eMedicine Specialties > Neurology > Neurological Infections

HIV-1 Associated Vacuolar Myelopathy

Author: Niranjan N Singh, MD, DNB, Fellow in Neurophysiology, Department of Neurology, St Louis University School of Medicine
Coauthor(s): Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Associate Program Director, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Contributor Information and Disclosures

Updated: Mar 14, 2007

Introduction

Background

Vacuolar myelopathy is the most common chronic myelopathy associated with HIV infection. It occurs during the late stages of HIV infection, when CD4+ lymphocyte counts are very low, often in conjunction with AIDS dementia complex, peripheral neuropathies, and opportunistic central nervous system and peripheral nervous system infections or malignancies (eg, cytomegalovirus, progressive multifocal leukoencephalopathy, lymphoma).

Pathophysiology

Several hypotheses have been proposed to explain the development of this common complication of HIV-1 infection.

  • One hypothesis is infiltration by HIV-infected mononuclear cells that secrete neurotoxic factors including cytokines, possibly in conjunction with neurotoxic astrocyte factors. A significant amount of scientific support exists for this paradigm.
  • Neurotoxic HIV proteins: Transgenic mice that express HIV gene products in oligodendrocytes develop clinical and histologic features that resemble the human disease.
  • Direct HIV infection of astrocytes and neurons: While this is reported in the brain and dorsal root ganglia, it is not a major feature in vacuolar myelopathy.
  • The impaired ability to utilize vitamin B-12 as a source of methionine in transmethylation metabolism for myelin maintenance in the spinal cord may be a contributing factor.

Frequency

United States

Before the introduction of highly active antiretroviral therapy (HAART), vacuolar myelopathy was seen in 5-20% of adult HIV patients in clinical studies and in 25-55% of adult HIV patients in histologic studies.

Since the introduction of HAART, it is estimated that fewer than 10% of AIDS patients develop HIV myelopathy.

Mortality/Morbidity

Most patients die within 6 months of developing symptoms of myelopathy.

Clinical

History

  • Patients have a history of slow progression, painless leg weakness, stiffness, sensory loss, imbalance, and sphincter dysfunction.
  • Relapsing-remitting courses have also been described.
  • Vacuolar myelopathy is often seen in conjunction with cognitive decline, distal limb pain, and numbness from peripheral neuropathy.
  • Back pain is not a prominent feature.
  • Arm function is usually normal except for advanced vacuolar myelopathy.

Physical

  • Slowly progressive spastic paraparesis
  • Hyperreflexia and extensor plantar responses
  • Sensory ataxia
  • Incontinence
  • Rarely, asymmetric features and involvement of upper extremities
  • Often associated with AIDS dementia complex and neuropathy
  • A discrete sensory level is usually absent; if present, this strongly suggests other causes of myelopathy.

More on HIV-1 Associated Vacuolar Myelopathy

Overview: HIV-1 Associated Vacuolar Myelopathy
Differential Diagnoses & Workup: HIV-1 Associated Vacuolar Myelopathy
Treatment & Medication: HIV-1 Associated Vacuolar Myelopathy
Follow-up: HIV-1 Associated Vacuolar Myelopathy
Multimedia: HIV-1 Associated Vacuolar Myelopathy
References
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References

  1. Anneken K, Fischera M, Evers S. Recurrent vacuolar myelopathy in HIV infection. J Infect. Jun 2006;52(6):e181-3. [Medline].

  2. Chong J, Di Rocco A, Tagliati M, et al. MR findings in AIDS-associated myelopathy. AJNR Am J Neuroradiol. Sep 1999;20(8):1412-6. [Medline].

  3. Di Rocco A, Tagliati M, Danisi F, et al. A pilot study of L-methionine for the treatment of AIDS-associated myelopathy. Neurology. Jul 1998;51(1):266-8. [Medline].

  4. Di Rocco A, Werner P, Bottiglieri T, et al. Treatment of AIDS-associated myelopathy with L-methionine: a placebo-controlled study. Neurology. Oct 12 2004;63(7):1270-5. [Medline].

  5. DiRocco A. HIV-associated myelopathy. Current Treatment Options in Infectious Diseases. Vol 5. 2003:457-465.

  6. Eilbott DJ, Peress N, Burger H, et al. Human immunodeficiency virus type 1 in spinal cords of acquired immunodeficiency syndrome patients with myelopathy: expression and replication in macrophages. Proc Natl Acad Sci U S A. May 1989;86(9):3337-41. [Medline].

  7. Fernandez-Fernandez FJ, de la Fuente-Aguado J, Ocampo-Hermida A. Remission of HIV-associated myelopathy after highly active antiretroviraltherapy. J Postgrad Med. 2004;50(3):195-6. [Medline].

  8. Gendelman HE, Lipton SA, Epstein L. The Neurology of AIDS. New York: Chapman & Hall;1998.

  9. Petito CK, Navia BA, Cho ES, et al. Vacuolar myelopathy pathologically resembling subacute combined degeneration in patients with the acquired immunodeficiency syndrome. N Engl J Med. Apr 4 1985;312(14):874-9. [Medline].

  10. Price RW, Perry SW. HIV, AIDS, and the Brain. New York: Raven Press;1994.

  11. Said G, Saimont AG, Lacroix C. Neurological Complications of HIV and AIDS. Philadelphia, Pa: WB Saunders;1998.

  12. Santosh CG, Bell JE, Best JJ. Spinal tract pathology in AIDS: postmortem MRI correlation with neuropathology. Neuroradiology. Feb 1995;37(2):134-8. [Medline].

  13. Sartoretti-Schefer S, Blattler T, Wichmann W. Spinal MRI in vacuolar myelopathy, and correlation with histopathological findings. Neuroradiology. Dec 1997;39(12):865-9. [Medline].

  14. Staudinger R, Henry K. Remission of HIV myelopathy after highly active antiretroviral therapy. Neurology. Jan 11 2000;54(1):267-8. [Medline].

  15. Tagliati M, Di Rocco A, Danisi F, Simpson DM. The role of somatosensory evoked potentials in the diagnosis of AIDS-associated myelopathy. Neurology. Apr 11 2000;54(7):1477-82. [Medline].

  16. Tyor WR, Glass JD, Baumrind N, et al. Cytokine expression of macrophages in HIV-1-associated vacuolar myelopathy. Neurology. May 1993;43(5):1002-9. [Medline].

  17. 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].

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Further Reading

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Keywords

HIV-1 associated myelopathy, AIDS myelopathy, AIDS dementia complex, peripheral neuropathies, opportunistic central nervous system infections, opportunistic peripheral nervous system infections, central nervous system malignancies, cytomegalovirus, progressive multifocal leukoencephalopathy, lymphoma, HIV-1 infection complications, neuropathy, HAART, highly active antiretroviral therapy

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Contributor Information and Disclosures

Author

Niranjan N Singh, MD, DNB, Fellow in Neurophysiology, Department of Neurology, St Louis University School of Medicine
Niranjan N Singh, MD, DNB is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Coauthor(s)

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Associate Program Director, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

Medical Editor

Michael J Schneck, MD, Associate Professor, Department of Neurology and Neurosurgery, Loyola University Chicago, Stritch School of Medicine
Disclosure: boehringer-ingelheim Honoraria Speaking and teaching; sanofi/bms Honoraria Speaking and teaching; pfizer Honoraria Speaking and teaching; genentech Honoraria Speaking and teaching; ucb pharma Honoraria Speaking and teaching; talecris Consulting fee Other; nmt medical  Independent contractor; NIH Grant/research funds Independent contractor; vernalis Grant/research funds Independent contractor; sanofi Grant/research funds Independent contractor

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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