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Show Journal of Clinical Neuro- ophthalmology 9( 4): 267- 269. 1989. Mydriasis in Giant- Cell Arteritis James R. Coppeto, M. D., and Thomas Greco, M. D. © 1989 Raven Press, Ltd., New York A previously healthy 60- year- old woman developed headache, cervical pain, bilateral mydriasis ( right greater than left), and bilateral conjunctival injection as the sole manifestations of acute giant- cell arteritis. Key Words: Mydriasis- Arteritis, giant- cell. From St. Mary's HospitaL Waterbury, Connecticut. Address correspondence and reprint requests to Dr. James R. Coppeto at 1906 North Main Street, Waterbury, CT 06704, U. S. A. 267 Mydriasis is the rarest neuro- ophthalmic association of giant- cell arteritis. Such dilated pupils are generally termed ischemic tonic pupils, principally because of pharmacologic supersensitivity, despite the fact that they are not typical tonic pupils clinically and the fact that their genesis from ischemia is purely presumptive. We present a patient with bilateral pupillary paresis, bilateral bulbar conjunctival injection, and head and neck pain as the only manifestation of acute giant- cell arteritis. We propose that such pupils may arise from immunogenic rather than vasculogenic mechanisms. CASE REPORT A previously healthy 60- year- old woman experienced intermittent right periorbital headaches for 4 months followed by 1 week of right tinnitus and tenderness of the right temple and right anterior neck. She was treated with amoxicillin. Several evenings later, she noted severe bulbar conjunctival injection and a " gritty sensation in both eyes." When she woke the next morning, she found her right pupil larger than the left. She sought medical advice when she became aware of " glare" in the right eye. Her physician ordered a Westergren sedimentation rate, which was 113 mm/ h. VORL and FTA- ABS were nonreactive. Lyme enzymelinked immunosorbent assay titers for IgG and IgM were negative « 100). On neuro- ophthalmic consultation, the only positive findings were severe bilateral bulbar conjunctival injection and pupillary abnormalities. In ambient light, the right pupil was 7.0 mm and reacted approximately 0.3 mm to light and approximately 0.5 mm to near effort; the left pupil was 4.5 mm and reacted 0.5 mm to light and approximately 1.0 mm to near effort. A cranial computed tomographic scan and lumbar puncture as well as neurologic examination were all normal. Review of old photographs showed 3- mm pupils back to the time of her wedding pictures. 268 J. R. COPPETO AND T. GRECO Prednisone 80 mg/ day was initiated and produced almost immediate relief of all head pain. Two days later, temporal artery biopsy was performed and revealed arteritis consisting of lymphocytic and histiocytic infiltrates with multifocal destruction of the internal lamina by multinucleated giant cells. Two days later, one drop of 0.1% pilocarpine hydrochloride in each eye produced miosis to 1 mm size of each pupil. Two months later the right pupil was 6.5 mm and reacted briskly to 5.5 mm with intense illumination or near effort. The left pupil was 4.5 mm and constricted briskly to 4 mm with intense illumination or near effort. Thereafter, pupillary findings remained unchanged although pharmacological supersensitivity abated. DISCUSSION Davis et al. first reported mydriasis from giantcell arteritis ( 1). A 69- year- old woman presented with a dilated right pupil that was fixed to light and near effort. The afferent visual system was completely spared. The pupil never recovered significant contractility and, although clinically not a tonic pupil, it was termed " tonic," presumably solely on the basis of temporary supersensitivity to 2.5% methacholine hydrochloride. Such a designation may be inaccurate because current formulations suggest that pharmacologic pupillary supersensitivity is insufficient for diagnosis of a tonic pupil because it may occur in a variety of other innervational pupillary disorders. The case discussed by Bronster et al. ( 2) is virtually identical clinically and pharmacologically to that of Davis et al. except that the mydriasis was bilateral and the patient had severe bilateral permanent visual loss from ischemic optic neuropathy. The case in Currie and Lessell is significantly different from the above two cases and is somewhat unusual in its manifestations and course ( 3). Their patient had severe visual loss in both eyes; pupils were only mildly dilated although they showed " segmental iris contractions" and pharmacologic supersensitivity au. However, it was interesting that the near pupillary reaction was not tonic; vision recovered significantly at 6 months in the right eye, and the right pupil recovered completely clinically although still retaining pharmacologic supersensitivity! Currie and Lessell postulate an ischemic mechanism for the pupillary abnormalities in their case. Our patient's findings were similar to those of Davis et al. and Bronster et al ( 1,2). Our patient's tf'mn!. · , n.- l npd fl;: 1in and tenderness, initially I Clin N~ ro- ophtlullmol, Vol. 9, No. 3. 1989 considered a manifestation of a viral illness or of migraine, were probably due to giant- cell arteritis. Her pupillary abnormalities probably reflected bilateral damage to the ciliary ganglia. Our case is distinctive because it manifested bilateral pupillary involvement without any evidence whatsoever of orbital or ocular ischemia, a manifestation difficult to reconcile with an ischemic mechanism. Barricks et al. reported a case that in many ways is the converse of our case ( 4). Their patient had total ophthalmoplegia from massive, necrotizing, orbital giant- cell arteritis, yet had total sparing of the ciliary ganglia histopathologically and appeared to have relatively normal pupillary functioning, considering the severe degree of visual loss. In their case the ciliary ganglia appeared to be a relatively " privileged" site with regard to orbital ischemia. Moreover, mydriasis appears to be relatively uncommon even in clearly documented ophthalmoplegic cases of giant- cell arteritis, with miosis actually being the rule rather than mydriasis ( 5). The bilaterality of the findings in our patient without any evidence of ocular ischemia as well as the lack of evidence of mydriasis in cases of ophthalmoplegia in giant- cell arteritis have led us to suspect that the etiology of the mydriasis that rarely occurs in giant- cell arteritis may be an immunological attack on the parasympathetic pupillary innervation, somewhat analogous to cases of mydriasis associated with infections and some autonomic neuropathies. Immunologic abnormalities occur in giant- cell arteritis. These include the variable presence of anti- nuclear antibodies, the occasional deposition of IgM and IgG on arterial basement membrane ( 6), the presence of both T4 helper/ inducer and T8 cytoxiclsuppressor lymphocytes ( 6), the frequent elevation of C- reactive protein ( 7), elevated levels of circulating immune complexes ( 8) ( by Raji cell radioimmunoassay), and the association of HLA- DR antigens ( 9). The clinical correlates of such abnormalities may include peripheral neuropathy, microangiopathic hemolytic anemia, pernicious anemia, synovitis, and uveitic glaucoma ( 10- 12). In this regard we have described a case of bilateral uveitic glaucoma in a patient with giant- cell arteritis without ischemic optic neuropathy in which the mechanism of the uveitis appeared to be at least partially immunologic and not solely ischemic ( 12). In conclusion, mydriasis is an exceedingly rare accompaniment of giant- cell arteritis. Such cases have not been documented to evolve into tonic pupils, although pharmacologic supersensitivity is uniformly present and in one instance, some aspects of a clinical tonic pupil were transiently MYDRIASIS IN GIANT- CELL ARTERITIS 269 present. There is no proof that such pupillary abnormalities are ischemic in origin. It is equally likely that they represent a manifestation of one of the rare immunological disturbances in giant- cell arteritis. Acknowledgment: The authors thank the staff of St. Mary's Hospital Library and Record Room. REFERENCES 1. Davis RH, Daroff RB, Hoyt WF. Tonic pupil after temporal arteritis. umcet 1968; 1: 822. 2. Bronster DJ, Rudolph SH, 5hanzer S. Pupillary light- near dissociation in cranial arteritis. Neuro- ophthalmology 1983; 3: 6:>-- 70. 3. Currie J, Lessell S. Tonic pupil with giant cell arteritis. Br J Ophthalmol 1984; 68: 13s-- 8. 4. Barricks ME, Traviesa DB, Glaser J5, Levy IS. Ophthalmoplegia in cranial arteritis. Brain 1977; 100: 209- 21. 5. Dimant J, Grob D, Brunner NG. Ophthalmoplegia, ptosis, and miosis in temporal arteritis. Neurology 1980; 30: 1054- 8. 6. Chess J, Albert DM, Bhan AK, Paluck EI, Robinson N, Collins B, Kaynor B. Serological and immunopathological findings in temporal arteritis. Am J Ophthalmol 1983; 96: 28J- 9. 7. Eshaghian J, Goeken JA. C- reactive protein in giant cell arteritis. Ophthalmology 1980; 87: 1160- 3. 8. Papaioannou CC, Gupta RC, Hunder GG, McDuffie FC Circulating immune complexes in giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum 1980; 23: 1021- 5. 9. Lowenstein MB, Bridgeford PH, Vasey FB, Germain BF, Espinoza LR. Increased frequency of HLA- DR3 and DR4 in polymyalgia rheumatica- giant cell arteritis. Arthritis Rheum 1983; 26: 92:>-- 7. 10. Mulcahy F, Juby LD, Chandler GN. Giant cell arteritis presenting with peripheral neuropathy. Postgrad Med J 1984; 60: 670- 1. 11. Zauber NP, Echikson AB. Giant cell arteritis and microangiopathic hemolytic anemia. Am J Med 1982; 73: 92&- 30. 12. Coppeto JR, Greco T. Giant cell arteritis with bilateral uveitic glaucoma. Ann Ophthalmol 1985; 17: 299- 302. , Clin Neuro- oplrthalmol, Vol. 9, No. 4, 1989 |
