Neurological misdiagnoses of lymphoma.
Atypical parkinsonism
CNS demyelinating disease
CNS lymphoma
Inflammatory transverse myelitis
Neurolymphomatosis
Journal
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175
Informations de publication
Date de publication:
May 2021
May 2021
Historique:
received:
25
05
2020
accepted:
15
09
2020
pubmed:
26
9
2020
medline:
15
5
2021
entrez:
25
9
2020
Statut:
ppublish
Résumé
Lymphoma of the nervous system is rare and usually involves the brain, spinal cord, or peripheral nerves. Hence, it has varied clinical presentations, and correct diagnosis is often challenging. Incorrect diagnosis delays the appropriate treatment and affects prognosis. We report 5 patients with delayed diagnosis of lymphoma involving the central and/or peripheral nervous system, initially evaluated for other neurological diagnoses. We also discuss the challenge of diagnosis and appropriate testing. Retrospective review of 2011-2019 records of patients with confirmed nervous system lymphoma diagnosed in a tertiary care medical center. We present 5 adult patients initially evaluated for inflammatory myelopathy, inflammatory lumbosacral plexopathy, atypical parkinsonism, and demyelinating disease of the CNS. Final diagnosis of the nervous system lymphoma was delayed by 4 to 18 months and was based on tissue biopsy in 4, and on CSF and bone marrow examination in 1 patient. Lymphoma may imitate various central and peripheral nervous system disorders. We suggest several red flags that indicate the need to consider lymphoma, including subacute but progressive symptomatic evolution, painful neurological deficit, unclear clinical diagnosis, and transient steroid responsiveness. Correct diagnosis often requires a combination of diagnostic tests, while pathology testing is crucial for early diagnosis and is strongly recommended in the appropriate clinical setting.
Sections du résumé
BACKGROUND
BACKGROUND
Lymphoma of the nervous system is rare and usually involves the brain, spinal cord, or peripheral nerves. Hence, it has varied clinical presentations, and correct diagnosis is often challenging. Incorrect diagnosis delays the appropriate treatment and affects prognosis. We report 5 patients with delayed diagnosis of lymphoma involving the central and/or peripheral nervous system, initially evaluated for other neurological diagnoses. We also discuss the challenge of diagnosis and appropriate testing.
METHODS
METHODS
Retrospective review of 2011-2019 records of patients with confirmed nervous system lymphoma diagnosed in a tertiary care medical center.
RESULTS
RESULTS
We present 5 adult patients initially evaluated for inflammatory myelopathy, inflammatory lumbosacral plexopathy, atypical parkinsonism, and demyelinating disease of the CNS. Final diagnosis of the nervous system lymphoma was delayed by 4 to 18 months and was based on tissue biopsy in 4, and on CSF and bone marrow examination in 1 patient.
CONCLUSIONS
CONCLUSIONS
Lymphoma may imitate various central and peripheral nervous system disorders. We suggest several red flags that indicate the need to consider lymphoma, including subacute but progressive symptomatic evolution, painful neurological deficit, unclear clinical diagnosis, and transient steroid responsiveness. Correct diagnosis often requires a combination of diagnostic tests, while pathology testing is crucial for early diagnosis and is strongly recommended in the appropriate clinical setting.
Identifiants
pubmed: 32974798
doi: 10.1007/s10072-020-04724-8
pii: 10.1007/s10072-020-04724-8
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1933-1940Références
Shiels MS, Pfeiffer RM, Besson C, Clarke CA, Morton LM, Nogueira L, Pawlish K, Yanik EL, Suneja G, Engels EA (2016) Trends in primary central nervous system lymphoma incidence and survival in the U.S. Br J Haematol 174(3):417–424. https://doi.org/10.1111/bjh.14073
doi: 10.1111/bjh.14073
pubmed: 27018254
pmcid: 4961566
Grommes C, DeAngelis LM (2017) Primary CNS Lymphoma. J Clin Oncol 35(21):2410–2418. https://doi.org/10.1200/JCO.2017.72.7602
doi: 10.1200/JCO.2017.72.7602
pubmed: 28640701
pmcid: 5516483
Bernstein SH, Unger JM, Leblanc M, Friedberg J, Miller TP, Fisher RI (2009) Natural history of CNS relapse in patients with aggressive non-Hodgkin’s lymphoma: a 20-year follow-up analysis of SWOG 8516 -- the Southwest Oncology Group. J Clin Oncol 27(1):114–119. https://doi.org/10.1200/JCO.2008.16.8021
doi: 10.1200/JCO.2008.16.8021
pubmed: 19047289
pmcid: 4879698
Mead GM, Kennedy P, Smith JL, Thompson J, Macbeth FR, Ryall RD, Williams CJ, Whitehouse JM (1986) Involvement of the central nervous system by non-Hodgkin’s lymphoma in adults. A review of 36 cases. Q J Med 60(231):699–714
pubmed: 3763820
Ferreri AJM (2017) Secondary CNS lymphoma: the poisoned needle in the haystack. Ann Oncol 28(10):2335–2337. https://doi.org/10.1093/annonc/mdx515
doi: 10.1093/annonc/mdx515
pubmed: 28961853
Haldorsen IS, Espeland A, Larsen JL, Mella O (2005) Diagnostic delay in primary central nervous system lymphoma. Acta Oncol 44(7):728–734. https://doi.org/10.1080/02841860500256272
doi: 10.1080/02841860500256272
pubmed: 16227164
Herrlinger U, Schabet M, Clemens M, Kortmann R-D, Petersen D, Will BE, Meyemand R, Dichgans J (1998) Clinical presentation and therapeutic outcome in 26 patients with primary CNS lymphoma. Acta Neurol Scand 97(4):257–264. https://doi.org/10.1111/j.1600-0404.1998.tb00647.x
doi: 10.1111/j.1600-0404.1998.tb00647.x
pubmed: 9576641
Braus DF, Schwechheimer K, Müller-Hermelink HK, Schwarzkopf G, Volk B, Mundinger F (1992) Primary cerebral malignant non-Hodgkin’s lymphomas: a retrospective clinical study. J Neurol 239(3):117–124. https://doi.org/10.1007/bf00833908
doi: 10.1007/bf00833908
pubmed: 1573413
Krough-Jensen M, Amore FD, Jensen MK, Christensen BE, Thorling K, Pedersen M, Johansen P, Boesen AM, Andersen E (1995) Clinicopathological features, survival and prognostic factors of primary central nervous system lymphomas: trends in incidence of primary central nervous system lymphomas and primary malignant brain tumors in a well-defined geographical area; Population-Based Data from the Danish Lymphoma Registry, Lyfo, And the Danish Cancer Reistry. Leuk Lymphoma 19(3-4):223–233. https://doi.org/10.3109/10428199509107892
doi: 10.3109/10428199509107892
Khong P, Pitham T, Owler B (2008) Isolated neurolymphomatosis of the cauda equina and filum terminale: case report. Spine (Phila Pa 1976) 33(21):E807–E811. https://doi.org/10.1097/BRS.0b013e31818441be
doi: 10.1097/BRS.0b013e31818441be
Diaz-Arrastia R, Younger DS, Hair L, Inghirami G, Hays AP, Knowles DM, Odel JG, Fetell MR, Lovelace RE, Rowland LP (1992) Neurolymphomatosis: a clinicopathologic syndrome re-emerges. Neurology 42(6):1136–1141. https://doi.org/10.1212/wnl.42.6.1136
doi: 10.1212/wnl.42.6.1136
pubmed: 1340762
Avila JD, Vivar C (2017) Neurolymphomatosis: a review of 82 cases (P5.141). Neurology 88(16 Supplement)
Nakashima H, Imagama S, Ito Z, Ando K, Kobayashi K, Ukai J, Muramoto A, Shinjyo R, Matsumoto T, Yamauchi I, Satou A, Ishiguro N (2014) Primary cauda equina lymphoma: case report and literature review. Nagoya J Med Sci 76(3-4):349–354
pubmed: 25741044
pmcid: 4345686
Flanagan EP, O'Neill BP, Porter AB, Lanzino G, Haberman TM, Keegan BM (2011) Primary intramedullary spinal cord lymphoma. Neurology 77(8):784–791. https://doi.org/10.1212/WNL.0b013e31822b00b9
doi: 10.1212/WNL.0b013e31822b00b9
pubmed: 21832220
Flanagan EP, O'Neill BP, Habermann TM, Porter AB, Keegan BM (2012) Secondary intramedullary spinal cord non-Hodgkin's lymphoma. J Neuro-Oncol 107(3):575–580. https://doi.org/10.1007/s11060-011-0781-4
doi: 10.1007/s11060-011-0781-4
Cohen-Gadol AA, Zikel OM, Miller GM, Aksamit AJ, Scheithauer BW, Krauss WE (2003) Spinal Cord Biopsy: A Review of 38 Cases. Neurosurgery 52(4):806–816. https://doi.org/10.1227/01.NEU.0000053223.77641.5E
doi: 10.1227/01.NEU.0000053223.77641.5E
pubmed: 12657176
Schwarz S, Zoubaa S, Knauth M, Sommer C, Storch-Hagenlocher B (2002) Intravascular lymphomatosis presenting with a conus medullaris syndrome mimicking disseminated encephalomyelitis. Neuro-Oncology 4(3):187–191. https://doi.org/10.1093/neuonc/4.3.187
doi: 10.1093/neuonc/4.3.187
pubmed: 12084349
pmcid: 1920637
Kumar N, Keegan BM, Rodriguez FJ, Hammack JE, Kantarci OH Intravascular lymphoma presenting as a longitudinally-extensive myelitis: diagnostic challenges and etiologic clues. J Neurol Sci 303(1):146–149. https://doi.org/10.1016/j.jns.2010.12.016
Ali K, Morris HR (2015) Parkinson’s disease: chameleons and mimics. Pract Neurol 15(1):14–25. https://doi.org/10.1136/practneurol-2014-000849
doi: 10.1136/practneurol-2014-000849
pubmed: 25253895
Sanchez-Guerra M, Cerezal L, Leno C, Diez C, Figols J, Berciano J (2001) Primary brain lymphoma presenting as Parkinson’s disease. Neuroradiology 43(1):36–40
doi: 10.1007/s002340000451
Gherardi R, Roualdes B, Fleury J, Prost C, Poirier J, Degos JD (1985) Parkinsonian syndrome and central nervous system lymphoma involving the substantia nigra. A case report. Acta Neuropathol 65(3-4):338–343
doi: 10.1007/BF00687019
Pramstaller PP, Salerno A, Bhatia KP, Prugger M, Marsden CD (1999) Primary central nervous system lymphoma presenting with a parkinsonian syndrome of pure akinesia. J Neurol 246(10):934–938
doi: 10.1007/s004150050485
Solomon AJ, Weinshenker BG (2013) Misdiagnosis of multiple sclerosis: frequency, causes, effects, and prevention. Curr Neurol Neurosci Rep 13(12):403. https://doi.org/10.1007/s11910-013-0403-y
doi: 10.1007/s11910-013-0403-y
pubmed: 24142849
Rudick RA, Miller AE (2012) Multiple sclerosis or multiple possibilities: the continuing problem of misdiagnosis. Neurology 78(24):1904–1906. https://doi.org/10.1212/WNL.0b013e318259e2e2
doi: 10.1212/WNL.0b013e318259e2e2
pubmed: 22581931
Solomon AJ, Klein EP, Bourdette D (2012) “Undiagnosing” multiple sclerosis: the challenge of misdiagnosis in MS. Neurology 78(24):1986–1991. https://doi.org/10.1212/WNL.0b013e318259e1b2
doi: 10.1212/WNL.0b013e318259e1b2
pubmed: 22581930
pmcid: 3369504
Kuker W, Nagele T, Thiel E, Weller M, Herrlinger U (2005) Primary central nervous system lymphomas (PCNSL): MRI response criteria revised. Neurology 65(7):1129–1131. https://doi.org/10.1212/01.wnl.0000178894.51436.54
doi: 10.1212/01.wnl.0000178894.51436.54
pubmed: 16217075
Husseini L, Saleh A, Reifenberger G, Hartung HP, Kieseier BC (2012) Inflammatory demyelinating brain lesions heralding primary CNS lymphoma. Can J Neurol Sci 39(1):6–10
doi: 10.1017/S0317167100012610
Kuhlmann T, Schroter A, Dechent P, Weber F, Rustenbeck HH, Fuzesi L, Bruck W, Ehrenreich H, Frahm J (2001) Diagnosis of a multifocal B cell lymphoma with preceding demyelinating central nervous system lesions by single voxel proton MR spectroscopy. J Neurol Neurosurg Psychiatry 70(2):259–262
doi: 10.1136/jnnp.70.2.259
Lin X, Tan K, Liauw L, Tin AS, Soon W, Eileen YSR, Zhu TBP, Lee HY, Yu W-Y (2014) Distinguishing clinico-radiological features in patients with central nervous system tumefactive white matter lesions (P5.177). Neurology 82(10 Supplement)
Geraldes R, Ciccarelli O, Barkhof F, De Stefano N, Enzinger C, Filippi M, Hofer M, Paul F, Preziosa P, Rovira A, GC DL, Kappos L, Yousry T, Fazekas F, Frederiksen J, Gasperini C, Sastre-Garriga J, Evangelou N, Palace J, Group MS (2018) The current role of MRI in differentiating multiple sclerosis from its imaging mimics. Nat Rev Neurol 14(4):199–213. https://doi.org/10.1038/nrneurol.2018.14
doi: 10.1038/nrneurol.2018.14
pubmed: 29521337
DeAngelis LM (1999) Primary central nervous system lymphoma. Curr Opin Neurol 12(6):687–691
doi: 10.1097/00019052-199912000-00005
Rubenstein J, Ferreri AJM, Pittaluga S (2008) Primary lymphoma of the central nervous system: epidemiology, pathology and current approaches to diagnosis, prognosis and treatment. Leuk Lymphoma 49(sup1):43–51. https://doi.org/10.1080/10428190802311441
doi: 10.1080/10428190802311441
pubmed: 18821432
pmcid: 4110179
Nowosielski M, Pardo-Moreno J, Penalver FJ, Buda G, Bennani H, Costopoulos M, Le Garff-Tavernier M, Soussain C, Schmid M, Orfao JA, Glantz M (2016) How to facilitate early diagnosis of CNS involvement in malignant lymphoma AU - Korfel, Agnieszka. Expert Rev Hematol 9(11):1081–1091. https://doi.org/10.1080/17474086.2016.1242405
doi: 10.1080/17474086.2016.1242405
pubmed: 27677656