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J Neurooncol. 2019 Jan;141(2):431-439. doi: 10.1007/s11060-018-03053-3. Epub 2019 Jan 3.

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

Author information

1
Department of Neurology, Yale School of Medicine, 15 York Street, LLCI 9th floor, New Haven, CT, 06510, USA.
2
Department of Neurology, Yale School of Medicine, 15 York Street, LLCI 9th floor, New Haven, CT, 06510, USA. joachim.baehring@yale.edu.
3
Department of Neurosurgery, Yale School of Medicine, New Haven, CT, USA. joachim.baehring@yale.edu.

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:

Neurologic; Outcome; Paraneoplastic; Presentation; Syndrome; Treatment

PMID:
30607708
DOI:
10.1007/s11060-018-03053-3
[Indexed for MEDLINE]

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