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Neurolymphomatosis: a single-center experience of neuromuscular manifestations, treatments, and outcomes

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Abstract

Objectives

Neurolymphomatosis (NL) is a disease characterized by the infiltration of malignant lymphocytes into the peripheral nervous system. We report clinical features, radiographic findings, modes of treatment, and outcomes of patients with NL.

Methods

We retrospectively investigated patients with NL. We extracted data, including clinical features, magnetic resolution imaging (MRI), 18F-fludeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) scans, cerebrospinal fluid cytology findings, the results of electrodiagnostic studies, as well as patient treatments and outcomes.

Results

Ten NL patients were identified. All patients reported pain/paresthesia and weakness in the affected area. The MRI scans were abnormal in eight out of nine patients with an enhancement of the spinal nerve root, plexus, peripheral nerve trunk, and cranial nerve. The FDG PET/CT scans were positive in all patients. Radiculopathy or radiculoplexopathy was the most common electrodiagnostic finding. Neurological improvement was observed in only three patients. The condition of the nine patients who underwent multimodality treatments for cancer eventually deteriorated and the patients died.

Conclusions

NL should be considered in the differential diagnosis of any type of neuropathy in patients with lymphoma. Because it could be confused with other neuropathies in lymphoma and various musculoskeletal diseases, a high index of suspicion and familiarity with clinical manifestation of NL are key. FDG PET/CT was the most sensitive diagnostic imaging modality to detect relevant neural invasion. The root within the spinal neural foramen was the most frequently affected neural structure. Early diagnosis of this rare neurologic manifestation of lymphoma may improve treatment outcomes.

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Authors and Affiliations

  1. Department of Physical and Rehabilitation Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Ilwon-ro, Gangnam-gu, Seoul, 06351, Korea

    Jisun Jeong, Sun Woong Kim & Duk Hyun Sung

Authors
  1. Jisun Jeong
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  2. Sun Woong Kim
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  3. Duk Hyun Sung
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Correspondence to Duk Hyun Sung.

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Conflicts of interest

We did not receive any financial support or other benefits from commercial sources for the work reported in this manuscript, nor did any of the authors have any financial interests with regard to the work that may create a potential conflict of interest or the appearance of one. This work has not been previously presented, submitted, or published in any form or any language. The authors declare that they have no conflicts of interest.

Ethics approval

All procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional review board and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Jeong, J., Kim, S.W. & Sung, D.H. Neurolymphomatosis: a single-center experience of neuromuscular manifestations, treatments, and outcomes. J Neurol 268, 851–859 (2021). https://doi.org/10.1007/s00415-020-10202-0

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  • DOI: https://doi.org/10.1007/s00415-020-10202-0

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