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Hashemi Eye Care - Neuroscience Institute and Research Center

Bilateral Progressive Vision Loss in Noninfectious HIV Retinopathy without Infectious Retinitis

Zhang J, Hashemi N, Lee AG

Journal of Ophthalmic and Vision Research, 2012

A case of a 59-year-old HIV-positive man on HAART with normal CD4 counts who developed bilateral progressive vision loss, nyctalopia, and dyschromatopsia due to primary HIV retinopathy, diagnosed through multifocal electroretinography despite a normal fundus examination.

6 min read

This case report presents an unusual presentation of noninfectious HIV retinopathy in a 59-year-old man on highly active antiretroviral therapy (HAART) with a normal CD4 count and non-detectable viral load, who developed bilateral progressive vision loss, nyctalopia (night blindness), and dyschromatopsia (color vision changes). Published as a letter in the Journal of Ophthalmic and Vision Research in 2012, this case highlights the importance of electrophysiological testing when clinical examination alone cannot explain visual dysfunction in HIV-positive patients.

Key Findings

  • Primary HIV retinopathy can cause significant vision loss even in patients with normal CD4 counts and non-detectable viral loads on HAART
  • Fundus examination was completely normal, with no cotton wool spots, hemorrhages, or microaneurysms typically associated with HIV retinopathy
  • Multifocal electroretinography (mfERG) was the key diagnostic test, revealing extinguished foveolar response OD and misshapen foveolar response OS with perifoveal changes bilaterally
  • Left-sided relative afferent pupillary defect (RAPD) was present along with light-near dissociation in both eyes, despite normal fundoscopic appearance
  • Best corrected visual acuity was significantly reduced at 20/100 OD and 20/70 OS
  • HIV retinopathy is a diagnosis of exclusion requiring thorough investigation for alternative etiologies before the diagnosis can be made

Background

Human immunodeficiency virus (HIV) can cause a spectrum of ocular complications. The most commonly recognized is opportunistic infectious retinitis, particularly cytomegalovirus (CMV) retinitis, which occurs in severely immunocompromised patients. However, a distinct entity known as noninfectious HIV retinopathy also exists and is characterized by microvascular changes in the retina, including cotton wool spots, microaneurysms, and retinal hemorrhages.

What makes noninfectious HIV retinopathy challenging is that it can cause visual dysfunction even when the fundus appears normal. This has been attributed to damage from evanescent (transient) cotton wool spots that have already healed by the time of examination. Visual loss from noninfectious HIV retinopathy is rarely reported and is typically associated with visual field depression, altered contrast sensitivity, and color vision changes. The condition is a diagnosis of exclusion, requiring that infectious retinitis, intracranial pathology, and other causes of visual loss be ruled out.

The Patient

A 59-year-old HIV-infected Caucasian man presented with:

  • Three-month history of bilateral progressive vision loss
  • Nyctalopia (difficulty seeing at night)
  • Dyschromatopsia (impaired color vision)
  • On HAART with non-detectable viral load and normal CD4 count of 550 cells/mm3
  • Past medical history: Hypertension, gallbladder resection, refractive eye surgery with secondary enhancement 11 years prior
  • Family history: Macular degeneration

Diagnostic Workup

Clinical Examination

  • Best corrected visual acuity: 20/100 OD, 20/70 OS
  • Pupil exam: 5.0 mm in dark, 3.0 mm in light; relative afferent pupillary defect (RAPD) in the left eye; light-near dissociation in both eyes
  • External, motility, and intraocular pressure: Within normal limits
  • Fundoscopic examination: Normal with no evidence of HIV-related retinopathy (no cotton wool spots, hemorrhages, or microaneurysms); cup-to-disc ratios 0.3 bilaterally

Visual Field Testing

Neuroimaging

  • MRI of the brain: Normal except for a small pineal cyst abutting the superior colliculus

Laboratory Studies

  • Blood tests: Negative for neuromyelitis optica, vitamin B12 deficiency, folate deficiency, and infectious etiologies including syphilis
  • Chest radiograph: Normal

Electrophysiological Testing

  • Flash electroretinography (ERG): Normal in both eyes
  • Multifocal electroretinography (mfERG): Extinguished foveolar response OD and misshapen foveolar response OS with many perifoveal changes in both eyes, suggestive of retinopathy

Results

The diagnosis of primary noninfectious HIV retinopathy was made based on the following key findings:

  • Bilateral progressive visual dysfunction (reduced acuity, nyctalopia, dyschromatopsia) in an HIV-positive patient
  • Normal fundoscopic examination without signs of infectious retinitis or typical HIV retinopathy findings
  • Normal CD4 count (550 cells/mm3) and non-detectable viral load, making opportunistic infection unlikely
  • Abnormal mfERG confirming retinal dysfunction at the foveal and perifoveal level despite the normal fundus appearance
  • Thorough negative workup for alternative causes including neuromyelitis optica, nutritional deficiencies, syphilis, and intracranial pathology

The light-near dissociation observed bilaterally could potentially be related to tectum compression from the small pineal cyst abutting the superior colliculus. However, no other signs of dorsal midbrain syndrome were identified, making this an incidental finding rather than the primary cause.

Clinical Significance

This case is unusual for several reasons:

  • Duration and severity of vision loss are atypical for noninfectious HIV retinopathy, which usually produces milder dysfunction
  • Normal CD4 count is noteworthy because HIV retinopathy has traditionally been associated with immunosuppression
  • Left-sided RAPD in the context of a normal fundus is a striking finding that underscores the occult nature of the retinal damage
  • mfERG proved essential for confirming retinal dysfunction when standard clinical examination was unremarkable

The mechanism of visual dysfunction in these patients is believed to involve damage from evanescent cotton wool spots that have already resolved by the time of examination. Previous mfERG studies in HIV-positive patients without infectious retinopathy have demonstrated similar patterns of foveolar and perifoveolar abnormalities.

This case reinforces that ophthalmologists should consider primary HIV retinopathy as a cause of visual loss in HIV-positive patients who are otherwise stable on HAART, even when the fundus appears normal and infectious causes have been excluded. Electrophysiological testing, particularly mfERG, has high diagnostic value in these patients.

Clinical Pearl: In HIV-positive patients presenting with progressive bilateral vision loss, color vision changes, and nyctalopia, do not be falsely reassured by a normal fundus examination and normal CD4 counts. Noninfectious HIV retinopathy can cause significant visual dysfunction even when the retina appears clinically normal. Multifocal electroretinography (mfERG) should be considered when standard workup fails to identify a cause.

Diagnostic Approach: Primary HIV retinopathy is a diagnosis of exclusion. Before attributing visual loss to HIV retinopathy, clinicians must rule out infectious retinitis (CMV, syphilis), optic neuritis, compressive optic neuropathy, toxic or nutritional optic neuropathy, and intracranial etiologies. A complete workup should include MRI, blood tests, visual field testing, and electrophysiological studies.

Citation

Zhang J, Hashemi N, Lee AG. Bilateral Progressive Vision Loss in Noninfectious HIV Retinopathy without Infectious Retinitis. Journal of Ophthalmic and Vision Research. 2012;7(3):268-270.

References

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