16% (3/18; 7 living patients censored) survived over 6 times the anticipated median expected by tumor type and stage.
PNS are rare, at an estimated incidence of 3.1 cases per million-person-years. Detection of CSF pleocytosis and MRI abnormalities depend on time of analysis. While PNS-related mortality was high, immunotherapy benefit correlated strongly with pre-treatment mRS and long-term survival is possible.
KeywordsParaneoplastic Neurologic Syndrome Presentation Treatment Outcome
Cerebro spinal fluid
Subacute cerebellar degeneration
Lambert–Eaton myasthenic syndrome
Small cell lung cancer
modified Rankin Scale
Non-small cell lung cancer
Compliance with ethical standards
Conflict of interest
All authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the Ethical Standards of the Institutional and/or National Research Committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
Informed consent requirement was waived by the Institutional Review Board (minimal risk; majority of patients had died at the time the study was initiated).
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