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Show Journal of Clinical Neuro-ophthalmology 8(3): 185-191,1988. Herpes Zoster Ophthalmoplegia Report of Six Cases P. Archambault, M.D., J. S. Wise, M.D., F.R.C.S.(C), J. Rosen, M.D., F.R.C.S.(c), R. C. Polomeno, M.D., F.R.C.S.(C), and N. Auger, O.D.(F) '£< 1988 Raven Press, Ltd., New York Ophthalmoplegia occurs infrequently in herpes zoster ophthalmicus. The third nerve appears to be the most commonly affected and the fourth nerve the least. We describe herein the clinical course of six patients with herpes zoster ophthalmoplegia. Spontaneous recovery occurred in four patients. The pathogenesis and clinical features of this syndrome are described. Key Words: Herpes zoster-Herpes zoster ophthalmicus- Herpes zoster ophthalmoplegia-Ophthalmoplegia- Zoster. From the Department of Ophthalmology, Sir Mortimer B. Davis-Jewish General Hospital, McGill University, Montreal, Quebec. Address correspondence and reprint requests to Dr. J. S. Wise, Department of Ophthalmology, Sir Mortimer B. DavisJewish General Hospital, 3755 Chemin de la Cote Ste-Cath~ rine, Montreal, Quebec, Canada H3T 1E2. Presented at the Canadian Orthoptic Society Annual Meeting, Montreal, Canada, June 1987. 185 The ocular complications of herpes zoster ophthalmicus, which usually occur during or after the cutaneous eruption, appear in approximately 50% of cases of herpes zoster ophthalmicus (range, 20-72%) (1-6). Extraocular muscle involvement occurs much less frequently. Over the last 3 years, six patients with herpes zoster ophthalmicus and ophthalmoplegia have presented to our neuro-ophthalmology clinic. CASE REPORTS Case 1 Two weeks after developing an eruption of cutaneous herpes zoster ophthalmicus involving the right side of her face, a 73-year-old woman noted horizontal diplopia. She had right-sided ptosis; right adduction paresis and a sluggish pupil, suggesting right third nerve paresis; and right eso- FIG. 1. Case 4, a 65-year-old man with left herpes zoster ophthalmicus. 186 P. ARCHAMBAULT ET AL. tropia in primary position increasing in right gaze and converting to frank exotropia in left gaze, indicating a right sixth nerve palsy. The absence of intorsion of the globe on attempted downgaze suggested a right fourth nerve palsy. Two months later, the levator palpebrae, right medial rectus, and right lateral rectus functions had improved. Eleven months later, the residual right lateral rectus palsy was symptomatically treated with base-out prisms. The fourth nerve palsy recovered, with no residual extorsion of the globe. Case 2 A 63-year-old man developed a complete rightsided ptosis with external ophthalmoplegia and pupil dilatation 2 weeks after the onset of right cutaneous herpes zoster ophthalmicus. The absence of intorsion of the globe in attempted downgaze indicated involvement of the fourth nerve. Two months later, the superior division of the third nerve had recovered. Seventeen months after the onset of diplopia, a residual partial right fourth and sixth nerve palsy persisted. Case 3 An 81-year-old woman noted vertical diplopia 3 weeks after the onset of right herpes zoster ophthalmicus. An isolated right fourth nerve palsy was diagnosed on the basis of increasing right hypertropia in left gaze with 5° of excydotorsion of the globe and a positive head tilt test. She recovered totally within 3 weeks. Case 4 A 65-year-old man noted horizontal diplopia 1 week after the onset of left herpes zoster involving J elm Ncuro-0l'htltalmvl. \.'1'1. ~..; .. " F!G. 2. Case 4: pupil-sparing left third nerve palsy. ..-.-,". HERPES ZOSTER OPHTHALMOPLEGIA 187 FIG. 3. Case 4: left third nerve palsy completely resolved within 3 months. the first and second division of the left trigeminal nerve (Fig. 1). A diagnosis of pupil-sparing left third nerve palsy was made (Fig. 2). The patient recovered completely within 3 months (Fig. 3). Case 5 A 43-year-old man developed right herpes zoster ophthalmicus. One month later he noted vertical diplopia causing him to tilt his head to the left. An isolated right fourth nerve palsy was diagnosed and resolved completely within 1 year. Case 6 A 70-year-old man developed external ophthalmoplegia 10 days after the onset of left herpes zoster ophthalmicus (Fig. 4). A pupil-sparing left third nerve palsy combined with a fourth nerve FIG. 4. Case 6, a 70-year-old man with left herpes zoster ophthalmiclls. J Oill Nellro-ophthalmol, Vol. 8, No.3, 1988 188 P. ARCHAMBAULT ET AL. and partial sixth nerve palsy was diagnosed (Fig. 5). Six weeks later, the fourth and sixth nerve palsy had healed (Fig. 6). Complete recovery occurred 12 weeks after onset (Fig. 7). DISCUSSION While herpes zoster most commonly affects the sensory nerves of the thoracic dermatomes (51 %), the cranial nerves are the second most commonly affected "distribution" (7). In a community-based study, herpes zoster ophthalmicus was diagnosed in 10% of all cases of herpes zoster; however, these patients were significantly older with a greater male:female ratio (1.3:1) than the rest of the population affected (5). Previous studies have shown that ophthalmoplegia occurs in 11-29% of patients with herpes zoster ophthalmicus (3,4,7,8). The patients in this series ranged in age from 43 to 81 years. There were four men and two women. Onset of diplopia varied from 1 to 4 weeks following the cutaneous eruption of herpes zoster ophthalmicus. Spontaneous and complete recovery without any evidence of aberrant regeneration occurred in four patients. The third nerve was involved in four cases, the fourth in five, and the sixth in three. Residual symptoms of diplopia required treatment with prisms only. Follow-up ranged from 3 weeks to 17 months. Hunt (9) reviewed 158 cases of various paralyses in herpes zoster and found 18 cases of third nerve palsy,S cases of sixth nerve palsy, and only 1 case of fourth nerve palsy. Carmody (2) later described a case of complete ophthalmoplegia with proptosis. In 1945, Edgerton reviewed 2,250 cases of herpes zoster ophthalmicus and found the following distribution of extraocular muscle palsy (3,4): total incidence, 13%; third nerve, 47%; sixth nerve, 23%; fourth nerve, 10%; all three, 20%. ~'upil-sparing left third, fourth, and partial sixth nerve palsies. / eli" Nt'llro-op1ltl1Dlmol, Vol. ti. N(I, .~. l~'li~ HERPES ZOSTER OPHTHALMOPLEGIA 189 FIG. 6. Case 6: left fourth and sixth nerve palsies recovered within 6 weeks; note residual left partial third nerve palsy. Hermann (10), Goldsmith (11), and Godtfredsen (12) independently described cases of isolated abducens paralysis, while Grimson and Glaser (13) reported five cases of isolated fourth nerve involvement. Marsh et al. (8) reviewed 58 cases of extraocular muscle palsy among 146 cases of herpes zoster ophthalmicus. All palsies were detected within the first week of the cutaneous eruption, and 28% were asymptomatic. In ipsilateral cases, the third nerve was affected in 29%, while the fourth and sixth nerves were each affected in 12% of cases. Fifteen percent of cases were contralateral, and 9% were bilateral. No cases of aberrant regeneration have been reported in the literature so far. The mechanism by which the ocular motor nerves are involved is not clear. Pathologic correlation has been limited because most patients with herpes zoster ophthalmicus do not die of the dis-ease. In 1871, Wyss (14) postulated that extraocular muscle paresis was secondary to a thrombophlebitis while Edgerton (3,4) and Godtfredsen (12) initially believed that extraocular muscle palsy originated from contiguous inflammation from the trigeminal to ocular motor nerves within the cavernous sinus or the superior orbital fissure. Goodbody (15) described a case with ipsilateral involvement of the ventrolateral nucleus of the third nerve, probably secondary to axonal spread. Kreibig (16) believed that a myositis associated with a perineuritis and a perivasculitis was the cause of the palsy. Goldsmith (11) postulated that neuritis developed from direct lymphocytic infiltration of the affected nerves via sensory ramifications of the trigeminal nerve to all motor nerves of the eye. Nauman et al. (17) described the histopathologic findings in a series of 21 eyes enucleated following herpes zoster ophthalmicus. A I Clill NeuT(l-l'I'hthalmol. Vol. 8. No.3. 1988 HERPES ZOSTER OPHTHALMOPLEGIA 191 4. Edgerton AE. Herpes zoster ophthalmicus (part II). Arch OphthalmoI1945;34:114-53. 5. Ragozzino MW, Melton III LJ, Kurland LT, et al. Population- based study of herpes zoster and its sequelae. Medicine 1982;61:310-6. 6. Womack LW, Liesegang TJ. Complications of herpes zoster ophthalmicus. Arch OphthalmoI1983;101:42-5. 7. Thomas JE, Howard FM. Segmental zoster paresis-a disease profile. Neurology 1972;22:459-66. 8. Marsh RJ, Dulley B, Kelly V. External ocular motor palsies in ophthalmic zoster: a review. Br I Opht/wlmol 1977; 61:677-82. 9. Hunt JR. The paralytic complications of herpes zoster of the cephalic extremity. lAMA 1909;53:1456-7. 10. Hermann JS. Isolated abducens paresis. Am I Ophthalmol 1962;54:298-301. 11. Goldsmith MO. Herpes zoster ophthalmicus with sixth nerve palsy. Can I OphthalnwI1968;3:279-83. 12. Godtfredsen E. Pathogenesis of cranial nerve lesions, notably ophthalmoplegias, complicating herpes zoster ophthalmicus. Acta Psychiatr Neural (Copenhagen) 1948;23:6977. 13. Grimson BS, Glaser JS. Isolated trochlear nerve palsies in herpes zoster ophthalmicus. Arch Ophthalmol 1978;96: 1233-5 14. Wyss O. Beitrag-zur kenntnis des herpes zoster. Arch Heilhllde 1871;16:261. 15. Goodbody RA. The pathology of acute herpes zoster ophthalmicus. I Pat/wi Bacterial 1953;65:221-7. 16. Kreibig W. Die Zostererkrankung des Auges. Klin Monatsbl Augenheilkd 1959;135:1-31. 17. Naumann G, Gass JDM, Font RL. Histopathology of herpes zoster ophthalmicus. Am I Ophthalmol 1968;65:53341. 18. Marsh RJ. Ophthalmic herpes zoster. Br I Hasp Med 1976; 15:609-18. 19. Scheie HG. Herpes zoster ophthalmicus. Trans Ophthalnwl Soc UK 1970;90:899-930. 20. Olson RJ. Herpes zoster. Int Ophthalm,)1 Clin 1984;24:39-48. 21. Liesegang TJ. Herpes zoster ophthalmicus. Int Ophthalmol Clill 1985;25:77-96. 22. Cobo LM, Foulks GN, Liesegang T, et al. Oral acyclovir in the treatment of acute herpes zoster ophthalmicus. 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