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Autoimmune Optic Neuropathy Ac- Tic Nerve Tissue Affected by Autoim- Tic Neuropathy: Evaluation and Treatment

Autoimmune Optic Neuropathy Ac- Tic Nerve Tissue Affected by Autoim- Tic Neuropathy: Evaluation and Treatment

CASE REPORTS AND SMALL CASE SERIES

the right eye. Her intraocular pres- thors noted that nevi or freckles did Evidence of Early Change sure was maintained in the high not increase in size and the likeli- in Color With teens to low 20s using this treat- hood of change was greatest in those Use ment regimen until she was seen in patients who had heterogeneous pig- 1993 with evidence of progression mentation at baseline. The adverse Latanoprost, a 17-phenyl–substi- of her defect. In 1994, the effect is likely related to an increase medical therapy was changed to 1% in melanin production in the mela- tuted analog of PGF2 ␣, has been shown to effectively lower intraocu- pilocarpine hydrochloride 4 times nocytes of the iris stroma. lar pressure in clinical trials and daily. During a 3-year period, the op- In the case presented herein, darken the irides in both subhu- tic nerve of her right eye pro- the patient was unilaterally treated, man primates and humans.1 The re- gressed with evidence of vertical thus the slightest change in iris color ported time of onset of the change elongation and a superior rim de- could be detected early. At base- in iris color has been noted to be as fect. In 1996, after a course of dor- line, the patient had an iris color with early as 3 months. To our knowl- zolamide hydrochloride was given mixed pigmentation, placing her at edge, this case represents the earli- in the right eye 3 times daily with high risk for the development of this est reported change in iris color minimum improvement, the pa- adverse effect. This report differs following the initiation of latano- tient was then given latanoprost in from previous reports in that the on- prost use. the right eye only for a 4-week pe- set of change occurred after 4 weeks riod. The iris color in the right eye of treatment. Previous reports2 in Report of a Case. A 78-year-old was the same as the left eye at that subhuman primates noted the white woman was first seen in 1986 time. During the 4-week period, the change after at least 6 weeks of treat- complaining of worsening vision, patient’s iris color changed from ment. We noted no change in the which was found to be secondary to blue-green to brown-green. Use of lashes, which may be related to the nuclear sclerosis involving her right the medication was subsequently short exposure to the drug.3 eye. She underwent an uncompli- discontinued (Figure). cated extracapsular extrac- Regine M. Pappas, MD tion with intraocular implan- Comment. Alm et al1 summarized Sharon Pusin, MD tation in December 1988. Prior to the results of 198 patients who pre- Eve J. Higginbotham, MD surgery, she had intraocular pres- viously participated in the original Baltimore, Md sure in the midteens and small sym- phase 3 clinical trials that assessed metric cups with healthy neuroreti- the safety and efficacy of latano- nal rims. In 1992, she had elevated prost. Photographs of the iris were Dr Higginbotham was an investiga- intraocular pressure in the right eye not taken prior to 2 months after the tor in the phase 3 clinical trial and is in the high 20s to low 30s. On vi- initiation of treatment. Darkening of currently an investigator in the com- sual field testing, she demonstrated the iris occurred in 14 patients (7%) bined latanoprost- study, which a nasal step. The patient was then at 6 months and in 24 patients (12%) is sponsored by Pharmacia-Upjohn, given timolol maleate twice daily in after 1 year of treatment. The au- Kalamazoo, Mich. Her expenses for

Magnified view of the affected right eye (left) and unaffected left eye (right).

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 travel to various meetings have also , and fluorescein angi- sis of a patient with bull’s-eye macu- been reimbursed by Pharmacia- ography showed a retinal pigment lopathy. Certainly, a history of chlo- Upjohn. epithelial window defect in the roquine or hydroxychloroquine use Reprints: Eve J. Higginbotham, macula surrounding a central area would lead the clinician to suspect MD, Department of , of normal fluorescence (Figure 1, toxicity from systemic medica- University of Maryland School of right). tions. Patients with cone dystro- Medicine, 22 S Greene St, Baltimore, Case 2. A 60-year-old man phy or generally MD 21201. noted central distortion of vision in have symptoms of visual loss within his left eye after he covered his right the first 2 decades of life and may re- 1. Alm A, Camras C, Watson P. Phase III latano- prost studies in Scandinavia, the United King- eye in 1978. Examination of the left port a family history of ocular dis- dom and the United States. Surv Ophthalmol. eye disclosed a stage 2 macular hole ease.2 In contrast, patients with id- 1997;41(suppl 2):S105-S110. Figure 2 iopathic macular holes generally 2. Selen G, Stjernschantz J, Resul B. Prostaglandin- ( , left) and a visual acuity induced iridial pigmentation in primates. Surv of 20/50. Twenty years later, best- have normal vision until the sixth Ophthalmol. 1997;41(suppl 2):S125-S128. corrected visual acuity was 20/200 through eighth decades of life and 3. Johnstone M. Hypertrichosis and increased pig- mentation of and adjacent hair in the OS. Fundus photography revealed a have no family history of macular region of the ipsilateral of patients treated characteristic bull’s-eye maculopa- disease. The bull’s-eye maculopa- with unilateral topical latanoprost. Am J Oph- thy (Figure 2, right). thy associated with chronic macu- thalmol. 1997;124:544-547. lar holes generally has very sharp Comment. The term bull’s-eye macu- borders between the depigmented lopathy refers to the ophthalmo- macular area and the surrounding Bull’s-eye Maculopathy scopic appearance of a central area normal retinal pigment epithe- Associated With Chronic of retinal pigment epithelial depig- Macular Hole mentation surrounded by rela- tively normal retinal pigment epi- Differential Diagnosis Bull’s-eye maculopathy has been as- thelium giving a “bull’s-eye” of Bull’s-eye Maculopathy* sociated with various macular dis- appearance to the macula. This ap- eases, most notably chloroquine and pearance is shared by a relatively Age-related hydroxychloroquine maculopathy, large group of unrelated condi- Bardet-Biedl syndrome 1 Benign concentric annular macular cone dystrophy, and Stargardt dis- tions. The 2 patients described dystrophy 1-4 ease (Table). To our knowledge, herein demonstrate that chronic Chloroquine and hydroxychloroquine bull’s-eye maculopathy associated macular holes should be included in maculopathy with chronic macular holes has not the differential diagnosis of bull’s- Clofazimine been previously reported. eye maculopathy. Selective depig- Cone dystrophy Cone-rod dystrophy mentation of the retinal pigment epi- Fenestrated sheen macular dystrophy Report of Cases. Case 1. A 71-year- thelium may occur under the cuff of Hallervorden-Spatz syndrome old woman developed progressive subretinal fluid that surrounds a Idiopathic central serous visual loss in her left eye in 1990. Ex- chronic macular hole. The retinal chorioretinopathy amination of the left eye disclosed pigment epithelial pigmentation un- Leber congenital amaurosis a macular hole and a visual acuity derlying the macular hole is usu- Lipofuscinosis Sorsby central areolar choroidal dystrophy of 20/200. Seven years later, best- ally preserved and corresponds to the Stargardt disease corrected visual acuity was 20/ center of the bull’s-eye pattern. 200 OS (Figure 1, left). Fundus Several historical and clinical *Differential diagnosis of bull’s-eye photography revealed a bull’s-eye features aid in the specific diagno- maculopathy listed in alphabetical order.1

Figure 1. Case 1. Left, Macular hole in the left eye associated with bull’s-eye maculopathy. Right, Fluorescein angiogram demonstrates retinal pigment epithelial window defect surrounding a circular area of intact retinal pigment epithelium in the central macula.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Figure 2. Case 2. Left, Macular hole in the left eye (in 1978). Right, Macular hole (in 1998) associated with bull’s-eye maculopathy.

lium, in contrast to the rather indis- 2nd ed. St Louis, Mo: Mosby–Year Book Inc; (right eye), resulting in bilateral 1994:xxxiv. tinct borders of the bull’s-eye macu- 4. Berkow JW, Flower RW, Orth DH, Kelley JS. . In 1993, the patient first lopathy associated with toxic Fluorescein and Indocyanine Green Angiogra- was seen by his ophthalmologist , cone dystrophy, or phy: Technique and Interpretation. 2nd ed. San with redness, discharge, and ocular Francisco, Calif: Palace Press; 1997:108-112. Stargardt disease. The configura- American Academy of Ophthalmology mono- irritation in the left eye. At that time, tion of the bull’s-eye associated with graph. he had an exposed area of conjunc- chronic macular hole appears more tiva superiorly over his scleral rounded in comparison to the more buckle. The buckle was still thought oval configuration in most other to be well positioned, and the pa- bull’s-eye maculopathies. On con- Acute Macular tient was treated using topical cipro- tact lens biomicroscopy, visualiza- Associated With an floxacin. He was seen periodically tion of a cuff of subretinal fluid usu- Infected Scleral Buckle and was thought to be doing well ally allows the clinician to confirm with vision of 20/25 OS with con- the diagnosis of bull’s-eye macu- Exposure or infection of a scleral servative management. In August lopathy due to chronic macular buckle is an unusual complication 1996, the patient had acute - holes. The is generally flat in of surgery. It has less visual loss to 20/60 OS. Exami- the other conditions associated with been reported in 0.5% to 18.0% of nation revealed an exposed scleral bull’s-eye maculopathy, and the el- all procedures.1 Patients may be seen buckle superiorly with purulent evated cuff of subretinal fluid around with ocular irritation, pain, dis- discharge (Figure 1). Fundu- a macular hole is a key finding in dis- charge, redness, and sometimes vi- scopic examination revealed dif- tinguishing a macular hole from sual loss. Reported causes of visual fuse macular and retinal edema. other entities in the differential di- loss include with vitreous There was no intraocular inflamma- agnosis of bull’s-eye maculopathy. clouding, recurrent retinal detach- tion. A fluorescein angiogram was Chronic macular hole should be in- ment secondary to proliferative vit- obtained, revealing diffuse macular cluded in the differential diagnosis reoretinopathy, and macular distor- hyperfluorescence consistent with of bull’s-eye maculopathy. tion.1-4 This case report describes the noted clinically angiographically documented dif- (Figure 2). There was no evi- Ingrid U. Scott, MD, MPH fuse macular edema associated with dence of vasculitis. The patient was Harry W. Flynn, Jr, MD in a patient with an given oral cefazolin, 250 mg 4 times William E. Smiddy, MD infected and extruded scleral buckle. daily, for 4 days and topical cipro- Miami, Fla floxacin drops 4 times daily and sub- Report of a Case. A 77-year-old sequently underwent surgical re- Supported in part by a Heed Ophthal- white man was referred to our clinic moval of the scleral buckle through mic Foundation Fellowship, Cleve- with a 2-week history of new-onset the superior conjunctival opening. land, Ohio (Dr Scott), and a Ronald visual blurring in the left eye. The An encircling band and tire were re- G. Michels Fellowship, Philadelphia, patient had an ocular history no- moved. Cultures of these elements Pa (Dr Scott). table for severe and retinal were negative for organisms. Marked 1. Gass JDM. Stereoscopic Atlas of Macular Dis- detachments in both eyes. The pa- scleral thinning was noted beneath eases: Diagnosis and Treatment. 4th ed. Vol 1. St tient’s medical history was notewor- the scleral buckle site at the time of Louis, Mo: Mosby–Year Book Inc; 1997:I-9. Figure 3 2. Reichel E. Hereditary cone dysfunction syn- thy only for controlled hyperten- surgery ( ). The conjunc- dromes. In: Albert DM, Jakobiec FA, eds. Prin- sion. He underwent scleral buckling tiva was closed over this area. Sub- ciples and Practice of Ophthalmology. Vol 2. Phila- procedures with silicone explants in conjunctival vancomycin, 50 mg, delphia, Pa: WB Saunders Co; 1994:1238- 1248. the late 1960s. He had cataract ex- was injected at the end of surgery. 3. Ryan SJ, Schachat AP, Murphy RB, eds. Retina. tractions in 1984 (left eye) and 1991 The patient was given polymixin-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Figure 1. Exposed scleral buckle with purulent discharge. Figure 3. Marked scleral thinning after removal of the infected scleral buckle.

Figure 2. Diffuse macular hyperfluorescence associated with clinical macular Figure 4. Angiogram taken 10 months after surgery shows resolved macular edema at the time first examined by us with the infected scleral buckle. edema after removal of the infected scleral buckle.

trimethoprim drops 4 times daily edema. In addition to macular tient, has been reported after infec- and continued to use oral cefazolin edema, long-standing uveitis may tion and removal of scleral buckles for a total of 7 days. Two weeks af- also cause and as well. One series4 showed that this ter surgery,1,4 the vision had im- that can also result in visual loss.5 occurred in nearly 30% of the cases. proved to 20/25 OS. Funduscopic Our patient’s infection oc- Macular edema may be diffi- examination revealed no appre- curred nearly 30 years after the pro- cult to detect in these patients with ciable residual macular thickening. cedure. Most infections occur much infected or extruded scleral buck- Further follow-up at 10 months re- earlier, with a mean of 2 months in les because discharge may affect cor- vealed no changes, with vision re- one series and 8 months after ini- neal clarity and the patient may be maining at 20/25 OS. tial surgery in another series.1,4 Our photosensitive. The use of fluores- patient had a dramatic return of vi- cein angiography may be a useful ad- Comment. This case demonstrates sual acuity with normalization of the junct to making the diagnosis. Rec- diffuse macular edema associated fluorescein angiogram (Figure 4). ognition of macular edema may with an infected scleral buckle re- Visual acuity results after removal of encourage earlier removal of the sulting in acute visual loss. Prompt an infected scleral buckle are rarely buckle so as to avoid complications removal of the infected scleral buckle reported in the literature. Retinal de- of chronic macular edema. may result in rapid visual recovery, tachment is the most common cause as noted in our patient. The macu- of poor vision after removal of scleral Sundeep Dev, MD lar edema is likely the result of the buckles.1,3 Late redetachment rates William F. Mieler, MD infection and associated . range from 45% to 39%, with the Robert A. Mittra, MD This inflammatory process can ex- highest percentage occurring when Anu Prasad, BS tend from the and result in the buckle has been in place for less Milwaukee, Wis several ocular complications, includ- than 6 months.1,2,6 Most detach- ing uveitis.5 Although no intraocu- ments occur within the first 6 Supported in part by an unrestricted lar was seen during months after removal.6 In our pa- grant from Research to Prevent Blind- our examination, it is also possible tient, the retina has remained at- ness Inc, New York, NY, and by core that prior inflammation existed and tached at 10 months after surgery. grant EY01931 from the National led to the development of macular Scleral thinning, as seen in our pa- Institutes of Health, Bethesda, Md.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Dr Dev is a Heed Ophthalmic Foun- ease, amniotic fluid embolism, ret- ther seizures. On the fourth day of dation (Cleveland, Ohio) Fellow. robulbar anesthesia, chronic renal his intensive care admission, an oph- Reprints: William F. Mieler, MD, failure, and thrombotic thrombocy- thalmic evaluation disclosed slug- Medical College of Wisconsin Eye topenia purpura.2 We report a case gishly reacting without rela- Institute, 925 N 87th St, Milwaukee, of Purtscher-like retinopathy in a pa- tive afferent pupillary defect and WI 53226-4812 (e-mail: wfmieler tient with hemolytic uremic syn- moderate conjunctival chemosis. @mcw.edu). drome (HUS). Funduscopic examination revealed ischemic retinal whitening in both 1. Smiddy WE, Miller D, Flynn HW. Scleral buckle removal following retinal reattachment. Oph- Report of a Case. A 31-month-old posterior poles, most prominently in thal Surg. 1993;24:440-445. boy was admitted to a hospital with the peripapillary area. Retinal arte- 2. Hilton GF, Wallyn RH. The removal of scleral a history of pallor, lethargy, and oli- riolar attenuation and scattered in- buckles. Arch Ophthalmol. 1978;96:2061-2063. 3. Hadden OB. Infection after retinal detachment guria preceded by gastroenteritis. Af- traretinal hemorrhages were also surgery. AustNZJOphthalmol. 1986;14:69-71. ter experiencing 2 brief tonic- present bilaterally. Additionally, a 4. Lincoff H, Nadel A, O’Connor P. Changing char- acter of the infecting scleral implant. Arch Oph- clonic seizures, he was transferred preretinal hemorrhage and peripap- thalmol. 1970;84:421-426. to the pediatric intensive care unit illary nerve fiber layer hemorrhage 5. Sainz de la Marza M, Foster CS, Jabbus NS. Scle- where examination revealed a le- were noted in the left eye (Figure 2). ritis: associated uveitis. Ophthalmology. 1997; 104:58-63. thargic child with scattered pete- The patient experienced car- 6. Schwartz PL, Pruett RC. Factors influencing reti- chiae and periorbital and periph- diovascular and neurologic decline nal redetachment after removal of buckling el- eral edema. The child was anuric. despite intensive management and ements. Arch Ophthalmol. 1977;95:804-807. Computed tomography of his brain was normal on admission. Labora- tory findings revealed microcytic Hemolytic Uremic hemolytic anemia (hemoglobin, Syndrome Associated With 68 g/L; mean corpuscular volume, Purtscher-like Retinopathy 72.5 mm3), thrombocytopenia (platelets, 33 ϫ 109/L), and acute re- Purtscher reported the findings of nal failure (creatinine, 407 µmol/L cotton-wool spots, hemorrhages, and [4.6 mg/dL]). Stool culture yielded edema in the posterior fundus of 2 Escherichia coli (0157:H7). Diag- patients who experienced severe nosed as having HUS, the patient un- head trauma. Later, angiopathica derwent plasmapheresis and hemo- retinae traumatica,1 or Purtscher reti- dialysis. Three days after admission nopathy, was described following to the pediatric intensive care unit, long-bone fractures, rapid decelera- a repeated computed tomographic tion injuries, and compressive inju- scan revealed bilateral infarctions of ries to the trunk. Subsequently, the basal ganglia (Figure 1). The Purtscher-like retinopathy was ob- patient had not experienced any Figure 1. Computed tomogram without contrast served in atraumatic cases such as trauma, cardiopulmonary resusci- reveals bilateral hypodensities in the basal pancreatitis, collagen vascular dis- tation, assisted ventilation, or fur- ganglia indicating hypoxic injury (arrows).

Figure 2. Fundus photographs taken 1 day before patient’s death reveal ischemic retinal whitening in the posterior pole, most prominent in the peripapillary area. Additionally, a preretinal hemorrhage and peripapillary nerve fiber layer hemorrhage were noted in the left eye (right). Left, Right eye.

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 died. He was thought to have had Hypotheses have implicated reflux less visual loss in her right eye 7 days multiple thrombotic infarcts to the hydrostatic injury, vasospasm, and prior to our examination. Four central nervous system and myocar- microembolism of fat, air, fibrin, am- months earlier, she was involved in a dium; the request for autopsy was niotic fluid, or granulocyte aggre- motor vehicle crash as a restrained denied. gates in retinal arterioles.2 The passenger. She sustained sternal and pathogenesis of HUS suggests the rib fractures and bilateral pneumo- Comment. Hemolytic uremic syn- Purtscher-like changes in our pa- thoraces, requiring 2 chest tubes. She drome is the most common cause of tient are a result of thrombotic reti- did not have any neurological or acute renal failure in young children nal microangiopathy caused by exo- ocular symptoms and was dis- and is marked by a prodrome of gas- toxin-induced endothelial injury. charged 1 week after the trauma. Her troenteritis followed in 5 to 10 days Purtscher-like retinopathy in medical history was notable for by pallor, irritability, weakness, leth- HUS is reported infrequently and its sickle cell trait and cigarette use of argy, and oliguria. Laboratory find- diagnosis in the acute phase is over- half a pack per day. She had discon- ings include microangiopathic hemo- shadowed by the grave systemic tinued her oral contraceptives 1 lytic anemia, thrombocytopenia, and complications of HUS. Awareness of month prior to the motor vehicle acute renal failure of severity vary- this entity may help explain visual crash. ing from mild insufficiency to renal loss in children who recover from Visual acuity was 20/40 OD and shutdown. Central nervous system acute HUS. 20/30 OS, and there was a right rela- manifestations occur in a minority tive afferent pupillary defect. Gold- of patients and portend a poorer Andreas Katsuya Lauer, MD mann visual fields showed a supero- prognosis. Stool culture frequently Michael L. Klein, MD nasal quadrantic defect in the right yields enterohemorrhagic E coli W. Daniel Kovarik, MD eye and were full in the left eye. Re- (0157:H7).3 Earl A. Palmer, MD sults of a fundus examination The gastroenteritis, hematologic Portland, Ore showed a branch retinal artery oc- abnormalities,andrenalfailureinHUS clusion of the inferotemporal ar- resultfromthromboticmicroangiopa- Supported by an unrestricted fund from cade in the right eye with a partial thy.Escherichiacoli(0157:H7)initiates the Research to Prevent Blindness Inc, cherry- spot (Figure 1). She was a pathogenic cascade by elaborating New York, NY. complaining of intermittent numb- verocytotoxin.ThisShigalikeexotoxin Corresponding author: Michael ness in her right arm and her radial targetsglycolipidGB3 expressedonthe L. Klein, MD, 3375 Terwilliger Blvd, pulse was decreased on the right endothelial surface of intestinal, renal, Casey Eye Institute, Oregon Health side, with asymmetric blood pres- and central nervous system arterioles Sciences University, Portland, OR sures (right arm, 72/51 mm Hg; left of predisposed individuals. Once in- 97201-4197. arm, 110/61 mm Hg). ternalized by receptor-mediated en- Carotid ultrasound examina- docytosis, a toxin subunit inhibits 1. Purtscher O. Noch unbekannte Befunde nach tions showed an occlusive thrombus Scha¨deltrauma. Ber Deutsch Ophth Gesellsch. protein synthesis and damages the en- 1910;36:294. in the right common carotid artery ex- dothelium. Platelet aggregation and 2. Gass JDM. Stereoscopic Atlas of Macular Dis- tending into the internal and exter- ensuing thrombotic microangiopathy eases. St Louis, Mo: CV Mosby Co; 1997:451- nal arteries. Angiography confirmed 455, 746-747. result in target organ ischemia. Eryth- 3. Siegler RL. The hemolytic uremic syndrome. Pe- total occlusion of the innominate ar- rocytes suffer mechanical damage as diatr Clin North Am. 1995;42:1505-1529. tery with no demonstrable flow in the 4. Siegler RL, Brewer ED, Swartz M. Ocular in- they transit altered vasculature, result- volvement in hemolytic-uremic syndrome. J Pe- right carotid system, even in its su- ing in hemolytic anemia, while intra- diatr. 1988;112:594-597. praclinoid portion (Figure 2). The luminal platelet damage and aggrega- eye was most likely supplied by the tion result in thrombocytopenia.3 external carotid artery by retrograde Purtscher retinopathy and flow from distal branches of the in- Purtscher-like retinopathy are char- Delayed Branch Retinal ternal maxillary artery and thyrocer- acterized by multiple patches of reti- Artery Occlusion Following vical trunks. Warfarin was used as nal whitening, hemorrhages, and ar- Presumed Blunt Common long-term anticoagulation therapy teriolar attenuation in the posterior Carotid Dissection with no further sequelae. pole. Siegler et al4 first reported reti- nal involvement in HUS with fea- Ischemic events to the eye can occur Comment. Traumatic dissections tures compatible with sequelae of the after cervical carotid dissections.1 can result from multiple nonpen- acute manifestation of retinal infarc- Ocular vaso-occlusions usually oc- etrating injuries such as motor tion seen in our case. The basal gan- cur within 1 month after the onset of vehicle crashes, chiropractic ma- glia infarcts in our patient and that dissection. We report a case of trau- nipulation, falls, and hanging.1,2 Dis- of Siegler et al are a similarity sug- matic common carotid occlusion, pre- section of the carotid arteries is an gesting Purtscher-like retinopathy in sumably caused by dissection, which important cause of cerebrovascular HUS could indicate concurrent is- led to an ischemic event to the right ischemic events in young adults,2 chemia elsewhere in the central ner- eye 4 months after trauma. and embolism is the most com- vous system. monly presumed mechanism. The pathogenesis of Purtscher Report of a Case. A 19-year-old Common carotid dissections are retinopathy remains controversial. woman complained of acute pain- almost always traumatic and can re-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Our patient’s ischemic event oc- curred 4 months after her accident. In a prospective series of 80 patients with internal carotid dissection, Bi- ousse et al4 reported that the major- ity of strokes (82%) occurred in the first 7 days, and the latest occurred 31 days after the first symptoms of dis- section. In a retrospective series of 21 patients with traumatic carotid dis- section, Mokri2 reported ischemic events in 15 patients (71%), 6 of whom had focal cerebral symptoms 2 months to 10 years after the trauma. Of interest in our case was the lack of any cervical pain, , or Horner syndrome, the most com- mon symptoms in cervical artery dissections.2,4 Acute, painless, mon- Figure 1. Fundus photography shows a branch retinal artery occlusion in the inferotemporal arcade of ocular vision loss in a young adult can the right eye. Arrows outline the area of edema resulting from the occlusion. be caused by carotid dissection, ei- ther traumatic or spontaneous. The diagnosis of either common carotid or internal carotid dissection should be entertained when a patient is seen with an ocular stroke, even as long as 4 months after trauma. David G. Godfrey, MD Valerie Biousse, MD Nancy J. Newman, MD Atlanta, Ga Thisworkwassupportedinpartbyade- partmental grant from Research to Pre- vent Blindness Inc, New York, NY, and COREgrantP30-EYO6360fromtheNa- tional Eye Institute, National Institutes of Health, Bethesda, Md. Corresponding author: Nancy J. Newman, MD, Neuro-Ophthalmol- ogy Unit, Emory Eye Center, 1365B Clifton Rd NE, Atlanta, GA 30322 (e- mail: [email protected]). Figure 2. Left, Aortic arch angiogram shows total occlusion of the right innominate artery (arrow). Right, Cerebral angiogram, anteroposterior view, after left internal carotid , shows intracerebral flow of 1. Newman NJ, Kline LB, Lessell S. Ocular stroke right carotid system assumed by the left carotid via the anterior communicating artery and the right and carotid artery dissection. . 1989; posterior communicating artery. The right ophthalmic artery fills from left-sided flow (arrow). 39:1462-1464. 2. Mokri B. Traumatic and spontaneous extracra- nial internal carotid artery dissections. J Neu- sult either from direct trauma or ex- commoncarotidarterylikelyprogres- rol. 1990;237:356-361. tension of a dissection of the aorta or sively extended into the internal ca- 3. Hirst AE, John VJ Jr, Kime SW Jr. Dissecting an- 3 eurysm of the aorta: a review of 505 cases. Medi- the innominate artery. Our patient rotid artery and eventually reached cine (Baltimore). 1958;37:217-279. most likely had a traumatic innomi- theleveloftheophthalmicartery,with 4. Biousse V, D’Anglejan-Chatillon J, Touboul PJ, nate and common carotid dissection a resultant embolus then lodging in Amarenco P, Bousser MG. Time course of symp- toms in extracranial carotid artery dissections. that led to an internal carotid throm- the inferotemporal retinal artery. Stroke. 1995;262:235-239. bus and, eventually, an embolus that Ocular strokes have been produced a branch retinal artery oc- reported after carotid dissection. clusion. As our patient was a re- Newman et al1 reported 2 cases of strained passenger, with a seat belt central retinal artery occlusion fol- Autoimmune Optic over her right shoulder, and was lowing previously undiagnosed ca- Neuropathy asymptomatic for 4 months, we can rotid artery dissection. In 1 of the only postulate that a dissection was cases, the patient had been in a mo- Optic is a syndrome of vi- the cause of the . An an- tor vehicle crash 1 week prior to his sual loss due to inflammation of the terograde thrombus occluding the ocular stroke. . It is usually either id-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Figure 1. Case 1. Left, Image from skin biopsy specimen shows dermoepidermal and perivascular IgM deposition; right, dermoepidermal and perivascular C3 deposition (immunofluorescent, original magnification ϫ100).

iopathic or associated with mul- resonance imaging, chest x-ray films, 1 week, the patient’s visual acuity tiple sclerosis. Less commonly, it can cerebrospinal fluid analysis, com- decreased to less than 20/200 OU accompany other systemic inflam- plete blood cell count, liver func- and results of a visual field exami- matory disorders such as systemic tion tests, angiotensin-converting nation found markedly depressed erythematosus, , or sar- enzyme levels, antinuclear antibod- vision bilaterally. The presence of a coidosis. An entity known as auto- ies, antineutrophilic cytoplasmic an- dense inferior altitudinal defect in immune , also tibodies, erythrocyte sedimenta- the right eye and a loss of the I2e called autoimmune , tion rate, human immunodeficiency isopter in the left eye were accom- appears to be distinct from other virus serology, VDRL, FTA-ABS, panied by a 0.6–log unit relative forms of optic neuritis because it CH50, Lyme titers, blood cultures, afferent pupillary defect in the has a different clinical course with and urinalysis. The patient was again right eye. The appear- characteristic associated laboratory treated with the same course of cor- ance and findings of the remainder findings.1,2 ticosteroids with substantial im- of the ophthalmologic examination We describe 2 patients with au- provement in his vision; visual acu- were unchanged. A 4-mm punch toimmune optic neuropathy and ity improved during treatment to biopsy specimen of non–sun- typical abnormalities on skin bi- 20/50 OU. Ten days after treatment exposed buttock skin was obtained opsy specimens. The first patient is was stopped, his vision again wors- by the dermatology service. the youngest case of this disorder re- ened and he was referred to the The hematoxylin-eosin– ported to date and histopathologic Neuro-ophthalmology Unit of the stained frozen section showed a mild findings of the optic nerve are avail- Department of Ophthalmology and mononuclear perivascular infil- able on the second patient. Visual Sciences at the University of trate within the dermis and no leu- Iowa, Iowa City. kocytoclastic response. Direct im- Report of Cases. Case 1. An other- At that time, his visual acuity munofluorescent staining was wise healthy 17-year-old boy no- was 20/80 − 1 OD and 20/50 − 1 OS positive for IgM and C3 in a granu- ticed loss of vision in his left eye with a spherical correction of −6.50 lar pattern in the walls of small blood while watching television. After 2 OD and −6.00 OS and there was a vessels within the superficial papil- days of worsening vision, he was small relative afferent pupillary de- lary dermis. In addition, IgM and C3 found to have visual acuity of 20/20 fect (0.5 log unit) in the right eye. were found at the dermoepidermal OD and hand motion at 1 ft (30 cm) There was moderate bilateral optic junction in a granular to fibrillar OS. His left optic disc had moder- disc pallor, more in the right eye. staining pattern (Figure 1). No spe- ate edema; the right optic disc was A magnetic resonance image of cific staining was seen for IgA. These normal. He was treated for 3 days the and brain showed mild en- results indicated the presence of with intravenous methylpredniso- hancement of the retrobulbar right immune complex deposition, and lone sodium succinate (1 g/d) fol- optic nerve. Laboratory measure- the diagnosis of autoimmune optic lowed by an 11-day course of oral ments of the previously listed tests neuropathy was made. prednisone (1 mg/kg per day). His were again normal, including a re- The patient again received iden- vision slowly improved to 20/50 OS. peated cerebrospinal analysis for im- tical treat- Two weeks after discontinuing the munoglobulins, oligoclonal bands, ment. His visual acuity began to im- prednisone, the patient experi- and basic protein. In addi- prove on day 3 of this regimen, as enced a similar episode in his right tion, assays for anticardiolipin an- did results of his kinetic perimetry eye. General medical and neuro- tibody, rheumatoid factor, lupus an- examination. Ten weeks later, his vi- logic examination results were ticoagulant, and mitochondrial DNA sual acuity had improved to 20/25 normal. screen for most common muta- OD and 20/30 OS. Both eyes had re- The following laboratory stud- tions found in Leber hereditary op- gained the ability to see the I2e isop- ies were normal: brain magnetic tic neuropathy were negative. Within ter and the inferior altitudinal de-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Figure 3. Histological section of the optic nerve. Perivascular aggregates of chronic inflammatory cells are present without evidence of necrotizing vasculitis or optic nerve necrosis (hematoxylin- eosin, original magnification ϫ 200).

hand motion in the temporal visual field of the right eye, a medial or- bitotomy and optic nerve biopsy was Figure 2. Patient 2. T1-weighted magnetic resonance image (with gadolinium and fat suppression) performed, which showed chronic demonstrates optic nerve enhancement of the right eye. The lacrimal glands are also enhanced slightly. perivascular nongranulomatous inflammation and mild gliosis fect in the right eye had resolved. ity was 6/200 OD and 20/15 OS and (Figure 3). No immune complex The patient continued to take 80 there was a 1.8–log unit relative af- staining was performed on the op- mg/d of oral prednisone for 8 addi- ferent pupillary defect in the right tic nerve specimen. tional weeks and his dose was then eye. Goldmann perimetry showed a Later, an assay for antiphospho- tapered over a year. His examina- large central with an en- lipid antibodies showed elevated IgM tion findings remained stable for 2 larged blind spot in the right eye. Re- (12.6 IgM phospholipid units, nor- years. sults of the visual field examina- mal value, Ͻ11 units). An assay for Case 2. A 25-year-old woman tion of the left eye were normal. Her IgG was normal. In addition, a 4-mm experienced rapidly progressive vi- examination was otherwise unre- punch biopsy specimen of non–sun- sual loss in her right eye approxi- markable except the right optic disc exposed (buttock) skin was ob- mately 5 months prior to referral. showed temporal pallor, while the tained, which showed direct immu- The visual loss was accompanied by left optic disc was normal. Her nofluorescent microscopy findings pain with eye movement but no general medical and neurologic ex- suggestive of autoimmune connec- other symptoms. She was diag- amination findings were otherwise tive tissue disease, specifically IgM and nosed as having optic neuritis and normal. C3 granular staining of the dermal- was treated with the same regimen A magnetic resonance image at epidermal junction and focal granu- as patient 1. She experienced recov- that time showed mild enhance- lar deposits in small vessel walls. Her ery of vision to 20/25 OD over the ment of the right intraorbital optic visual acuity remained at light per- following 7 weeks. She then expe- nerve and both lacrimal glands ap- ception in the right eye and 18 rienced the same symptoms in the peared mildly enlarged (Figure 2). months later she developed progres- right eye 1 month later. A magnetic A laboratory evaluation, again simi- sive visual loss over a 6-day period in resonance image showed a small lar to that of patient 1, was re- her left eye. Visual acuity worsened scarlike lesion in the thalamus. She peated and showed no abnormali- to 20/40 OS and Goldmann perim- underwent a detailed evaluation ties. In addition, was etry revealed inferonasal depression similar to that of patient 1, with nor- suspected and bilateral conjuncti- and a cecocentral scotoma. mal results. The patient was again val biopsies were performed, re- She was pretreated with lithium treated with an identical regimen of sults of which were normal. A lac- carbonate to prevent mania and then intravenous and oral corticoste- rimal gland biopsy was performed given the methylprednisolone and roids and experienced improve- through an anterior orbitotomy with prednisone regimen described above. ment of visual function in her right the specimen showing no signs of Her visual acuity improved to 20/20 eye. inflammation. OS over 2 months. Results of Gold- She had a mild episode of ar- A trial of oral prednisone was mann visual examination returned thritis as a child and had occa- then initiated with some subjective to normal during this same time. She sional . She had improvement after the third day of is currently being treated with a very a brother and paternal aunt with sys- treatment. However, the patient ex- slow prednisone taper while taking temic . perienced mania with psychotic lithium. Following a third episode of features due to the prednisone, visual loss in her right eye, the pa- which was then discontinued. Nine Comment. Autoimmune retrobul- tient was referred to us. Visual acu- days later, with her visual acuity at bar optic neuropathy, now more

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 commonly called autoimmune op- cence microscopy showed IgM, IgG, can be difficult to diagnose unless it tic neuropathy, was first described IgA, or complement in immune com- is suspected and it is appropriately by Dutton et al.1 They described a plex deposition around dermal blood evaluated. disorder characterized by progres- vessels and at the dermal-epidermal sive visual loss and serologic evi- junction. Both patients displayed a Patrick Riedel, MD dence of an autoimmune disorder. similar IgM and C3 staining pattern. Michael Wall, MD Long-term or immu- For non–sun-exposed skin, the rate Allen Grey, MD nosuppressive therapy seemed to of immune complex deposition Thomas Cannon, MD promote visual recovery and pre- around blood vessels is between 0% Robert Folberg, MD vent further attacks. Affected pa- and 1.7%.4 H. Stanley Thompson, MD tients had laboratory evidence of Autoimmune optic neuropa- Iowa City, Iowa an autoimmune phenomenon but thy should be considered when- lacked the clinical manifestations ever a patient with optic neuritis has This study was supported in part by of a systemic collagen vascular an atypical recurring course. This grants from Research to Prevent Blind- disease. Several groups have at- usually is seen as recurrent bouts of ness Inc, New York, NY, and a De- tempted to identify reliable labora- visual loss as treatment is tapered or partment of Veterans Affairs Merit tory markers (antinuclear antibod- stopped. Our first patient was ini- Review. ies, anticardiolipin antibodies, skin tially seen at age 17 years, and is to Corresponding author: Michael biopsies with immunofluorescent our knowledge the youngest re- Wall, MD, University of Iowa, Col- staining) to allow earlier diagnosis ported patient in the literature with lege of Medicine, Department of Neu- and treatment.2,3 Use of these mark- this condition. Our second case is rology, 200 Hawkins Dr, 2007 RCP, ers is complicated by negative re- important for 2 reasons. First, it Iowa City, IA 52242-1053. sults when prior pulses of cortico- shows the importance of long- 1. Dutton JJ, Burde RM, Klingele TG. Autoim- steroids have been given. term, slow tapering of immuno- mune retrobulbar optic neuritis. Am J Ophthal- Abnormal skin biopsy findings therapy. Second, this case provides mol. 1982;94:11-17. were the most consistent markers of the first histopathologic study of op- 2. Kupersmith MJ, Burde RM, Warren FA, Klingele TG, Frohman LP, Mitnick H. Autoimmune op- autoimmune optic neuropathy ac- tic nerve tissue affected by autoim- tic neuropathy: evaluation and treatment. J Neu- cording to Bielory et al.3 Hematoxylin- mune optic neuropathy. It implies rol Neurosurg Psychiatry. 1988;51:1381-1386. 3. Bielory L, Kupersmith M, Warren F, et al. Skin eosin staining typically showed a peri- autoimmune optic neuropathy is a biopsies in the evaluation of atypical optic neu- vascular infiltration by lymphocytes disorder characterized by chronic ropathies. Ocul Immunol Inflamm. 1993;1: and neutrophils, which was found in perivascular nongranulomatous in- 231-241. 4. Blenkinsopp WK, Clayton RJ, Haffenden GP. Im- the biopsy specimens from both of flammation. These cases remind us munoglobulin and complement in normal skin. our patients. Direct immunofluores- that autoimmune optic neuropathy J Clin Pathol. 1978;31:1143-1146.

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