Diagnostic delay in a case of T-cell neurolymphomatosis

Abstract

A 69-year-old woman presented with severe subacute painful meningoradiculoneuritis. Neurophysiology showed a patchy, proximal axonal process with widespread denervation. Cerebrospinal fluid (CSF) was lymphocytic (normal T-cell predominant) with negative cytology. MRI revealed multiple sites of enhancement, but fluorodeoxyglucose positron emission tomography was negative. Bone marrow aspirate and trephine (BMAT) showed no evidence of a lymphoproliferative condition. Right brachial plexus biopsy demonstrated mixed T-cell/B-cell endoneurial inflammation not fulfilling criteria for vasculitis. She was stabilised with high-dose steroids and cyclophosphamide, followed by mycophenolate for inflammatory myeloradiculoneuritis. However, symptoms recurred when prednisolone was weaned. Although T-cell receptor gene analysis from the initial CSF demonstrated clonal rearrangements, it was only when the same clones were identified on two repeat BMATs and CSF that T-cell neurolymphomatosis, an exceedingly rare condition, was diagnosed. This case highlights the diagnostic challenge in peripheral neurolymphomatosis related to patchy disease, variable sensitivity and specificity of investigative tools, and the influence of therapies on traditional cytological definitions of lymphoma. The clinical picture, exhaustive exclusion of alternative causes and the persistence of an abnormal T-cell clone ultimately lead to a diagnostic consensus between specialist neurology and haematology clinicians.

Primary Cauda Equina Lymphoma Treated with CNS-Centric Approach: A Case Report and Literature Review

“…Radiologic review of her imaging scans from the time of initial presentation did not show any evidence of intramedullary involvement of the spinal cord, but they did reveal a mass encasing multiple nerve roots in the cauda equina. Unlike typical neurolymphomatosis which most commonly represents a challenging diagnostic entity due to patchy nerve involvement requiring multiple biopsies and investigations over time, 5,6 our patient presented with a solid tumor due to lymphomatous involvement of multiple nerve roots in the cauda equina region in a tight spinal canal. This unique presentation, in contrast to most cases of neurolymphomatosis, allowed for a more expedient diagnosis and prevented any delays in treatment.…”

Acute Lymphocytic Meningoradiculoneuritis Complicating Primary Sjögren Syndrome

“…The pathophysiology of peripheral neuropathy during pSS is not well known; several mechanisms may be involved: vasanervorum vasculitis, cryoglobulinemia, hypergammaglobulinemia, autoimmunity, and inflammation [5,6,15]. The main differential diagnoses to be discussed in front of acute meningoradiculoneuritis in patient with pSS are infectious causes (particularly Lyme disease/neuroborreliosis and Varicella-Zoster Virus reactivation) [16,17], and lymphomas (neurolymphomatosis) [18]. Opportunistic infections as well as lymphomatous transformation are common during pSS.…”