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November 18, 2020ArticleOpen Access

Paraneoplastic myeloneuropathies: Clinical, oncologic and serologic accompaniments

View ORCID ProfileShailee Shah, View ORCID ProfileRocio Vazquez Do Campo, Neeraj Kumar, Andrew McKeon, Eoin P. Flanagan, Christopher Klein, Sean J. Pittock, Divyanshu Dubey
First published November 18, 2020, DOI: https://doi.org/10.1212/WNL.0000000000011218
Shailee Shah
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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  • ORCID record for Shailee Shah
Rocio Vazquez Do Campo
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Neeraj Kumar
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Andrew McKeon
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
2Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Eoin P. Flanagan
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
2Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Christopher Klein
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
2Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Sean J. Pittock
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
2Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Divyanshu Dubey
1Departments of Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
2Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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Paraneoplastic myeloneuropathies: Clinical, oncologic and serologic accompaniments
Shailee Shah, Rocio Vazquez Do Campo, Neeraj Kumar, Andrew McKeon, Eoin P. Flanagan, Christopher Klein, Sean J. Pittock, Divyanshu Dubey
Neurology Nov 2020, 10.1212/WNL.0000000000011218; DOI: 10.1212/WNL.0000000000011218

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Abstract

Objective: To test the hypothesis that myeloneuropathy is a presenting phenotype of paraneoplastic neurological syndromes, we retrospectively reviewed clinical, radiological and serological features of 32 patients with concomitant paraneoplastic spinal cord and peripheral nervous system involvements.

Methods: Observational study investigating patients with myeloneuropathy and underlying cancer and/or onconeural antibody seropositivity.

Results: Among 32 paraneoplastic myeloneuropathy patients, twenty (63%) were women with median age 61 years (range 27-84 years). Twenty-six patients (81%) had classified onconeural antibodies (amphiphysin, n=8; ANNA1 [anti-Hu] n=5; CRMP5 [anti-CV2], n=6; PCA1 [anti-Yo], n=1; PCA2, n=2; KLHL11, n=1; and combinations thereof: ANNA1/CRMP5, n=1; ANNA1/amphiphysin, n=1; ANNA3/CRMP5, n=1). Cancer was confirmed in twenty-five cases (onconeural antibodies, n=19; unclassified antibodies, n=3; no antibodies, n=3). Paraneoplastic myeloneuropathies had asymmetric paresthesias (84%), neuropathic pain (78%), subacute onset (72%) sensory ataxia (69%), bladder dysfunction (69%), and unintentional weight loss >15 pound (63%). Neurological examination demonstrated concomitant distal or asymmetric hyporeflexia and hyperreflexia (81%), impaired vibration and proprioception (69%), Babinski response (68%), and asymmetric weakness (66%). MRI showed longitudinally-extensive (45%), tract-specific spinal-cord T2-hyperintensities (39%) and lumbar nerve root enhancement (38%). Ten of twenty-eight (36%) were unable to ambulate independently at last follow-up (median: 24 months, range 5-133 months). Combined oncologic and immunologic therapy had more favorable modified Rankin scores at post-treatment follow-up compared to those receiving either oncologic or immunologic therapy alone (2 [range 1 to 4] vs 4 [range 2 to 6] p<0.001).

Conclusions: Paraneoplastic etiologies should be considered in the evaluation of subacute myeloneuropathies. Recognition of key characteristics of paraneoplastic myeloneuropathy may facilitate in early tumor diagnosis and initiation of immunosuppressive treatment.

  • Received April 23, 2020.
  • Accepted in final form September 21, 2020.
  • Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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