Journal of Neuro-Oncology

, Volume 109, Issue 3, pp 581–586 | Cite as

Lymphomatosis cerebri presenting with orthostatic hypotension, anorexia, and paraparesis

  • Anjeni Keswani
  • Eileen Bigio
  • Sean Grimm
Case Report


To increase awareness about lymphomatosis cerebri by describing a patient with a unique presentation Case report a 58 year old woman presented with progressive lower extremity weakness, postural hypotension, and 90 pound weight loss over 3 months a brain magnetic resonance image revealed multiple non-enhancing foci of T2 hyperintensity in the periventricular white matter despite treatment with corticosteroids, she expired autopsy demonstrated normal gross appearance of the brain and spinal cord microscopic inspection revealed diffuse infiltration of the central nervous system (CNS) parenchyma and white matter by large atypical B cells, consistent with a diagnosis of lymphomatosis cerebri lymphomatosis cerebri is a primary CNS lymphoma variant that is poorly recognized and often misdiagnosed it commonly presents as a rapidly progressive dementia, although patients may present with neurologic dysfunction without dementia diagnosis requires a pathological examination treatment with intravenous high–dose methotrexate based chemotherapy should be considered in appropriate patients.


Lymphomatosis cerebri Primary central nervous system lymphoma Neuro-oncology 


  1. 1.
    DeAngelis LM (2001) Brain tumors. N Engl J Med 344:114–123PubMedCrossRefGoogle Scholar
  2. 2.
    Kanai R, Shibuya M, Hata T, Hori M, Hirabayashi K, Terada T, Fujii K (2008) A case of ‘lymphomatosis cerebri’ diagnosed in an early phase and treated by whole brain radiation: case report and literature review. J Neurooncol 86:83–88PubMedCrossRefGoogle Scholar
  3. 3.
    Bakshi R, Mazziotta JC, Mischel PS, Jahan R, Seligson DB, Vinters HV (1999) Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings. Dement Geriatr Cogn Disord 10:152–157PubMedCrossRefGoogle Scholar
  4. 4.
    Carlson BA (1996) Rapidly progressive dementia caused by nonenhancing primary lymphoma of the central nervous system. AJNR 17:1695–1697PubMedGoogle Scholar
  5. 5.
    de Toledo M, Lopez-Valdes E, Ferreiro M, Cervera JL, Ramos A, Cabello A, Hernandez-Lain A, Montes-Montes S, Lagares A, Alvarez-Linera Prado J (2008) Lymphomatosis cerebri as the cause of leukoencephalopathy. Revista de Neurologia 46:667–670PubMedGoogle Scholar
  6. 6.
    Furusawa T, Okamoto K, Ito J, Kojima N, Oyanagi K, Tokiguchi S, Sakai K (1998) Primary central nervous system lymphoma presenting as diffuse cerebral infiltration. Radiat Med 16:137–140PubMedGoogle Scholar
  7. 7.
    Kitai R, Hashimoto N, Yamate K, Ikawa M, Yoneda M, Nakajima T, Arishima H, Takeuchi H, Sato K, Kikuta K (2012) Lymphomatosis cerebri: clinical characteristics, neuroimaging, and pathological findings. Brain Tumor Pathol 29:47–53PubMedCrossRefGoogle Scholar
  8. 8.
    Leschziner G, Rudge P, Lucas S, Andrews T (2011) Lymphomatosis cerebri presenting as a rapidly progressive dementia with a high methylmalonic acid. J Neurol 258:1489–1493PubMedCrossRefGoogle Scholar
  9. 9.
    Lewerenz J, Ding X, Matschke J, Schnabel C, Emami P, von Borczyskowski D, Buchert R, Krieger T, de Wit M, Munchau A (2007) Dementia and leukoencephalopathy due to lymphomatosis cerebri. J Neurol Neurosurg Psychiatr 78:777–778PubMedCrossRefGoogle Scholar
  10. 10.
    Pandit L, Chickabasaviah Y, Raghothaman A, Mustafa S, Vasudevan A (2010) Lymphomatosis cerebri—a rare cause of leukoencephalopathy. J Neurol Sci 293:122–124PubMedCrossRefGoogle Scholar
  11. 11.
    Raz E, Tinelli E, Antonelli M, Canevelli M, Fiorelli M, Bozzao L, Di Piero V, Caramia F (2011) MRI findings in lymphomatosis cerebri: description of a case and revision of the literature. J Neuroimaging 21:e183–e186PubMedCrossRefGoogle Scholar
  12. 12.
    Rollins KE, Kleinschmidt-DeMasters BK, Corboy JR, Damek DM, Filley CM (2005) Lymphomatosis cerebri as a cause of white matter dementia. Hum Pathol 36:282–290PubMedCrossRefGoogle Scholar
  13. 13.
    Vital A, Sibon I (2007) A 64-year-old woman with progressive dementia and leukoencephalopathy. Brain Pathol (Zurich, Switzerland) 17: 117–118, 121 Google Scholar
  14. 14.
    Weaver JD, Vinters HV, Koretz B, Xiong Z, Mischel P, Kado D (2007) Lymphomatosis cerebri presenting as rapidly progressive dementia. Neurologist 13:150–153PubMedCrossRefGoogle Scholar
  15. 15.
    Poussaint TY, Barnes PD, Nichols K, Anthony DC, Cohen L, Tarbell NJ, Goumnerova L (1997) Diencephalic syndrome: clinical features and imaging findings. AJNR 18:1499–1505PubMedGoogle Scholar
  16. 16.
    Ashworth B (1982) Cerebral histiocytic lymphoma presenting with loss of weight. Neurology 32:894–896PubMedCrossRefGoogle Scholar
  17. 17.
    Miyoshi Y, Yunoki M, Yano A, Nishimoto K (2003) Diencephalic syndrome of emaciation in an adult associated with a third ventricle intrinsic craniopharyngioma: case report. Neurosurgery 52: 224–227; discussion 227Google Scholar
  18. 18.
    Fleischman A, Brue C, Poussaint TY, Kieran M, Pomeroy SL, Goumnerova L, Scott RM, Cohen LE (2005) Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance. Pediatrics 115:e742–e748PubMedCrossRefGoogle Scholar
  19. 19.
    Hsu CY, Hogan EL, Wingfield W Jr, Webb JG, Perot PL Jr, Privitera PJ, Talbert OR, Balentine JD (1984) Orthostatic hypotension with brainstem tumors. Neurology 34:1137–1143PubMedCrossRefGoogle Scholar
  20. 20.
    Telerman-Toppet N, Vanderhaeghen JJ, Warszawski M (1982) Orthostatic hypotension with lower brain stem glioma. J Neurol Neurosurg Psychiatr 45:1147–1150PubMedCrossRefGoogle Scholar
  21. 21.
    Thomas JE, Schirger A, Love JG, Hoffman DL (1961) Orthostatic hypotension as the presenting sign in craniopharyngioma. Neurology 11:418–423PubMedCrossRefGoogle Scholar
  22. 22.
    Abrey LE, Yahalom J, DeAngelis LM (2000) Treatment for primary CNS lymphoma: the next step. J Clin Oncol 18:3144–3150PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2012

Authors and Affiliations

  1. 1.Department of Medicine, Feinberg School of MedicineNorthwestern UniversityChicagoUSA
  2. 2.Department of Pathology, Feinberg School of MedicineNorthwestern UniversityChicagoUSA
  3. 3.Department of Neurology, Feinberg School of MedicineNorthwestern UniversityChicagoUSA

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