Paraneoplastic neurological syndromes: a single institution 10-year case series.

Abstract

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.