Clinical Neurology and Neuroscience

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Primary Leptomeningeal Lymphoma Presenting as Low Back Pain and Dementia: A Case Report

Received: 31 May 2018    Accepted: 04 July 2018    Published: 30 July 2018
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Abstract

Primary leptomeningeal lymphoma (PLL) is an extremely rare condition, with only a handful of cases reported in literature. Most often, these are misdiagnosed as other disease entities that are more commonly observed in practice. Currently, there are no clinical algorithms that may guide clinicians to clinching a diagnosis of PLL. This paper discusses a remarkable case of PLL presenting with atypical symptoms namely dementia and low back pain. This is a case of an apparently well adult male who initially presented with low back pain that was treated for two years as a case of lumbar disc herniation. This developed into lower extremity weakness and was subsequently followed by dementia and seizures. The patient was initially treated for tuberculous meningitis after CSF findings showed lymphocytic predominance. However, lack of clinical improvement prompted further work up revealing a malignant process in the lumbosacral meninges. Dural biopsy showed atypical mononuclear infiltrates consistent with a B-cell non-Hodgkin lymphoma with immunohistochemistry showing CD20 positive, CD79a positive, CD3 negative. Being MUM1 positive and BCL2 positive suggests it being a diffuse large B cell lymphoma. A primary malignancy of the leptomeninges was ruled in after PET CT showed no other sites of high metabolic activity. The patient was able to complete 6 cycles of high dose Methotrexate and three Rituximab doses. Remarkably, after more than two years after his initial presentation, he still has mild signs of dementia but is able to carry out most activities of daily living with minimal assistance. The patient has survived beyond the expected life expectancy for PLL.

DOI 10.11648/j.cnn.20180202.13
Published in Clinical Neurology and Neuroscience (Volume 2, Issue 2, June 2018)
Page(s) 33-40
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Primary Leptomeningeal Lymphoma, Atypical Back Pain, Dementia

References
[1] Ropper A. H., Samuels M. A., Klein J. P. Adams and Victor's Principles of Neurology. 10th ed. New York: Mc-Graw-Hill Educational Medical; 2009.
[2] Fischer, L., Martus, P., Weller, M., Klasen, H.-A., Rohden, B., Röth, A.,… Korfel, A. (2008). Meningeal dissemination in primary CNS lymphoma. Neurology, 71, 1102–1108.
[3] Lachance, D. H., O’Neill, B. P., Macdonald, D. R.,… Posner J. B. (1991) Primary leptomeningeal lymphoma: report of 9 cases, diagnosis with immunocytochemical analysis, and review of the literature. Neurology. Jan; 41 (1): 95-100.
[4] Taylor, J. W., Flanagan E. P., O’Neill B. P., Siegal T…Schiff, D. (2013). Primary leptomeningeal lymphoma. Neurology, Nov 5; 81 (10):1690-1696.
[5] Galarza, M., Gazzeri, R., Elfeky, H. a, & Johnson, R. R. (2006). Primary diffuse large B-cell lymphoma of the dura mater and cranial vault. Case report and literature review. Neurosurgical Focus, 21 (5), E10. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/17134112.
[6] Iwamoto, F., & Abrey, L. (2006). Primary dural lymphomas: a review. Neurosurg Focus, 21 (5), 1–5.
[7] Freilich R. J., Krol G., DeAngelis L. M. (1995) Neuroimaging and Cerebrospinal Fluid Cytology in the Diagnosis of Leptomeningeal Metastasis. Ann Neurol. 1995; 38:51-57.
[8] Carlson C. L., Hartman R., Ly J. Q., Beall D. P. (2003) Primary leptomeningeal lymphoma of the lumbar spine. Clin Imaging, 27: 389-393.
[9] Gijtenbeek, J. M. M., Rosenblum, M. K., & DeAngelis, L. M. (2011). Primary Central Nervous System T-cell Lymphoma. Neurology, 57 (4), 716-718.
[10] Shenkier, T. N., Blay, J., Neill, B. P. O., Poortmans, P., Thiel, E., Jahnke, K., … Connors, J. M. (2005). Primary CNS Lymphoma of T-Cell Origin : A Descriptive Analysis From the International Primary CNS Lymphoma Collaborative Group. Journal of Clinical Oncology, 23 (10), 2233–2239. http://doi.org/10.1200/JCO.2005.07.109.
[11] Chong, A., Song, H.-C., Byun, B.-H., Hong, S.-P., Min, J.-J., Bom, H.-S., … Lee, J.-K. (2013). Changes in (18) f-fluorodeoxyglucose uptake in the spinal cord in a healthy population on serial positron emission tomography/computed tomography. Chonnam Medical Journal, 49 (1), 38–42. http://doi.org/10.4068/cmj.2013.49.1.38.
[12] Cappellari, M., Benedetti, F., Fiaschi A., Benedetti M. D. (2011). Multiple radiculopathy as the presenting manifestation of primary spinal leptomeningeal B cell lymphoma detected by flow cytometry of cerebrospinal fluid. Neurol Sci, 32:1171-1174.
[13] Beitzke, M., Enzinger, C., Beitze, D….Fazekas, F. (2010) Primary leptomeningeal lymphoma of the cauda equina: a rare cause of radiculopathy. J Neurol, 257:1734-1737.
[14] Omuro, A. M. P., Lallana, E. C., Bilsky, M. H., & Deangelis, L. M. (2005). Venticuloperitoneal shunt in patients with leptomeningeal metastasis. Neurology, 64, 1625–1628.
[15] Clarridge, J. E. I., Shawar, R. M., Shinnick, T. M., & Plikaytis, B. B. (1993). Large-Scale Use of Polymerase Chain Reaction for Detection of Mycobacterium tuberculosis in a Routine Mycobacteriology Laboratory. Journal of Clinical Microbiology, 31 (8), 2049–2056.
[16] Perry, A. M., Mitrovic, Z., & Chan, W. C. (2012). Biological prognostic markers in diffuse large B-cell lymphoma. Cancer Control, 19 (3), 214–226.
[17] Hwang, H. S., Park, C., Yoon, D. H., Suh, C., & Huh, J. (2014). High Concordance of Gene Expression Profiling – correlated Immunohistochemistry Algorithms in Diffuse Large B-cell Lymphoma,. American Journal of Surgical Pathology, 38 (8), 1046–1057.
[18] MacNealy, M. W. C., Newton, H. B., McGregor, J. M., Bell, S. D., Ray Chaudhury, A., Slone, H. W., & Bourekas, E. C. (2008). Primary meningeal CNS lymphoma treated with intra-arterial chemotherapy and blood-brain barrier disruption. Journal of Neuro-Oncology, 90 (3), 329–333. http://doi.org/10.1007/s11060-008-9667-5.
[19] Ulep, V., & dela Cruz, N. A. (2014). Analysis of Out-of-Pocket Expenditures in the Philippines.
Author Information
  • Department of Neurosciences, University of the Philippines, Philippine General Hospital, Manila, Philippines

  • Department of Neurosciences, University of the Philippines, Philippine General Hospital, Manila, Philippines

  • St. Luke’s Medical Center Global City, Taguig, Philippines

Cite This Article
  • APA Style

    Vida Margarette de Vera Andal, Julette Marie Feliciano Batara, Joven Racpan Cuanang. (2018). Primary Leptomeningeal Lymphoma Presenting as Low Back Pain and Dementia: A Case Report. Clinical Neurology and Neuroscience, 2(2), 33-40. https://doi.org/10.11648/j.cnn.20180202.13

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    ACS Style

    Vida Margarette de Vera Andal; Julette Marie Feliciano Batara; Joven Racpan Cuanang. Primary Leptomeningeal Lymphoma Presenting as Low Back Pain and Dementia: A Case Report. Clin. Neurol. Neurosci. 2018, 2(2), 33-40. doi: 10.11648/j.cnn.20180202.13

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    AMA Style

    Vida Margarette de Vera Andal, Julette Marie Feliciano Batara, Joven Racpan Cuanang. Primary Leptomeningeal Lymphoma Presenting as Low Back Pain and Dementia: A Case Report. Clin Neurol Neurosci. 2018;2(2):33-40. doi: 10.11648/j.cnn.20180202.13

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  • @article{10.11648/j.cnn.20180202.13,
      author = {Vida Margarette de Vera Andal and Julette Marie Feliciano Batara and Joven Racpan Cuanang},
      title = {Primary Leptomeningeal Lymphoma Presenting as Low Back Pain and Dementia: A Case Report},
      journal = {Clinical Neurology and Neuroscience},
      volume = {2},
      number = {2},
      pages = {33-40},
      doi = {10.11648/j.cnn.20180202.13},
      url = {https://doi.org/10.11648/j.cnn.20180202.13},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.cnn.20180202.13},
      abstract = {Primary leptomeningeal lymphoma (PLL) is an extremely rare condition, with only a handful of cases reported in literature. Most often, these are misdiagnosed as other disease entities that are more commonly observed in practice. Currently, there are no clinical algorithms that may guide clinicians to clinching a diagnosis of PLL. This paper discusses a remarkable case of PLL presenting with atypical symptoms namely dementia and low back pain. This is a case of an apparently well adult male who initially presented with low back pain that was treated for two years as a case of lumbar disc herniation. This developed into lower extremity weakness and was subsequently followed by dementia and seizures. The patient was initially treated for tuberculous meningitis after CSF findings showed lymphocytic predominance. However, lack of clinical improvement prompted further work up revealing a malignant process in the lumbosacral meninges. Dural biopsy showed atypical mononuclear infiltrates consistent with a B-cell non-Hodgkin lymphoma with immunohistochemistry showing CD20 positive, CD79a positive, CD3 negative. Being MUM1 positive and BCL2 positive suggests it being a diffuse large B cell lymphoma. A primary malignancy of the leptomeninges was ruled in after PET CT showed no other sites of high metabolic activity. The patient was able to complete 6 cycles of high dose Methotrexate and three Rituximab doses. Remarkably, after more than two years after his initial presentation, he still has mild signs of dementia but is able to carry out most activities of daily living with minimal assistance. The patient has survived beyond the expected life expectancy for PLL.},
     year = {2018}
    }
    

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    T1  - Primary Leptomeningeal Lymphoma Presenting as Low Back Pain and Dementia: A Case Report
    AU  - Vida Margarette de Vera Andal
    AU  - Julette Marie Feliciano Batara
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    AB  - Primary leptomeningeal lymphoma (PLL) is an extremely rare condition, with only a handful of cases reported in literature. Most often, these are misdiagnosed as other disease entities that are more commonly observed in practice. Currently, there are no clinical algorithms that may guide clinicians to clinching a diagnosis of PLL. This paper discusses a remarkable case of PLL presenting with atypical symptoms namely dementia and low back pain. This is a case of an apparently well adult male who initially presented with low back pain that was treated for two years as a case of lumbar disc herniation. This developed into lower extremity weakness and was subsequently followed by dementia and seizures. The patient was initially treated for tuberculous meningitis after CSF findings showed lymphocytic predominance. However, lack of clinical improvement prompted further work up revealing a malignant process in the lumbosacral meninges. Dural biopsy showed atypical mononuclear infiltrates consistent with a B-cell non-Hodgkin lymphoma with immunohistochemistry showing CD20 positive, CD79a positive, CD3 negative. Being MUM1 positive and BCL2 positive suggests it being a diffuse large B cell lymphoma. A primary malignancy of the leptomeninges was ruled in after PET CT showed no other sites of high metabolic activity. The patient was able to complete 6 cycles of high dose Methotrexate and three Rituximab doses. Remarkably, after more than two years after his initial presentation, he still has mild signs of dementia but is able to carry out most activities of daily living with minimal assistance. The patient has survived beyond the expected life expectancy for PLL.
    VL  - 2
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