Neurolymphomatosis: a single-center experience of neuromuscular manifestations, treatments, and outcomes
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Neurolymphomatosis: a single-center experience of neuromuscular manifestations, treatments, and outcomes Jisun Jeong1 · Sun Woong Kim1 · Duk Hyun Sung1 Received: 31 May 2020 / Revised: 27 August 2020 / Accepted: 29 August 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords Neurolymphomatosis · Lymphoma · Peripheral nervous system · FDG-PET/CT · MRI
Introduction Neurolymphomatosis (NL) is a rare manifestation of nonHodgkin’s lymphoma (NHL), defined as a direct malignant lymphocytic invasion of the peripheral nerve, spinal nerve roots, plexus, and cranial nerves. While most NL results from the progression or relapse of previous systemic disease or primary central nervous system lymphoma (secondary NL), NL can occur as isolated peripheral nervous system involvement with malignant lymphocytes at the first presentation (primary NL). NL should be distinguished from other neurological complications of lymphoma, such as mechanical compressive * Duk Hyun Sung [email protected] 1
Department of Physical and Rehabilitation Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Ilwon‑ro, Gangnam‑gu, Seoul 06351, Korea
neuropathy due to tumor mass, paraneoplastic ne
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