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Journal of Neuro-Oncology

, Volume 141, Issue 2, pp 431–439 | Cite as

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

  • Amy M. Chan
  • Joachim M. BaehringEmail author
Clinical Study

Abstract

Background

Given its rare incidence, there are few epidemiological case series on paraneoplastic neurologic syndromes (PNS).

Methods

We present a 10-year series compiled in the Section of Neuro-Oncology, Yale Cancer Center between 2002 and 2012.

Results

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.

Conclusions

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.

Keywords

Paraneoplastic Neurologic Syndrome Presentation Treatment Outcome 

Abbreviations

PNS

Paraneoplastic syndrome

ONA

Onconeural antibodies

CSF

Cerebro spinal fluid

LE

Limbic encephalitis

SCD

Subacute cerebellar degeneration

SSN

Sensory neuronopathy

OM

Opsoclonus–myoclonus

EM

Encephalomyelitis

BE

Brainstem encephalitis

LEMS

Lambert–Eaton myasthenic syndrome

RN

Retinopathy

SCLC

Small cell lung cancer

mRS

modified Rankin Scale

MePRDL

Methylprednisolone

IVIG

Intravenous immunoglobulin

PLEX

Plasma exchange

NSCLC

Non-small cell lung cancer

Notes

Compliance with ethical standards

Conflict of interest

All authors declare that they have no conflict of interest.

Ethical approval

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

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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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of NeurologyYale School of MedicineNew HavenUSA
  2. 2.Department of NeurosurgeryYale School of MedicineNew HavenUSA

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