AUTHOR AND EDITOR INFORMATION

Section 1 of 7  

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

INTRODUCTION

Section 2 of 7  

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

Background

Patients may have more than one type of HIV-related neuromuscular disease. The type of neuromuscular problem is related to the stage of HIV disease. Patients also are susceptible to the same neuromuscular diseases as the general public (eg, carpal tunnel syndrome in an HIV-positive barber or computer operator). A wide spectrum of neuromuscular conditions is associated with HIV infection, including the following:

• Mononeuropathy multiplex
• Acute or chronic, inflammatory, demyelinating polyradiculoneuropathy
• Distal, symmetric, often painful, and predominantly sensory polyneuropathy
• Autonomic neuropathy (eg, erectile dysfunction)
• Antiretroviral-associated neuropathy
• Polyradiculopathy
• HIV myopathy
• Zidovudine myopathy
• HIV wasting syndrome
• Neurotoxin-associated peripheral neuropathy (typically a complication of treatment for associated conditions; antiretrovirals, infections, lymphoma, sarcoma, prophylaxis)
• Nonspecific myalgias occur as part of the flulike illness during seroconversion.

Pathophysiology

Relevant pathophysiologic mechanisms include the following:

• Nutritional deficits (eg, vitamin B12) can be secondary to enteropathies and malabsorption caused by intestinal HIV, cytomegalovirus (CMV), or herpes simplex virus infection.
• Drug toxicity: Zidovudine is associated with myopathy. Didanosine, dideoxycytidine, and stavudine can cause polyneuropathy, possibly in part due to mitochondrial toxicity.
• Autoimmune mechanisms result in inflammatory demyelinating neuropathies, mononeuropathy multiplex, and polymyositis.
• Co-infection with CMV, herpes simplex virus, varicella zoster virus, Mycobacterium avium-intracellulare, Treponema pallidum, and other bacteria can cause neuromuscular complications.
• The likelihood of a particular syndrome correlates with viral load and CD4 lymphocyte counts. Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome) and isolated acute cranial nerve palsies are common at seroconversion. Distal symmetric, often painful sensorimotor polyneuropathy and CMV infection are more common in the late stages of AIDS.
• AIDP and CIDP may be the initial manifestation of disease, related to autoimmune dysfunction. CSF shows pleocytosis and increased protein. Nerve conduction studies (NCSs) and biopsy are compatible with demyelination.
• Mononeuropathy multiplex is an inflammatory response in the early stages of disease. Late MM is typically associated with CMV infection. May appear as IDP or PP. EMG and NCS show axonal degeneration and asymmetric involvement. SDF shows pleocytosis and elevated protein level.
• Progressive polyradiculopathy (PP) is typically associated with CMV and herpes infections. Highly active antiretroviral therapy (HAART) has reduced the incidence of PP. CSF shows pleocytosis and elevated protein level. It typically presents with a cauda equina–like picture, and EMG shows denervation of the lower extremities. NCSs are mildly slow.
• Autonomic neuropathy usually presents with sexual dysfunction, diarrhea, and bladder dysfunction. It can be related to medication effects.
• Myopathy shows proximal weakness and is confirmed with EMG. Elevated CK may also be seen, and muscle biopsy can be helpful, demonstrating necrosis and inflammation. HIV-related myopathy must be differentiated from toxin (AZT) related myopathies.

Frequency

United States

HIV-1 associated neuromuscular complications are still clinically apparent in more than 30% of patients, but certain complications of HIV, such as DSPN, has decreased since the advent of HAART. They can be clinically silent, and many additional neuromuscular abnormalities are detected by EMG/NCSs, biopsy, or autopsy.

• In one study, inflammatory changes, type II fiber atrophy, or denervation were detected in more than half of asymptomatic HIV-seropositive patients without weakness.
• In another report of untreated patients with mild muscle wasting, inflammation and fiber necrosis were found in one third, type II atrophy in more than one half, and denervation in more than three fourths of patients.

Mortality/Morbidity

• Mortality and morbidity are related to the stage of HIV infection.

Age

• Neuropathies are much more common in adults than in the pediatric HIV population.

DIFFERENTIALS

Section 3 of 7  

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

WORKUP

Section 4 of 7  

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

Lab Studies

• Depends on the type of neuromuscular complication that is suspected. A full neuropathy workup for suspected neuropathies, or myopathy workup for suspected myopathies, and can be found in eMedicine Neurology for each specific disorder. Typically, the workup includes serum laboratory testing, CSF examination, EMG/NCSs, and possible nerve and/or muscle biopsy. See HIV-1 Associated Acute/Chronic Inflammatory Demyelinating Polyradiculoneuropathy, HIV-1 Associated Distal Painful Sensorimotor Polyneuropathy, HIV-1 Associated Multiple Mononeuropathies, HIV-1 Associated Myopathies, and HIV-1 Associated Progressive Polyradiculopathy.

Histologic Findings

HIV RNA and/or the virus have been detected in nerve and dorsal root ganglia. Infected cells include satellite and mononuclear cells and occasional dorsal root ganglion neurons. In the skin, epidermal nerve fiber densities are reduced in symptomatic and asymptomatic distal HIV polyneuropathy compared with controls.

TREATMENT

Section 5 of 7  

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

Medical Care

Treatment depends on the specific HIV-related neuromuscular condition encountered and is discussed in the individual topics found in eMedicine Neurology.

HIV-1 Associated Acute/Chronic Inflammatory Demyelinating Polyradiculoneuropathy

HIV-1 Associated Distal Painful Sensorimotor Polyneuropathy

HIV-1 Associated Multiple Mononeuropathies

HIV-1 Associated Myopathies

HIV-1 Associated Progressive Polyradiculopathy

MISCELLANEOUS

Section 6 of 7  

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

Medical/Legal Pitfalls

• Some HIV-1 associated neuromuscular complications, for example, CMV polyradiculopathy, are often rapidly progressive and quickly lead to death unless expeditiously treated. While its clinical and cerebrospinal fluid patterns are fairly typical, the clinician must be careful not to ascribe progression of pain and weakness to a chronic painful distal neuropathy without evaluating other causes by electromyogram/nerve conduction study and cerebrospinal fluid analysis. Failure to do so may be seen as a deviation from standard medical care.

REFERENCES

Section 7 of 7

• Authors and Editors
• Introduction
• Differentials
• Workup
• Treatment
• Miscellaneous
• References

• Chariot P, Gherardi R. Myopathy and HIV infection. Curr Opin Rheumatol. Nov 1995;7(6):497-502. [Medline].
• Dalakas MC, Semino-Mora C, Leon-Monzon M. Mitochondrial alterations with mitochondrial DNA depletion in the nerves of AIDS patients with peripheral neuropathy induced by 2'3'-dideoxycytidine (ddC). Lab Invest. 2001;81:1537-1544. [Medline].
• Gendelman HE, Lipton SA, Epstein L. The Neurology of AIDS. New York: Chapman & Hall;1998.
• Herrmann DN, McDermott MP, Henderson D. Epidermal nerve fiber density, axonal swellings and QST as predictors of HIV distal sensory neuropathy. Muscle & Nerve. 2004;29:420-427. [Medline].
• Luciano CA, Pardo CA, McArthur JC. Recent developments in the HIV neuropathies. Curr Opin Neurol. Jun 2003;16(3):403-9. [Medline].
• Mancall E, ed. Neuromuscular Disorders in HIV-1 Infection. Continuum. Lippincott Williams & Wilkins;6 number 5 Part A:2000:73-79.
• Maschke M, Kastrup O, Esser S, et al. Incidence and prevalence of neurological disorders associated with HIV since the introduction of highly active antiretroviral therapy (HAART). J Neurol Neurosurg Psychiatry. Sep 2000;69(3):376-80. [Medline].
• Said G, Saimont AG, Lacroix C. Neurological Complications of HIV and AIDS. Philadelphia, Pa: WB Saunders;1998.
• Simpson DM, Olney RK. Peripheral neuropathies associated with human immunodeficiency virus infection. Neurol Clin. Aug 1992;10(3):685-711. [Medline].
• de Gans J, Portegies P. Neurological complications of infection with human immunodeficiency virus type 1. A review of literature and 241 cases. Clin Neurol Neurosurg. 1989;91(3):199-219. [Medline].

Article Last Updated: May 3, 2006