Unique Presentation of 3rd and 6th Cranial Nerve Palsies, Nodular Scleritis and Nummular Keratouveitis in an Immunocompetent Patient following an Attack of Herpes Zoster Ophthalmicus

Abstract

Aim: To report a unique presentation of 3rd and 6th cranial nerve palsies with nodular scleritis and nummular keratouveitis following an attack of herpes zoster ophthalmicus (HZO).

Methods: Case report.

Case Report and Results: A 56-year-old woman with a 1-month history of HZO presented with drooping of the right upper eyelid, diplopia, and pain around the right eye. She was noted to have right 3rd and 6th cranial nerve palsies. She developed nodular scleritis and nummular keratouveitis at 2 and 4 months follow-up, respectively, which were treated with antivirals and steroids. At 10 months follow-up, although the diplopia in right lateral gaze persisted, there was no recurrence of ocular inflammation with complete recovery of ptosis.

Conclusions: A unique presentation of multiple cranial nerve palsies with nodular scleritis and nummular keratouveitis in an immunocompetent patient following an attack of HZO is highlighted in this report.

KEYWORDS::

• 3rd cranial nerve palsy
• 6th cranial nerve palsy
• Herpes zoster ophthalmicus
• Keratouveitis
• Scleritis

Herpes zoster ophthalmicus (HZO) is caused by the varicella zoster virus (VZV). Complications associated with HZO include uveitis, scleritis, retinitis, optic neuritis, and central nervous system (CNS) involvement.¹ We report a unique presentation of 3rd and 6th cranial nerve palsies along with development of nodular scleritis and nummular keratouveitis in an immunocompetent patient following an attack of HZO.

CASE REPORT

A 56-year-old woman was referred for a history of complete drooping of the right upper lid following a 3-week history of HZO. At the time of referral, she had already received oral acyclovir 800 mg 5 times a day for 10 days, given at the time of HZO. Her BCVA was 6/9 in both the eyes. Her right eye showed ptosis along with limitation of extraocular movement in all gazes (Figure 1). Pupillary evaluation, slit-lamp, fundus, and intraocular pressure examinations were normal. Left eye examination was normal. MRI of the orbit and cranium did not reveal any abnormality. She was referred to a neurologist who subsequently started her on a tapering regimen of oral steroids (1 mg/kg body weight over 4 weeks), tablet gabapentin 300 mg bd, and tab famciclovir 500 mg bd (3 weeks). As her ptosis began to improve she complained of diplopia. At 2-month follow-up, she developed nodular scleritis involving the upper sclera in the right eye (Figure 2A). A detailed review of systems and laboratory investigations were negative (complete hemogram, ESR, C reactive protein, rheumatoid factor, antinuclear antibodies, antinuclear cytoplasmic antibodies, chest X-ray, liver and renal function tests, VDRL, Mantoux test, and routine urine evaluation). She was restarted on a course of oral famciclovir (500 mg bd for 2 weeks) along with a tapering regimen of oral prednisolone 1 mg/kg weight and 1% prednisolone acetate eyedrops, 6 times/day. The nodular scleritis resolved over 4 weeks (Figure 2B). At 4 months follow-up, she still complained of diplopia, although only in right lateral gaze. She developed multiple nummular lesions in the anterior and midcorneal stroma, along with pigmented medium-sized keratic precipitates distributed throughout the cornea and 1+ AC cells (Figure 3A). Her intraocular pressures and the fundus examination were normal. An aqueous tap from the right eye was positive for varicella zoster virus by a qualitative PCR and negative for herpes simplex, cytomegalovirus, and Mycobacterium tuberculosis. She was started on oral acyclovir 400 mg bd for 4 weeks along with a tapering dose of prednisolone acetate 1% eyedrops 6 times/day. Her anterior chamber inflammation resolved over 4 weeks (Figure 3B). At 10 months follow-up, there was no recurrence of inflammation. Although the ptosis has improved remarkably, the diplopia continues to exist in the right lateral gaze.

FIGURE 1  External photograph showing ptosis following HZO and limitation of extraocular movements in all gazes in the right eye.

FIGURE 2  Photograph showing nodular scleritis in the right eye (A) and its resolution with treatment (B).

FIGURE 3  Slit-lamp photograph showing nummular lesions in the cornea with pigmented medium-sized keratic precipitates (A) and its disappearance following treatment with antivirals and topical steroids (B).

DISCUSSION

The neurological complications of HZO¹ include post-herpetic neuralgia, cranial nerve palsies,²,³ vision loss due to optic neuritis,¹ meningitis, encephalitis, contralateral hemiplegia, and pupillary palsies.^1–3 The pathogenesis could be related to a direct cytopathic effect of the virus on the surrounding tissue, an allergic response to the virus in the tissues, occlusive vasculitis induced by the virus, or activation of a latent neuropathic virus by the VZV.¹ Complete recovery of nerve palsies usually occurs between 6 and 24 months.

Medline search did not reveal cases of HZO with multiple sequelae involving the eye and the CNS in a single immunocompetent individual. The persistence of the viral antigen in the aqueous could have resulted in the nummular keratouveitis and scleritis.

Declaration of interest: The authors report no conflicts of interest.