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

• Published in: Journal of neurology Vol. 268; no. 3; pp. 851 - 859
• Main Authors: Jeong, Jisun, Kim, Sun Woong, Sung, Duk Hyun
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.03.2021; Springer Nature B.V
• Subjects: Cerebrospinal fluid; Computed tomography; Cranial nerves; Cytology; Differential diagnosis; Familiarity; Lymphocytes; Lymphoma; Magnetic resonance imaging; Medicine; Medicine & Public Health; Musculoskeletal diseases; Nervous system; Neurology; Neuroradiology; Neurosciences; Original Communication; Paresthesia; Patients; Peripheral nerves; Peripheral neuropathy; Positron emission tomography; Spinal nerves; Tomography; Peripheral nervous system; FDG-PET/CT; Neurolymphomatosis; MRI; Lymphoma
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-020-10202-0

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), 18 F-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.