Paraneoplastic Neurological Syndromes: A Single Institution 10-Year Case Series (P7.016)

OBJECTIVE: To summarize the incidence, presentation, sensitivities of diagnostic procedures, treatments and outcome of PNS patients seen at Yale New Haven Hospital, a tertiary referral center for Southern Connecticut, over the last 10 years. BACKGROUND: Recognition of paraneoplastic neurological syndromes (PNS) can lead to early immune-modulating therapy and neoplasm diagnosis. Given low occurrence, there are few single institution case series compiling epidemiological data, clinical presentations and outcomes. DESIGN/METHODS: Retrospective case review of all neuro-oncology consults from 2003 - 2013 with diagnostic code ICD 323.9 (unspecified causes of encephalitis, myelitis and encephalomyelitis) or PNS. Clinical presentation, time to diagnosis, investigation results including MRI, PET, CSF analysis and outcomes were compiled. RESULTS: 27 cases met the PNS Euronetwork criteria. The most common clinical syndromes were limbic encephalitis (9/27), sub-acute cerebellar degeneration (5), sensory neuronopathy (5), retinopathy (4), brainstem encephalitis (3), opsoclonus-myoclonus (2) and myasthenia (2). Most cases (18/27) had no known neoplasm. Neoplasm was found in 17/18 cases within median of 2 months from symptom onset. New metastatic disease was found in 5/9 cases within median of 6 months. 2 cases were lost to follow-up. Mortality rate was high (11/25), from both neoplasm progression (5/11) and complications from PNS (6/11). Most frequent onconeural antibodies were: Anti-Hu (5), Anti-Yo (3), Anti-NDMA (2), Anti-Ri (2), Anti-CV2 (1), Anti-Ma1/Anti-Ma2 (1), and anti-PQ calcium channel (1). Survival data based on Kaplan Meier survival estimates, predictors of survival based on Cox proportional hazards model, sensitivity of diagnostic procedures, and incidence of PNS in our population will be presented. CONCLUSIONS: PNS is a rare complication of cancer. Early recognition is of utmost importance to prevent devastating neurologic disability in patients who may have more favorable long-term survival from their cancer. While therapeutic guidelines from prospective clinical trials are missing, empiric treatment algorithms should be followed. Study Supported by: Disclosure: Dr. Chan has nothing to disclose. Dr. Baehring has received personal compensation for activities with Sigma Tau and Roche Diagnostics Corporation.