AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

HIV infection is now an important cause of inflammatory demyelinating neuropathies. Exclude HIV infection in any patient who presents with these conditions. The history, physical examination, and course resemble those of HIV-seronegative patients. In two case series, up to 30% of patients presenting with Guillian-Barré syndrome (GBS) were found to be HIV positive.

Pathophysiology

While many of the clinical manifestations of HIV infection are caused by immune deficiency, others, such as thrombocytopenic purpura, are a manifestation of the general state of immune activation evidenced by T-cell activation and hypergammaglobulinemia, which can result in autoimmunity.

Similar mechanisms are responsible for the increased incidence of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) compared with the HIV-seronegative population. Several in vitro studies have shown that the binding of HIV envelope protein Gp120 to chemokine receptors leads to activation of caspase pathway and axonal degeneration. Perineuronal Schwann cells may have a neuroprotective role.

Frequency

United States

Two studies demonstrate that inflammatory demyelinating neuropathies are diagnosed in one third of HIV-seropositive patients referred for peripheral nerve diseases.

While CIDP was much more frequent than AIDP in two European and North American reports, the converse was true in a larger series of African patients: 16 AIDP but no CIDP patients were found.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

While AIDP presents most often at the time of seroconversion, it also can occur in late AIDS. CIDP can occur at any phase of HIV disease.

AIDP progresses over days to less than 4 weeks.

CIDP has a chronic progression for more than 2 months or a relapsing and/or remitting course.

HIV-GBS has been associated with more frequent recurrent episodes or the development of CIDP as compared to HIV-seronegative GBS.

Characteristic features include the following:

• Distal and proximal weakness
• Mild sensory complaints and pain
• Respiratory paralysis in AIDP
• Cranial neuropathy (VII, X, XII) in AIDP and CIDP

Physical

AIDP and CIDP present with an acute, subacute, chronic, or relapsing and/or remitting course, with the following signs:

• Distal and proximal weakness
• Mildly impaired sensation
• Autonomic dysfunction with cardiac arrhythmia, urinary retention, and blood pressure instability
• Hyporeflexia or areflexia

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Acute or subacute neuropathies of other causes
Hereditary Motor and Sensory Neuropathies
Lumbosacral disk syndromes
Lumbosacral spondylosis
Paraneoplastic neuropathy
Paraneoplastic sensory neuropathy
Paraproteinemic Neuropathy

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Cerebrospinal fluid
• • In HIV-GBS, CSF may demonstrate a mild lymphocytic pleocytosis (10-50 cells/µL), which helps differentiate it from GBS in HIV-seronegative patients. Similarly, the presence of pleocytosis in a patient with AIDP warrants consideration of underlying HIV disease.
  • Significantly elevated protein is another characteristic finding.
• Electromyography nerve conduction studies (EMG/NCS) - Findings consistent with a primarily demyelinating process, often with secondary axonal degeneration
• • Reduced motor nerve conduction velocity
  • Conduction block or temporal dispersion
  • Prolonged F-wave latencies
  • Prolonged distal latencies
  • Variable reduction of compound muscle action potential amplitudes
  • Variable spontaneous activity due to axonal degeneration

Histologic Findings

Segmental demyelination, mononuclear perivascular infiltration in the endoneurium, perineurial edema, variable Wallerian degeneration, viral particles, and RNA usually are not present.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

• Treatment is similar to that of HIV-seronegative patients. Intravenous immunoglobulin (IVIG) and plasmapheresis (plasma exchange) are preferred to immunosuppression. In patients with CIDP, oral prednisone can also be used until clinical improvement, and then it can be titrated off.
• • During a 7- to 10-day period, 4-5 plasmapheresis procedures may be performed as described in standard protocols. Potential complications include autonomic instability, hypercalcemia, and bleeding caused by depletion of clotting factors.
  • Decide among IVIG, plasmapheresis, and steroids on an individual basis. The cost of IVIG, the invasive nature of plasmapheresis, and the long-term side effects of steroids are important points to consider when making a selection.
• In advanced HIV disease, with CD4⁺ count of less than 100 cells/µL, opportunistic infections such as CMV may need to be investigated and empiric therapy instituted.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

The goal is to diminish the general state of immune activation as evidenced by T-cell activation and hypergammaglobulinemia, which may be responsible for autoimmunity.

Drug Category: Blood products

IVIG is an effective treatment of autoimmune neuropathies in general. It reduces the duration of hospitalization and the need for mechanical ventilation.

Drug Category: Glucocorticoids

These agents modify the body's immune response to diverse stimuli. Likely mechanisms of action include inhibition of synthesis and/or secretion of TNF-alpha, IL-6, IL-2, and IFN-gamma and modulation of serum and leukocyte-bound levels of cell adhesion molecules.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Prognosis

• The prognosis of HIV-1 associated AIDP and CIDP is similar to that of HIV-seronegative varieties discussed in separate eMedicine articles (Acute Inflammatory Demyelinating Polyradiculoneuropathy, Chronic Inflammatory Demyelinating Polyradiculoneuropathy).
• In one study comparing GBS in HIV-seropositive patients to HIV-seronegative patients in the ICU requiring ventilation, no significant difference existed between the two groups in days spent in the ICU or days ventilated, particularly if patient with HIV-associated GBS had a CD4 T-lymphocyte count greater than 200 X 10⁶ cells/L at admission.

Patient Education

• For excellent patient education resources, visit eMedicine's Immune System Center and Sexually Transmitted Diseases Center. Also, see eMedicine's patient education article HIV/AIDS.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Failure to ascertain HIV status in patients carrying these diagnoses. As mentioned, AIDP can occur at HIV seroconversion in what appears to be an otherwise healthy individual.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Bradley WG, Daroff RB, Fenichel GM. Neurological manifestations of human immunodeficiency virus infection in adults. Neurol in Clin Pract. 2004;2:1581-1602.
• Brannagan TH, Zhou Y. HIV-associated Guillain-Barre syndrome. J Neurol Sci. Apr 15 2003;208(1-2):39-42. [Medline].
• Cornblath DR, Hoke A. Recent advances in HIV neuropathy. Curr Opin Neurol. 2006;19(5):446-50. [Medline].
• Cornblath DR, McArthur JC, Kennedy PG, et al. Inflammatory demyelinating peripheral neuropathies associated with human T-cell lymphotropic virus type III infection. Ann Neurol. Jan 1987;21(1):32-40. [Medline].
• Ferrari S, Vento S, Monaco S. Human immunodeficiency virus-associated peripheral neuropathies. Mayo Clin Proc. Feb 2006;81(2):213-9. [Medline].
• Gendelman HE, Lipton SA, Epstein L. The Neurology of AIDS. New York: Chapman & Hall;1998.
• Kumar S, Alexander M, Markandeyulu V. Guillain-Barre syndrome presenting in the anti-HIV seroconversion period. Neurol India. Dec 2003;51(4):559. [Medline].
• Leger JM, Bouche P, Bolgert F, et al. The spectrum of polyneuropathies in patients infected with HIV. J Neurol Neurosurg Psychiatry. Dec 1989;52(12):1369-74. [Medline].
• Said G, Saimont AG, Lacroix C. Neurological Complications of HIV and AIDS. Philadelphia: WB Saunders;1998.
• Schleicher GK, Black A, Mochan A, Richards GA. Effect of human immunodeficiency virus on intensive care unit outcome of patients with Guillain-Barre syndrome. Crit Care Med. Jun 2003;31(6):1848-50. [Medline].
• Simpson DM, Olney RK. Peripheral neuropathies associated with human immunodeficiency virus infection. Neurol Clin. Aug 1992;10(3):685-711. [Medline].
• Thornton CA, Latif AS, Emmanuel JC. Guillain-Barre syndrome associated with human immunodeficiency virus infection in Zimbabwe. Neurology. Jun 1991;41(6):812-5. [Medline].
• Verma A. Epidemiology and clinical features of HIV-1 associated neuropathies. J Peripher Nerv Syst. Mar 2001;6(1):8-13. [Medline].
• Verma S, Micsa E, Estanislao L, Simpson D. Neuromuscular complications in HIV. Curr Neurol Neurosci Rep. Jan 2004;4(1):62-7. [Medline].
• de Gans J, Portegies P. Neurological complications of infection with human immunodeficiency virustype 1. A review of literature and 241 cases. Clin Neurol Neurosurg. 1989;91(3):199-219. [Medline].

Article Last Updated: Mar 13, 2007