Herpes Zoster Optic Neuritis

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Article initiated by:

Linda Cassidy Davis, Mark Robinson, MD, MPH

All contributors:

Nagham Al-Zubidi, MD, Ayman Okla Suleiman, MD, Mark Robinson, MD, MPH

Assigned editor:

Ayman Okla Suleiman, MD

Review:

Assigned status Up to Date

 by Ayman Okla Suleiman, MD on October 4, 2024.

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Herpes zoster optic neuritis (HZON) is a rare sequela of herpes zoster ophthalmicus (HZO), It can occur either in conjunction with HZO or, more frequently, presents as a postherpetic complication, and has been reported up to 10 weeks after HZO onset. Other diseases should be considered in the differential diagnosis, including giant cell arteritis. There is no constant laboratory or imaging finding in HZON. Different treatments have been recommended for isolated HZON, from oral antivirals to IV acyclovir 10mg/kg 7-10 days. If there are other associated neurologic symptoms or other involvement on MRI brain, then acyclovir 10-15mg/kg every 8 hours for 2-3 weeks is indicated.

Disease Entity

Herpes Zoster Optic Neuritis

Disease

Herpes zoster optic neuritis (HZON) is a rare sequela of herpes zoster ophthalmicus(HZO), or herpes zoster in the distribution of the ophthalmic branch of the trigeminalnerve. It can occur either in conjunction with HZO or, more frequently, presents as a postherpetic complication, with cases reported up to 10 weeks after HZO onset.

Pathophysiology

The pathophysiology of how VZV damages the optic nerve is uncertain. There are three proposed pathophysiologic mechanisms of HZON.

• Direct nerve infection through the cavernous sinus
• Inflammatory demyelination
• Systemic or CNS infection with reactive immune response leading to edema of the optic disc

Primary prevention

HZO should be treated with oral antiviral therapy (acyclovir, valacyclovir, or famciclovir) plus topical steroid drops if immunocompetent or IV acyclovir (10 mg/kg three times daily for seven days) if immunocompromised. Although this may not prevent HZON, it may reduce the incidence of it.

Diagnosis

The diagnosis of HZON should be suspected when an acute, unilateral or bilateral, anterior or retro-bulbar, optic neuropathy occurs in the setting of HZO or within about 3 months of its onset. Other disease processes should be considered as well, as discussed in the differential diagnosis section.

History

Symptoms of HZO (fever, malaise, eye pain, headache prior to eruption of rash in distribution of the ophthalmic branch of the trigeminal nerve) or has a previous diagnosis of HZO and presents with vision loss.

Physical examination

• Visual acuity is decreased in the affected eye
• Relative afferent pupillary defect in the affected eye
• Fundus exam can initially show a normal or edematous optic nerve that may progress to optic nerve atrophy
• Neurological exam can reveal hypoesthesia in the area supplied by the ophthalmic branch of the trigeminal nerve on the affected side

Diagnostic procedures

• MRI of orbit can show enhancement and restricted diffusion of the affected optic nerve
• T2 weighted MRI can also show linear hyperintense lesions in trigeminal nucleus of a patient with herpes zoster optic neuritis
• Fluorescein angiography can show late staining of affected optic disc
• Doppler studies of central retinal artery, posterior ciliary artery, and ophthalmic artery of affected eye are usually normal
• Consider lumbar puncture with CSF analysis to include sending paired samples of serum and CSF or intraocular fluid (in patients with uveitis) to the lab for PCR and serology, although case reports have shown negative PCR results in some suspected cases of HZON

Laboratory test

• Other causes of optic neuropathy should be excluded by patient history and examination, including giant cell arteritis.  Considerthe following tests:

• Complete blood count (CBC), erythrocyte sedimentation rate (ESR), c-reactive protein (CRP), Treponema pallidum hemagglutination (TPHA), Venereal Disease Research Lab (VDRL), antinuclear antibody (ANA), and aquaporin-4 antibody, Myelin oligodendrocyte glycoprotein (MOG)

Differential diagnosis

Differential Diagnosis of Acute Optic Neuropathy:

Optic neuritis, Non-Arteritic Ischemic Optic Neuropathy, Posterior Ischemic Optic Neuropathy, Arteritic Ischemic Optic Neuropathy (Giant Cell Arteritis), Central Retinal Artery Occlusion, Toxic (should be symmetric vision loss), Infiltrative, Leber’s Hereditary Optic Neuropathy

Management

Different treatments have been recommended for isolated HZON, from oral antivirals to IV acyclovir 10mg/kg 7-10 days. Adjunct oral corticosteroid is controversial, with some treatment recommendations consisting of oral prednisone 60 mg per day with a topical steroid if ocular inflammation is present, while others recommend avoiding adjunct oral prednisone because of lack of evidence for definitive benefit and possible risk of VZV retinitis.  If there are associated neurologic symptoms or other involvement on MRI brain, then acyclovir 10-15mg/kg every 8 hours for 2-3 weeks is indicated.

Prognosis

Visual recovery is variable.  Poor visual prognosis is usually seen in patients with concomitant ocular complications of HZO. Restricted diffusion of the optic nerve on MRI has been proposed as a predictor for poor visual recovery.

References

5. Albrecht MA. Treatment of herpes zoster in the immunocompetent host. UpToDate. https://www.uptodate.com/contents/treatment-of-herpes-zoster-in-the-immunocompetent-host?sectionName=Herpes zoster ophthalmicus&search=herpes zoster ophthalmicus&topicRef=8327&anchor=H18&source=see_link#H1595824332. Published December 10, 2018. Accessed April 5, 2020.
6. Bagheri N, Wajda B, Calvo C, Durrani A. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 7th ed. Wolters Kluwer Health; 2016.
7. Cason JB. Herpes Zoster Ophthalmicus. EyeWiki. https://eyewiki.org/Herpes_Zoster_Ophthalmicus#Complications. Published February 16, 2017. Accessed April 5, 2020.
8. Kahloun R, Attia S, Jelliti B, et al. Ocular involvement and visual outcome of herpes zoster ophthalmicus: review of 45 patients from Tunisia, North Africa. Journal of Ophthalmic Inflammation and Infection. 2014;4(1). doi:10.1186/s12348-014-0025-9.
9. Kaufman AR, Myers EM, Moster ML, Stanley J, Kline LB, Golnik KC. Herpes Zoster Optic Neuropathy. Journal of Neuro-Ophthalmology. 2018;38(2):179-189. doi:10.1097/wno.0000000000000607.
10. Vanikieti K, Poonyathalang A, Jindahra P, Cheecharoen P, Patputtipong P, Padungkiatsagul T. Isolated optic neuritis with a concurrent abnormal trigeminal nucleus on imaging: case report of a rare complication of herpes zoster ophthalmicus. BMC Neurology. 2018;18(1). doi:10.1186/s12883-018-1168-3.