Skip to main content

Advertisement

Log in

Signs of rapidly progressive dementia in a case of intravascular lymphomatosis

  • ORIGINAL PAPER
  • Published:
European Archives of Psychiatry and Clinical Neuroscience Aims and scope Submit manuscript

Abstract

Intravascular lymphomatosis (IVL), a rare type of non–Hodgkin’s lymphoma, is an uncommon cause of progressive dementia, usually followed by death within a few months of onset of clinical disease. Often this aggressive tumor is only diagnosed at autopsy, because of misleading clinical features mimicking a broad spectrum of syndromes and the absence of circulating lympoma cells in the blood, bone marrow or cerebrospinal fluid in many cases. Here we present IVL in a 78–year–old woman with findings leading to the clinical diagnosis of vascular dementia with sudden beginning and positive 14–3–3 protein in the CSF, commonly reported in Creutzfeldt–Jakob disease (CJD).

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Beristein X, Azzarelli B (2002) The neurological masquerade of intravascular lymphomatosis. Arch Neurol 59:439–443

    Article  PubMed  Google Scholar 

  2. Bhawan J, Wolff SM, Ucci AA, et al. (1985) Malignant lymphoma and malignant angioendotheliomatosis: one disease. Cancer 55:570–576

    PubMed  Google Scholar 

  3. Carroll TJ, Schleper RL, Goeken JA, Kemp JD (1986) Neoplastic angioendotheliomatosis: Immunopathologic and morphologic evidence for intravascular malignant lymphomatosis. Am J Clin Pathol 85:169–175

    PubMed  Google Scholar 

  4. Chapin JE, Kornfeld M, Mandler RN (1995) Neurologic manifestations of intravascular lymphomatosis. Acta Neurol Scand 91:494–499

    PubMed  Google Scholar 

  5. DiGiuseppe JA, Nelson WG, Seifter EJ, et al. (1994) Intravascular lymphomatosis: A clinicopathologic study of 10 cases and assessment of response to chemotherapy. J Clin Oncol 12:2573–2579

    PubMed  Google Scholar 

  6. Drlicek M, Grisold W, Liszka U, et al. (1991) Angiotropic lymphoma (malignant angioendotheliomatosis) presenting with rapidly progressive dementia. Acta Neuropathol 82:533–535

    Article  PubMed  Google Scholar 

  7. Fredericks RK, Walker FO, Elster A, Challa V (1991) Angiotropic intravascular large-cell lymphoma (malignant angioendotheliomatosis): report of a case and review of the literature. Surg Neurol 35:218–223

    PubMed  Google Scholar 

  8. Fulling KH, Gersell DJ (1983) Neoplastic angioendotheliomatosis. Cancer 51:1107–1118

    PubMed  Google Scholar 

  9. Glass J, Hochberg FH, Miller DC (1993) Intravascular lymphomatosis. A systemic disease with neurologic manifestations. Cancer 71:3156–3164

    PubMed  Google Scholar 

  10. Kitagawa M, Matsubara O, Song S-Y, et al. (1985) Neoplastic Angioendotheliosis. Cancer 56:1134–1143

    PubMed  Google Scholar 

  11. Koizumi M, Nishimura M, Yokota A, et al. (2001) Successful treatment of intravascular lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation. Bone Marrow Transplantation 27:1101–1103

    Article  PubMed  Google Scholar 

  12. Lemestra AW, van Meegen MT, Vreyling JP, et al. (2000) 14-3-3 testing in diagnosing Creutzfeldt-Jakob disease. A prospective study in 112 patients. Neurology 55:514–516

    PubMed  Google Scholar 

  13. Lie JT (1992) Malignant angioendotheliomatosis (intravascular lymphomatosis) clinically simulating primary angiitis of the central nervous system. Arthritis Rheum 35:831–834

    PubMed  Google Scholar 

  14. Liszka U, Drlicek M, Hitzenberger P, et al. (1994) Intravascular lymphomatosis: a clinicopathological study of three cases. J Cancer Res Clin Oncol 120:164–168

    PubMed  Google Scholar 

  15. Lopez OL, Rabin BS, Huff FJ, et al. (1992) Serum antibodies in patients with Alzheimer’s disease and vascular dementia and nondemented control subjects. Stroke 23:1078–1083

    PubMed  Google Scholar 

  16. Möller HJ, Hartmann A, Kessler C, et al. (2001) Naftidrofuryl in the treatment of vascular dementia. Eur Arch Psychiatry Clin Neurosci 251:247–254

    PubMed  Google Scholar 

  17. Molina A, Lombard C, Donlon T, et al. (1990) Immunohistochemical and cytogenetic studies indicate that malignant angioendotheliomatosis is a primary intravascular (angiotropic) lymphoma. Cancer 66:474–479

    PubMed  Google Scholar 

  18. Murase T, Nakamura S, Tashiro K, et al. (1997) Malignant histiocytosis-like B-cell lymphoma,a distinct pathologic variant of intravascular lymphomatosis: a report of five cases and review of literature. Br J Haematol 99:656–664

    Article  PubMed  Google Scholar 

  19. Otrakji CL, Voigt W, Amador A, et al. (1988) Malignant angioendotheliomatosis – a true lymphoma: A case of intravascular malignant lymphomatosis studied by southern blot hybridization analysis. Human Pathol 19:475–478

    Google Scholar 

  20. Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichenberger N, Julien J, Vital C, Ghetti B, Gambetti P, Kretzschmar HA (1999) Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46:224–233

    PubMed  Google Scholar 

  21. Roux S, Bandt M De, Palazzo E, Vachon F, Kahn MF (1995) Angiotropic large cell lymphoma with mononeuritis multiplex mimicking systemic vasculitis. J Neurol Neurosurg Psychiatry 58:363–366

    PubMed  Google Scholar 

  22. Sepp N, Schuler G, Romani N, et al. (1990) Intravascular lymphomatosis (angioendotheliomatosis): Evidence for a T-cell origin in two cases. Human Pathol 21:1051–105

    Article  Google Scholar 

  23. Sheibani K, Battifora H, Winberg CD, et al. (1986) Further evidence that “malignant angioendotheliomatosis” is an angiotropic large cell lymphoma. N Engl J Med 314:943–948

    PubMed  Google Scholar 

  24. Sokol DS, Azzarelli B, Smith RR, et al. (1998) Primary intravascular lymphomatosis associated with mycobacterium marinum. J Neuroimag 8:47–49

    Google Scholar 

  25. Song DK, Boulis NM, McKeever PE, Quint DJ (2002) Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis. AJNR Am J Neuroradiol 23:239–242

    PubMed  Google Scholar 

  26. Strouth JC, Donahue S, Ross A, Aldred AV (1965) Neoplastic angioendotheliosis. Neurology 15:644–648

    PubMed  Google Scholar 

  27. Tateyama H, Eimoto T, Tada T, et al. (1991) Congenital angiotropic lymphoma (intravascular lymphomatosis) of T-cell type. Cancer 67:2131–2136

    PubMed  Google Scholar 

  28. Wick MR, Banks PM, McDonald TJ (1981) Angioendotheliomatosis of the nose with fatal systemic dissemination. Cancer 48:2510–2517

    PubMed  Google Scholar 

  29. Wrotnowski U, Mills SE, Cooper PH (1985) Malignant angioendotheliomatosis. An angiotropic lymphoma? Am J Clin Pathol 83:244–248

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to H. A. Kretzschmar.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Albrecht, R., Krebs, B., Reusche, E. et al. Signs of rapidly progressive dementia in a case of intravascular lymphomatosis. Eur Arch Psychiatry Clin Neurosci 255, 232–235 (2005). https://doi.org/10.1007/s00406-004-0551-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00406-004-0551-9

Key words

Navigation