Neuromuscular Case Vignettes

Diagnosis: GBS associated with HIV infection

HIV-1 Ab titer was positive HIV RNA was 61,967 copies/mL, CD4 was 189 cells/mL: 

Polyneuropathy is the most common neurological manifestation of HIV infection, with prevalence as high as half but up to two thirds of the patients in some studies 1,2. A variety of polyneuropathies complicate human immunodeficiency virus (HIV) infection or its treatment. A distal predominantly sensory axonal polyneuropathy is the most common type, which could be the result of infection with HIV virus itself. HIV neuropathy was associated with advanced immunosuppression before the era of antiretroviral treatment (ART), but no correlation was found between distal sensory neuropathy and the CD4 count in the ART era 2. Another common cause of distal length dependent neuropathy in the HIV patients is use of antiretrovirals, especially the “d” drugs: zalcitabine (ddC), didanosine (ddI), with stavudine (d4T) being the most toxic 3.  Other possible causes of distal axonal neuropathy in the older HIV patients include diabetes, alcoholism and hepatitis C virus infection.

Inflammatory demyelinating neuropathies, i.e. Guillain–Barre´ syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are also known to complicate GBS even in the earlier stages of the disease.  GBS, can be the first manifestation of HIV infection, and may occur at the time of seroconversion 10. On the other hand, GBS may occur with more advanced disease and lower CD4 count; i.e. in a case series by Brannagan, et al, 4 of 10 patients with HIV+ GBS had CD4 counts between 55 and 190 4. It is recommended that GBS, in HIV infected patients with CD4 cell counts less than 50 should be treated presumptively for cytomegalovirus infection 4. Another important feature of GBS associated with HIV infection is mild CSF pleocytosis, but lack of pleocytosis does not exclude HIV infection 10. HIV-associated GBS can be associated with more frequent recurrent episodes than GBS alone, or with the development of CIDP.

References

1.              Ghosh S, Chandran A, Jansen JP. Epidemiology of HIV-related neuropathy: a systematic literature review. AIDS research and human retroviruses. 2012;28:36-48.

2.              Morgello S, Estanislao L, Simpson D, et al. HIV-associated distal sensory polyneuropathy in the era of highly active antiretroviral therapy: the Manhattan HIV Brain Bank. Archives of neurology. 2004;61:546-551.

3.              Simpson DM, Tagliati M. Nucleoside analogue-associated peripheral neuropathy in human immunodeficiency virus infection. Journal of acquired immune deficiency syndromes and human retrovirology : official publication of the International Retrovirology Association. 1995;9:153-161.

4.           Brannagan TH, 3rd, Zhou Y. HIV-associated Guillain-Barre syndrome. Journal of the neurological sciences.2003;208:39-42.