Abstract
Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating disease of the central nervous system, which occurs in immunosuppressed individuals. This disease is caused by a reactivation of the polyomavirus JC (JCV). Clinical presentation can be variable from patient to patient as lesions can occur anywhere in the CNS white matter; however, they appear to spare the optic nerves and the spinal cord. The authors present a case of PML in the setting of acquired immunodeficiency syndrome (AIDS) who developed PML lesions in the spinal cord, discovered during the postmortem examination. This finding is significant because PML has recently been diagnosed in patients with multiple sclerosis (MS) treated with the novel immunomodulatory medication natalizumab. Indeed, spinal cord lesions are frequent in MS. Therefore clinicians should be aware that in addition to the brain, PML may also affect the spinal cord white matter.
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References
Bauer W, Chamberlin W, Horenstein S (1969). Spinal demyelination in progressive multifocal leukoencephalopathy. Neurology 18: 287–288.
Berger JR, Koralnik IJ (2005). Progressive multifocal leukoencephalopathy and natalizumab-unforeseen consequences. N Engl J Med, 353: 414–416.
Kleinschidt-Demasters BK, Tyler KL (2005). Progressive multifocal leukoencephalopathy complicating treatment with natalizumab and interferon beta 1-a for multiple sclerosis. N Engl J Med. 353: 369–374.
Koralnik IJ (2006). Progressive multifocal leukoencephalopathy revisited: has the disease outgrown its name? Ann Neurol, 60: 162–173.
Langer-Gould A, Atlas SW, Gree AJ, Bollen AW, Pelletier D (2005). Progressive multifocal leukoencephalopathy in a patient treated with natalizumab. N Engl J Med 353: 375–381.
Shintaku M, Matsumoto R, Sawa H, Nagashima K (2000). Infection with JC virus and possible dysplastic ganglion-like transformation of the cerebral cortical neurons in a case of progressive multifocal leukoencephalopathy. J Neuropathol Exp Neurol, 59: 921–929.
Von Einsiedel RW, Fife TD, Aksamit AJ, Cornford ME, Secor DL, Tomiyasu U, Itabashi HH, Vinters HV (1993). Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature. J Neurol, 240: 391–406.
Weber T, Trebst C, Frye S, et al (1997). Analysis of the systemic and intrathecal humoral immune response in progressive multifocal leukoencephalopathy. J Infect Dis 176: 250–254.
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This work was supported by NIH R01 NS/AI 041198 and 047029, and the Ellen R. Cavallo research fund to IJK.
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Bernal-Cano, F., Joseph, J.T. & Koralnik, I.J. Spinal cord lesions of progressive multifocal leukoencephalopathy in an acquired immunodeficiency syndrome patient. Journal of NeuroVirology 13, 474–476 (2007). https://doi.org/10.1080/13550280701469178
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DOI: https://doi.org/10.1080/13550280701469178