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

  • Clinical Study
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Journal of Neuro-Oncology Aims and scope Submit manuscript

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.

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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

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Authors and Affiliations

  1. Department of Neurology, Yale School of Medicine, 15 York Street, LLCI 9th floor, New Haven, CT, 06510, USA

    Amy M. Chan & Joachim M. Baehring

  2. Department of Neurosurgery, Yale School of Medicine, New Haven, CT, USA

    Joachim M. Baehring

Authors
  1. Amy M. Chan
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  2. Joachim M. Baehring
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Corresponding author

Correspondence to Joachim M. Baehring.

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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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Cite this article

Chan, A.M., Baehring, J.M. Paraneoplastic neurological syndromes: a single institution 10-year case series. J Neurooncol 141, 431–439 (2019). https://doi.org/10.1007/s11060-018-03053-3

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  • DOI: https://doi.org/10.1007/s11060-018-03053-3

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