Pesky Papilledema

Pesky Papilledema
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doi: 10.62055/jxblodhjmfxt

INTRODUCTION:

Welcome to the “Neuro Nuggets” column within the Journal of Medical Optometry (JoMO)!  This column aims to make neuro-ophthalmic disease more approachable by blending real-world clinical cases with evidence-based medicine.  The patient in this edition’s column has been well-known in our clinic for many years and helps to highlight a lesser-known mechanism for papilledema.  Enjoy!

 

CASE PRESENTATION:

A 41 y.o. Black female presented with a constellation of symptoms.  She noticed intermittently blurred “fuzzy” vision in both eyes lasting less than one hour in duration for the preceding few months.  The patient also noted peripheral numbness with a tingling sensation in her arms and legs, occasional nausea, and headaches (bilateral temporal in location, variable severity).  The patient’s past medical history included testing positive for human immunodeficiency virus (HIV+) dating back 20 years prior to presentation.  Of note, the patient had discontinued her prescribed HIV medications for several months prior to presentation due to fear of side effects.  The patient’s body mass index was 25.

On examination, the patient’s best-corrected visual acuity was 20/20 in each eye.  Color vision by Ishihara was intact in each eye.  Pupils were reactive without any afferent pupillary defect.  Ocular motility evaluation was normal.  Visual field testing (HVF 24-2 SITA Fast) was full/intact and normal in the right eye, and there was a mildly enlarged blind spot but otherwise intact visual field in the left eye.  Slit lamp exam and intraocular pressure were normal in each eye.  There was evidence of moderate optic disc edema and associated peripapillary hemorrhages in each eye (FIGURE 1).

fig 1 pesky papilledema

MRI brain with/without contrast was normal, with no evidence of mass, hemorrhage, or cerebral venous sinus thrombosis.  Lumbar puncture (LP) in the lateral decubitus position was measured as 31 cm H2O.  Cerebrospinal fluid (CSF) analysis showed elevated white cells (97% lymphocytes), elevated protein, and normal glucose.  An extensive battery of infectious disease serologies and CSF studies showed no evidence of infection (TABLE 1) aside from the patient’s known HIV status.  The patient’s HIV viral load at presentation was 31,600 copies/mL with a CD4 count of 146 cells/microliter on serologic testing.

 

pesky papilledema table1

 

The patient was urged to resume HIV therapy and was restarted on efavirenz and emtricitabine/tenofovir.  One month after resuming HIV therapy, the patient’s exam demonstrated resolution of the optic disc edema (FIGURE 2).  Visual acuity remained intact, and visual field testing normalized to baseline.  On follow-up lab work, the patient’s HIV viral load was not detected, and the CD4 count ultimately normalized to 628 cells/microliter.  The patient has now been followed for 18 years since the initial presentation, and her vision has remained intact without any recurrence of optic disc edema in either eye.

fig 2 pesky papilledema

 

DISCUSSION:

HIV has been reported to impact the optic nerves and visual pathway through several different mechanisms.  First, the HIV virus itself can impact optic nerve function via direct viral infection contributing to axonal degeneration as well as from the effects of associated inflammatory mediators.1  Second, HIV can render an individual susceptible to secondary infection from a host of pathogenic mechanisms (i.e., Cryptococcus or syphilis infection), resulting in infectious optic neuropathy.1  Third, HIV is reported to alter CSF composition and can contribute to intracranial hypertension with resultant papilledema,1 as demonstrated in this case.

The HIV virus contributes to CSF pleocytosis (increased cell count), which increases CSF viscosity and decreases outflow, thereby elevating the intracranial pressure.1  Elevated intracranial pressure is transmitted along the optic nerve/sheath and often manifests with clinical evidence of optic disc edema.  Of note, intracranial hypertension may be the only clinical sign of HIV infection,2 and clinicians should consider including this test in their work-up for patients presenting with optic disc edema.

Importantly, this patient’s CSF analysis was abnormal, with elevated protein and cell counts.  Therefore, a diagnosis of idiopathic intracranial hypertension could not be made based on current clinical criteria.3  While this patient’s optic disc edema improved after resuming anti-retroviral therapy alone, others may go on to require medications or surgery to normalize their intracranial pressure.  This case helps illustrate the importance of properly recognizing the underlying mechanism of intracranial hypertension to guide appropriate management.  Clinicians should maintain a low threshold to consult with infectious disease providers in HIV+ patients presenting with neuro-ophthalmic disease.

 

CLINICAL PEARLS:

  • High levels of HIV viral load are thought to contribute to CSF pleocytosis, increase CSF viscosity, and decrease CSF outflow.
  • Eye care providers should be aware of the potential for HIV to contribute to elevated intracranial pressure and result in papilledema.
  • In addition to retinopathy, HIV may cause optic neuropathy.

 

REFERENCES:

  1. Lynn K. Gordon & Helen Danesh-Meyer. Neuro-Ophthalmic Manifestations of HIV Infection, Ocular Immunology and Inflammation. 2020. 28:7, 1085-1093, DOI: 10.1080/09273948.2019.1704024
  2. Alqahtani et al. Acute HIV Infection Masquerading as Idiopathic Intracranial Hypertension: A Case Report and Literature Review. Case Rep Neurol 2020. 12:56. DOI: 10.1159/000505721,
  3. Chen et al.  Expanding the clinical spectrum of idiopathic intracranial hypertension. Curr Opin Neurol. 2023, 36:43–50, DOI:10.1097/WCO.0000000000001131.

 

The author has no financial disclosures, and no sponsorship or funding was involved in this work.

Boston VA Healthcare System | Boston, MA

Dr. Kane graduated from New England College of Optometry in 2015 and went on to complete an ocular disease/primary care residency at VA Boston Jamaica Plain from 2015-2016. He is currently an attending optometrist at VA Boston. His interests include clinical teaching, neuro-ophthalmic disease, retinal vascular disease, glaucoma, and ocular manifestations of systemic disease.

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