Complete Unilateral Ophthalmoplegia in Herpes Zoster Ophthalmicus

Abstract

Based on a review of 20 well-documented cases reported in the English literature between 1968 and 2008, herpes zoster ophthalmicus (HZO) may rarely be associated with complete unilateral ophthalmoplegia, defined here as impaired ocular ductions in all 4 directions within 3 months of onset of manifestations of HZO. Ophthalmoplegia occurred equally in immune-competent and immune-incompetent individuals. HZO preceded ophthalmoplegia in 75% by a mean interval of 9.5 days and a range of 2 to 60 days, occurred simultaneously with ophthalmoplegia in 20%, and followed by 2 days the onset of ophthalmoplegia in only 5%. Concurrent conjunctival inflammation, keratitis, or anterior uveitis was present in 90%. Lumbar puncture showed features of aseptic meningitis in 88%, slightly more than the 40%-50% found in patients with HZO without ophthalmoplegia. On orbit/brain imaging, abnormal enlargement of the extraocular muscles was present in 33%, and orbital soft tissue swelling was present in 17%. Enhancement of ocular motor cranial nerves was not reported. Complete or near-complete resolution of ophthalmoplegia occurred in 65% within a range of 2 weeks to 1.5 years (mean 4.4 months). A single autopsy report described granulomatous angiitis of the meninges and large vessels in the anterior cerebral circulation, as well as periaxial infarction in the optic nerve, pons, and medulla but without viral inclusion bodies or antigen. Unsettled issues are whether the pathogenesis is direct viral invasion or an immune reaction to the virus, whether the impaired ocular ductions are based on myopathic or neuropathic injury, whether there are predisposing factors to the combination of HZO and complete ophthalmoplegia, and whether treatment is effective.