Neurologic Clinical, Electrophysiologic, and Pathologic Characteristics of Primary vs Secondary Neurolymphomatosis.

Autor: Skolka MP; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Suanprasert N; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Martinez-Thompson JM; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., King RL; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Macon WR; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Mauermann ML; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Klein CJ; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Habermann TM; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Johnston PB; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Micallef IN; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Khurana A; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Amrami K; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Spinner RJ; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Mandrekar J; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Dyck PJ; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok., Dyck PJB; From the Departments of Neurology (M.P.S., J.M.M.-T., M.L.M., C.J.K., P.J.D., P.J.B.D.), Laboratory Medicine and Pathology (R.L.K., W.R.M.), Hematology (T.M.H., P.B.J., I.N.M., A.K.), Radiology (K.A.), Neurosurgery (R.J.S.), and Quantitative Health Sciences (J.M.), Mayo Clinic, Rochester, MN; and Neurological Institute of Thailand (N.S.), Bangkok.
Jazyk: angličtina
Zdroj: Neurology [Neurology] 2024 Sep 24; Vol. 103 (6), pp. e209777. Date of Electronic Publication: 2024 Sep 03.
DOI: 10.1212/WNL.0000000000209777
Abstrakt: Background and Objectives: Neurolymphomatosis (NL) is characterized by lymphomatous infiltration of the peripheral nervous system presenting as the initial manifestation of a lymphoma (primary NL [PNL]) or in relapse of a known lymphoma (secondary NL [SNL]). This report details and compares the neurologic clinicopathologic characteristics of these 2 groups.
Methods: This retrospective study was performed on patients diagnosed with pathologically confirmed NL in nerve between January 1, 1992, and June 31, 2020. Patient clinical characteristics, neurologic examination, imaging studies, EMG, and nerve biopsy data were collected, analyzed, and compared between PNL and SNL.
Results: A total of 58 patients were identified (34 PNL 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 wide spectrum of EMG-confirmed different neuropathy patterns were observed, but patients with SNL had increased numbers of mononeuropathies (n = 8) compared with 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 compared with PNL ( p = 0.025).
Discussion: While PNL and SNL are both primarily painful and asymmetric neuropathies with axonal and demyelinating features on EMG and nerve biopsy, SNL presents somewhat differently than PNL with fulminant, asymmetric often mononeuropathies better detected on MRI than FDG-PET/CT. The focal pattern of SNL is likely a result of residual cancer cells that evaded initial chemotherapy, which does not cross the blood-nerve barrier, and these cells can later recur and result in fulminant disease. Although still resulting in a poorer prognosis, identifying SNL is important because this changed treatment and management in every SNL case.
Databáze: MEDLINE