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The following is a summary of “Neurologic Clinical, Electrophysiologic, and Pathologic Characteristics of Primary vs Secondary Neurolymphomatosis,” published in the September 2024 issue of Neurology by Skolka et al.
Neurolymphomatosis (NL) is a disorder where lymphoma cells infiltrate the peripheral nervous system, either as a primary NL (PNL) or a secondary NL (SNL).
Researchers conducted a retrospective study describing and comparing the neurologic clinicopathologic features of patients with PNL and SNL.
They analyzed patients diagnosed with pathologically confirmed NL in the nerve (January 1, 1992, and June 30, 2020). Patient clinical characteristics, neurologic examination, imaging studies, EMG, and nerve biopsy data were collected, analyzed, and compared between PNL and SNL.
The results showed 58 patients, 34 PNLs, and 24 SNL. Time from neurologic symptom onset to diagnosis was longer in PNL at 18.5 months compared with 5.5 months in SNL (P=0.01). Neurologic symptoms were similar in both patient groups and included primarily sensory loss (98%), severe pain (76%), and asymmetric weakness (76%). A broad spectrum of EMG-confirmed different neuropathy patterns was observed. Still, patients with SNL had increased numbers of mononeuropathies (n = 8) than PNL (n = 1, P=0.01), MRI studies detected NL more frequently (86%) compared with fluorodeoxyglucose (FDG)-PET CT imaging studies (60%) (P=0.007). Nerve biopsies revealed B-cell lymphoma (PNL n = 32, SNL n = 22), followed by T-cell lymphoma (PNL n = 2, SNL n = 2), with increased demyelination in both groups and increased axonal degeneration (P=0.01) and multifocal myelinated fiber loss (P=0.04) significant in SNL vs PNL. Identifying SNL resulted in patient treatment modifications but a worse prognosis than PNL (P=0.025).
They concluded that PNL and SNL were similar neuropathies, but SNL often presented with fulminant, asymmetric mononeuropathies and was better detected on MRI than FDG-PET/CT, despite a poorer prognosis, identifying SNL was crucial for tailored treatment and management.
Source: neurology.org/doi/10.1212/WNL.0000000000209777
