Progressive multifocal leucoencephalopathy limited to the posterior fossa as first manifestation of HIV infection

Leucoenfalopatia multifocal progressiva limitada à fossa posterior como manifestação inicial de infecção por HIV

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).

Figure
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 unusual^1,2 and descriptions as the first manifestation of HIV are very rare³.

References

• ¹
  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.0b013e31828c2fa1
• ²
  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/10.1590/0004-282X20130154
• ³
  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/S0037-8682199800060001

Publication Dates

• Publication in this collection
  June 2015

History

• Received
  09 Dec 2014
• Reviewed
  28 Dec 2014
• Accepted
  21 Jan 2015