A healthy 58-year-old male presented with dysarthria, bilateral horizontal nystagmus, right dysmetria, and marked gait ataxia, which had started 6 days before, after falling from a tree. MRI (Figure) revealed lesions in the right cerebellum extending to the peduncle and pons. Thorough laboratory investigations revealed a positive HIV-1 serology with a serum CD4 count of 47 and a positive PCR for JC virus in otherwise normal CSF, establishing the diagnosis of progressive multifocal leukoencephalopathy (PML).
An MRI (1,5 Tesla) of the brain showing a lesion (arrow) in the right cerebellar white matter extending to right cerebellar peduncle and pons. This lesion is hyperintense on T2/Flair weighted image (A, B and D) and hypointense on T1, without mass effect or gadolinium enhancement (F) and with diffusion restriction (E). The rest of the brain parenchyma is within normal limits (C).
PML presentation with lesions confined to the posterior fossa alone is unusual11. Berger JR, Aksamit AJ, Clifford DB, Davis L, Koralnik IJ, James J. Sejvar JJ et al. PML diagnostic criteria: consensus statement from the AAN Neuroinfectious Disease Section. Neurology. 2013;80(15):1430-8. http://dx.doi.org/10.1212/WNL.0b013e31828c2fa1
https://doi.org/10.1212/WNL.0b013e31828c...
,22. Lima MA. Progressive multifocal leukoencephalopathy: new concepts. Arq Neuropsiquiatr. 2013;71(9B):699-702. http://dx.doi.org/10.1590/0004-282X20130154
https://doi.org/10.1590/0004-282X2013015...
and descriptions as the first manifestation of HIV are very rare33. Lima MA, Andrade FV, Etchebehere RM, Silva-Vergara ML. [Progressive multifocal leukoencephalopathy as initial manifestation of acquired immunodeficiency]. Rev Soc Bras Med Trop. 1998;31(6):569-74. Portuguese. http://dx.doi.org/10.1590/S0037-8682199800060001
https://doi.org/10.1590/S0037-8682199800...
.
References
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1Berger JR, Aksamit AJ, Clifford DB, Davis L, Koralnik IJ, James J. Sejvar JJ et al. PML diagnostic criteria: consensus statement from the AAN Neuroinfectious Disease Section. Neurology. 2013;80(15):1430-8. http://dx.doi.org/10.1212/WNL.0b013e31828c2fa1
» https://doi.org/10.1212/WNL.0b013e31828c2fa1 -
2Lima MA. Progressive multifocal leukoencephalopathy: new concepts. Arq Neuropsiquiatr. 2013;71(9B):699-702. http://dx.doi.org/10.1590/0004-282X20130154
» https://doi.org/10.1590/0004-282X20130154 -
3Lima MA, Andrade FV, Etchebehere RM, Silva-Vergara ML. [Progressive multifocal leukoencephalopathy as initial manifestation of acquired immunodeficiency]. Rev Soc Bras Med Trop. 1998;31(6):569-74. Portuguese. http://dx.doi.org/10.1590/S0037-8682199800060001
» https://doi.org/10.1590/S0037-8682199800060001
Publication Dates
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Publication in this collection
June 2015
History
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Received
09 Dec 2014 -
Reviewed
28 Dec 2014 -
Accepted
21 Jan 2015