I've waited two days to say anything after my Cleveland Clinic Visit for the chest and back pain, as well as my current and re-accuring problems with balance and walking. I've gone 12 years without a diagnosis. 12 long years of misdiagnosis, pain killers and medication that didn't work and the brush off when nothing could be found. It's been emotionally exhaustive. I've currently been with a home visiting nurse, a home physical therapist and a social worker. I've also was fortunate enough to get an adjustable hospital bed, which I love by the way. But it has been unbearable since the middle of January.
HIV-Associated Vacuolar Myelopathy
OverviewVacuolar myelopathy is the most common chronic myelopathy associated with HIV infection. HIV-associated vacuolar myelopathy 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 infections or malignancies of the central or peripheral nervous system (eg, cytomegalovirus, progressive multifocal leukoencephalopathy, lymphoma).
For patient education information, see the Immune System Center, Dementia Center, and Sexually Transmitted Diseases Center, as well as Dementia Due to HIV Infection and HIV/AIDS.
PathophysiologySeveral 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. Transgenic mice that express HIV gene products in oligodendrocytes develop clinical and histologic features that resemble the human disease.
Although direct HIV infection of astrocytes and neurons 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.
Epidemiology and PrognosisBefore 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.
The prognosis in HIV patients with vacuolar myelopathy is poor.
Arm function is usually normal except for advanced vacuolar myelopathy.
Vacuolar myelopathy is often associated with AIDS dementia complex and peripheral neuropathy. In such cases, patients have cognitive decline and distal limb pain and numbness.
Physical examination:The following may be noted on physical examination:
- Slowly progressive spastic paraparesis
- Hyperreflexia and extensor plantar responses
- Sensory ataxia
- Rarely, asymmetric features and involvement of upper extremities
Diagnostic ConsiderationsThe differential diagnosis includes neurosyphilis. Other problems to be considered in the differential diagnosis of HIV-related vacuolar myelopathy include the following:
- Herpes simplex virus infection
- Cytomegalovirus infection
- Cervical disk syndromes
- HIV-1 associated conditions (eg, Kaposi sarcoma, lymphoma, toxoplasmosis) in the spinal canal
- Mycobacterium tuberculosis infection
- Compressive myelopathy (eg, tumor, abscess, herniated disc, arteriovenous malformation)
- Human T-cell lymphotropic virus type 1 (HTLV-1)–associated myelopathy/tropical spastic paraparesis (can co-exist with HIV infection)
- Spinal epidural abscess in HIV-infected parenteral drug usersDiagnostic Tests
Serum studies can determine vitamin B-12 and folic acid levels. In patients with borderline low B-12 levels, elevated homocysteine and methylmalonic acid levels are better indicators of a deficiency. B-12 levels are usually normal in vacuolar myelopathy.
CT scan results are often noncontributory but may reveal unsuspected coexisting conditions such as extramedullary or intramedullary infections, neoplasms, degenerative disk disease, or degenerative joint disease of the spine.
Magnetic Resonance Imaging:
Spinal cord atrophy is the most common abnormal finding involving the thoracic cord with or without cervical cord involvement. T2-weighted MRI often shows symmetric nonenhancing high-signal areas, which are present on multiple contiguous slices and are usually symmetrical; these may result from extensive vacuolation.[3, 4, 5]
- Anneken K, Fischera M, Evers S. Recurrent vacuolar myelopathy in HIV infection. J Infect. Jun 2006;52(6):e181-3. [View Abstract]
- 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. [View Abstract]
- 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. [View Abstract]
- Santosh CG, Bell JE, Best JJ. Spinal tract pathology in AIDS: postmortem MRI correlation with neuropathology. Neuroradiology. Feb 1995;37(2):134-8. [View Abstract]
- Sartoretti-Schefer S, Blattler T, Wichmann W. Spinal MRI in vacuolar myelopathy, and correlation with histopathological findings. Neuroradiology. Dec 1997;39(12):865-9. [View Abstract]
- 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. [View Abstract]
- 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. [View Abstract]
- 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. [View Abstract]
- Bizaare M, Dawood H, Moodley A. Vacuolar myelopathy: a case report of functional, clinical, and radiological improvement after highly active antiretroviral therapy. Int J Infect Dis. Jul 2008;12(4):442-4.[View Abstract]
- 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. [View Abstract]
- 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. [View Abstract]
- 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. [View Abstract]
- Staudinger R, Henry K. Remission of HIV myelopathy after highly active antiretroviral therapy. Neurology. Jan 11 2000;54(1):267-8. [View Abstract]