Objectives. To describe the features of the clinical picture and estimate the incidence of HIV-associated cerebellar degeneration in patients with progressive cerebellar ataxia. Materials and methods. A total of 377 patients with progressive cerebellar ataxia were examined using brain MRI, the Scale for the Assessment and Rating of Ataxia (SARA), and screening for cognitive impairment using the Montreal Cognitive Assessment test (MoCa). Autoimmune, deficiency, and other causes of ataxia, opportunistic infections, multiple system atrophy, and common forms of hereditary spinocerebellar ataxia were excluded in patients with HIV infection. Results. Five patients (1.3%) with combined ataxia and HIV infection were identified (two men, three women, aged 31–52 years). The median duration of HIV infection was five years and the duration of ataxia was one year. The clinical picture included not only progressive ataxia, but also pyramidal signs, dysphagia, and, less commonly, ophthalmoparesis, dystonia, postural hand tremor, and affective and moderate cognitive impairment. Brain MRI scans revealed signs of olivopontocerebellar atrophy in three patients and degeneration of the cerebellum (mainly the vermis) in two. All patients received combined antiretroviral therapy in various regimens, though despite this, ataxia was progressive. Conclusions. HIV infection is a rare cause of cerebellar degeneration. This diagnosis remains a diagnosis of exclusion. Cerebellar degeneration can occur and progress even after achieving stable remission of HIV infection on the background of highly active antiretroviral therapy.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
World Health Organization, HIV/AIDS.
Wächter, C., Eiden, L. E., Naumann, N., et al., “Loss of cerebellar neurons in the progression of lentiviral disease: effects of CNSpermeant antiretroviral therapy,” J. Neuroinflammation, 13, No. 1, 272 (2016).
González-Scarano, F. and Martín-García, J., “The neuropathogenesis of AIDS,” Nat. Rev. Immunol., 5, No. 1, 69–81 (2005).
Evzel’man, M. A., Snimshchikova, I. A., Koroleva, L. Ya., and Kamchatnov, P. R., “Neurological complications of HIV infection,” Zh. Nevrol. Psikhiatr., 115, No. 3, 89–93 (2015).
Graus, F., Ribalta, T., Abos, J., et al., “Subacute cerebellar syndrome as the first manifestation of AIDS dementia complex,” Acta Neurol. Scand., 81, No. 2, 118–120 (1990).
Tagliati, M., Simpson, D., Morgello, S., et al., “Cerebellar degeneration associated with human immunodeficiency virus infection,” Neurology, 50, No. 1, 244–251 (1998).
Puertas, I., Jiménez-Jiménez, F. J., Gómez-Escalonilla, C., et al., “Progressive cerebellar syndrome as the first manifestation of HIV infection,” Eur. Neurol., 50, No. 2, 120–121 (2003).
Nakamura, S., Wate, R., Shinde, A., et al,” Rinsho Shinkeigaku, 49, No. 10, 651–655 (2009).
Elsheikh, B. H., Maher, W. E., and Kissel, J. T., “Cerebellar atrophy associated with human immunodeficiency virus infection,” Arch. Neurol., 67, No. 5, 634–635 (2010).
Ganos, C., Bernreuther, C., Matschke, J., et al., “Ataxia and HIV: clinicopathologic correlations in a case of HIV-associated cerebellar leukoencephalopathy,” Cerebellum, 11, No. 3, 816–819 (2012).
Anand, K. S., Wadhwa, A., and Garg, J., “A case of cerebellar ataxia associated with HIV infection,” J. Int. Assoc. Provid. AIDS Care, 13, No. 5, 409–410 (2014).
Nagao, S., Kondo, T., Nakamura, T., et al., “A case of human immunodeficiency virus infection with cerebellar ataxia that suggested by an association with autoimmunity,” Rinsho Shinkeigaku, 56, No. 4, 255–259 (2016).
Hoyer, C., Alonso, A., Schlotter-Weigel, B., et al., “HIV-Associated cerebellar dysfunction and improvement with aminopyridine therapy: A case report,” Case Rep. Neurol., 9, No. 2, 121–126 (2017).
Pedroso, J. L., Vale, T. C., Gama, M. T. D., et al., “Cerebellar degeneration and progressive ataxia associated with HIV-virus infection,” Parkinsonism Relat. Disord., 54, 95–98 (2018).
Paul, R., Cohen, R., Navia, B., and Tashima, K., “Relationships between cognition and structural neuroimaging findings in adults with human immunodeficiency virus type-1,” Neurosci. Biobehav. Rev., 26, No. 3, 353–359 (2002).
Krebs, F. C., Ross, H., McAllister, J., and Wigdahl, B., “HIV-1-associated central nervous system dysfunction,” Adv. Pharmacol., 49, 315–385 (2000).
Klunder, A. D., Chiang, M. C., Dutton, R. A., et al., “Mapping cerebellar degeneration in HIV/AIDS,” Neuroreport, 19, No. 17, 1655–1659 (2008).
Weis, S., Neuhaus, B., and Mehraein, P., “Activation of microglia in HIV-1 infected brains is not dependent on the presence of HIV-1 antigens,” Neuroreport, 5, No. 12, 1514–1516 (1994).
Kallianpur, K. J., Shikuma, C., Kirk, G. R., et al., “Peripheral blood HIV DNA is associated with atrophy of cerebellar and subcortical gray matter,” Neurology, 80, No. 19, 1792–1799 (2013).
Abe, H., Mehraein, P., and Weis, S., “Degeneration of the cerebellar dentate nucleus and the inferior olivary nuclei in HIV-1-infected brains: a morphometric analysis,” Acta Neuropathol., 92, No. 2, 150–155 (1996).
Cheng, X., Mukhtar, M., Acheampong, E. A., et al., “HIV-1 Vpr potently induces programmed cell death in the CNS in vivo [published correction appears in DNA Cell. Biol., 26, No. 3, 192 (2007); Cheng, Xiandong [corrected to Cheng, Xiaodong]],” DNA Cell Biol., 26, No. 2, 116–131 (2007).
Savio, T. and Levi, G., “Neurotoxicity of HIV coat protein gp120, NMDA receptors, and protein kinase C: a study with rat cerebellar granule cell cultures,” J. Neurosci. Res., 34, No. 3, 265–272 (1993).
Orsini, M. J., Debouck, C. M., Webb, C. L., and Lysko, P. G., “Extracellular human immunodeficiency virus type 1 Tat protein promotes aggregation and adhesion of cerebellar neurons,” J. Neurosci., 16, No. 8, 2546–2552 (1996).
Reiss, K. A., Bailey, J. R., Pham, P. A., and Gallant, J. E., “Raltegravir-induced cerebellar ataxia,” AIDS, 24, No. 17, 2757 (2010).
Author information
Authors and Affiliations
Corresponding author
Additional information
Translated from Zhurnal Nevrologii i Psikhiatrii imeni S. S. Korsakova, Vol. 123, No. 5, pp. 123–130, May, 2023.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Nuzhnyi, E.P., Brsikyan, L.A., Fedotova, E.Y. et al. Cerebellar Degeneration Associated with HIV Infection. Neurosci Behav Physi 53, 1391–1396 (2023). https://doi.org/10.1007/s11055-023-01532-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11055-023-01532-3