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Abstract
RÉSUMÉ
La leucoencéphalopathie multifocale progressive est une affection démyélinisante subaiguë du système nerveux central liée à une infection opportuniste par le virus JC, survenant quasi-exclusivement dans un contexte de déficit de l’immunité cellulaire. Elle doit être systématiquement évoquée devant toute situation neurologique évocatrice, y compris en l’absence de SIDA surtout au cours d’une leucémie lymphoïde chronique et un traitement aux immunosuppresseurs. Il faut savoir répéter une PCR du virus qui peut être négative au début dans le liquide céphalorachidien. Nous reportons un cas de LEMP chez un homme âgé de 67 ans porteur d’une leucémie lymphoïde chronique traitée par l’association Rituximab – Fludarabine – Cyclophosphamide. La recherche du génome viral par PCR dans le LCR s’est révélée positive. Sous Cidofovir, l’état clinique du patient est resté stable dans un premier temps avant de se dégrader.
ABSTRACT
Progressive multifocal leukoencephalopathy is a subacute demyelinating disease of the central nervous system. It is related to an opportunistic infection caused by the JC virus, occurring almost exclusively in a context of cellular immune deficiency. It should be suspected in case of any suggesting neurological condition, even in the absence of AIDS, especially when treatment with immunosuppressors is associated. It is important to repeat the PCR of the JC virus, which can be negative at the onset of the disease. We report one case of Progressive multifocal leukoencephalopathy in 67 years old male patient with chronic lymphoid leukemia treated with Rituximab – Fludarabine – Cyclophosphamide. PCR search in the spinal fluid was positive. He patient was treated with cidofovoir and its state worsened after an initial stabilization.
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References
- Gallia GL, Houff SA, Major EO, Khalili K. 1997. Review: JC virus infection of lymphocytes-revisited. J Infect Dis, 176:1603-1609.
- Gasnault J, Taoufik Y. 2006. New trends in progressive multifocal leukoencephalopathy. Rev Neurol, 162:43-56.
- Knowles WA. 2006. Discovery and epidemiology of the human polyomaviruses BK virus (BKV) and JC virus (JCV). Adv Exp Med Biol, 577:19-45.
- Koralnik IJ, Boden D, Mai VX, Lord CI, Letvin NL. 1999. JC virus DNA load in patient with and without progressive multifocal leukoencephalopathy. Neurology, 15:253-260.
- Cinque P, Koralnik IJ, Clifford DB. 2003. The evolving face of human immunodeficiency virus-related progressive multifocal leukoencephalopathy: defining a consensus terminology. J Neurovirol, 9 (Suppl 1):88–92.
- Itoh K, Kano T, Nagashio C, Mimori A, Kinoshita M, Sumiya M. 2006. Progressive multifocal leukoencephalopathy in patients with systemic lupus erythematosus. Arthritis Rheum, 54:1020–1022.
- Pagnoux C, Hayem G, Roux F, Rouidi SA, Palazzo E, Henin D, et al. 2003. JC virus leukoencephalopathy complicating Wegener’s granulomatosis. Joint Bone Spine, 70:376–379.
- Brooks BR, Walker DL. 1984. Progressive multifocal leukoencephalopathy. Neurol Clin, 2:299-313.
- Hou J, Major EO. 2000. Progressive multifocal leukoencephalopathy: JC virus induced demyelination in the immune compromised host. J Neurovirol, 6(Suppl 2):S98–S100.
- Brooks BR, Walker DL. 1984. Progressive multifocal leukoencephalopathy. Neurol Clin, 2:299-313.
- Bienfait HP, Louwerse ES, Portegies P, Van Der Meer JT. 1998. Progressive multifocal leukoencephalopathy presenting as a solitary gray matter lesion. J Neurol, 245:557-558.
- Berger JR, Kaszovitz B, Post MJ, Dickinson G. 1987. Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. A review of the literature with a report of 16 cases. Ann Intern Med, 107:78-87.
- Ferrari S, Monaco S, Morbin M et al. 1998. HIV-associated PML presenting as epilepsia partialis continua. J Neurol Sci, 161:180-184.
- Hamilton RL, Martinez AJ. 1998. Case of the month: September1997 – A 26 year old woman with new onset seizures. Brain Pathol, 8:239-240.
- Moulignier A, Mikol J, Pialoux G, Fénelon G, Gray F, Thiebaut JB. 1995. AIDS-associated progressive multifocal leukoencephalopathy revealed by new-onset seizures. Am J Med, 99: 64-68.
- Bhatia KP, Morris JH, Frackowiac RS. 1996. Primary progressive multifocal leukoencephalopathy presenting as an extrapyramidal syndrome. J Neurol, 243:91-95
- Alafuzoff I, Hartikainen P, Hanninen T et al. 1999. Rapidly progressive multifocal leukoencephalopathy with substantial cell-mediated inflammatory response and with cognitive decline of non-Alzheimer type in a 75-year-old female patient. Clin Neuropathol, 18:113-123.
- Piccolo I, Causarano R, Sterzi R et al. Chorea in patients with AIDS. 1999. Acta Neurol Scand, 100:332-336.
- Post JD, Yiannoutsos C, Simpson D et al. 1999. Progressive multifocal leukoencephalopathy in AIDS: are there any MR findings useful to patient management and predictive of patient survival? AJNR Am J Neuroradiol, 20:1896-1906.
- Bosch J, Sumalla J, Mauleon A et al. 1999. Progressive multifocal leukoencephalopathy in elderly immunocompetent patients. Report of 2 cases. Rev Neurol, 29:133-137.
- Case records of the Massachusetts general hospital. Weekly clinicopathological exercises. 1995. Case 20-1995. A 66 year-old man with a history of rheumatoid arthritis treated with adrenocorticosteroids, with the development of aphasia and right-sided weakness. N Engl J Med, 332:1773-1780.
References
Gallia GL, Houff SA, Major EO, Khalili K. 1997. Review: JC virus infection of lymphocytes-revisited. J Infect Dis, 176:1603-1609.
Gasnault J, Taoufik Y. 2006. New trends in progressive multifocal leukoencephalopathy. Rev Neurol, 162:43-56.
Knowles WA. 2006. Discovery and epidemiology of the human polyomaviruses BK virus (BKV) and JC virus (JCV). Adv Exp Med Biol, 577:19-45.
Koralnik IJ, Boden D, Mai VX, Lord CI, Letvin NL. 1999. JC virus DNA load in patient with and without progressive multifocal leukoencephalopathy. Neurology, 15:253-260.
Cinque P, Koralnik IJ, Clifford DB. 2003. The evolving face of human immunodeficiency virus-related progressive multifocal leukoencephalopathy: defining a consensus terminology. J Neurovirol, 9 (Suppl 1):88–92.
Itoh K, Kano T, Nagashio C, Mimori A, Kinoshita M, Sumiya M. 2006. Progressive multifocal leukoencephalopathy in patients with systemic lupus erythematosus. Arthritis Rheum, 54:1020–1022.
Pagnoux C, Hayem G, Roux F, Rouidi SA, Palazzo E, Henin D, et al. 2003. JC virus leukoencephalopathy complicating Wegener’s granulomatosis. Joint Bone Spine, 70:376–379.
Brooks BR, Walker DL. 1984. Progressive multifocal leukoencephalopathy. Neurol Clin, 2:299-313.
Hou J, Major EO. 2000. Progressive multifocal leukoencephalopathy: JC virus induced demyelination in the immune compromised host. J Neurovirol, 6(Suppl 2):S98–S100.
Brooks BR, Walker DL. 1984. Progressive multifocal leukoencephalopathy. Neurol Clin, 2:299-313.
Bienfait HP, Louwerse ES, Portegies P, Van Der Meer JT. 1998. Progressive multifocal leukoencephalopathy presenting as a solitary gray matter lesion. J Neurol, 245:557-558.
Berger JR, Kaszovitz B, Post MJ, Dickinson G. 1987. Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. A review of the literature with a report of 16 cases. Ann Intern Med, 107:78-87.
Ferrari S, Monaco S, Morbin M et al. 1998. HIV-associated PML presenting as epilepsia partialis continua. J Neurol Sci, 161:180-184.
Hamilton RL, Martinez AJ. 1998. Case of the month: September1997 – A 26 year old woman with new onset seizures. Brain Pathol, 8:239-240.
Moulignier A, Mikol J, Pialoux G, Fénelon G, Gray F, Thiebaut JB. 1995. AIDS-associated progressive multifocal leukoencephalopathy revealed by new-onset seizures. Am J Med, 99: 64-68.
Bhatia KP, Morris JH, Frackowiac RS. 1996. Primary progressive multifocal leukoencephalopathy presenting as an extrapyramidal syndrome. J Neurol, 243:91-95
Alafuzoff I, Hartikainen P, Hanninen T et al. 1999. Rapidly progressive multifocal leukoencephalopathy with substantial cell-mediated inflammatory response and with cognitive decline of non-Alzheimer type in a 75-year-old female patient. Clin Neuropathol, 18:113-123.
Piccolo I, Causarano R, Sterzi R et al. Chorea in patients with AIDS. 1999. Acta Neurol Scand, 100:332-336.
Post JD, Yiannoutsos C, Simpson D et al. 1999. Progressive multifocal leukoencephalopathy in AIDS: are there any MR findings useful to patient management and predictive of patient survival? AJNR Am J Neuroradiol, 20:1896-1906.
Bosch J, Sumalla J, Mauleon A et al. 1999. Progressive multifocal leukoencephalopathy in elderly immunocompetent patients. Report of 2 cases. Rev Neurol, 29:133-137.
Case records of the Massachusetts general hospital. Weekly clinicopathological exercises. 1995. Case 20-1995. A 66 year-old man with a history of rheumatoid arthritis treated with adrenocorticosteroids, with the development of aphasia and right-sided weakness. N Engl J Med, 332:1773-1780.