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Show /. Oill. Nfllrt1-IJl'iJtiJll/llh1/. 5: 63-/16, 1985. Homonymous Hemianopia and Systemic Lupus Erythematosus ELIAS l. TRABOULSI, M.D. AHMAD M. MANSOUR, M.D. MILHIM I. ASWAD, M.D. WAllO GHARZUOOIN, M.D. RIDA A. FRAYHA, M.D, F.A.c.r. Abstract The case of a 23-year-old woman with systemic lUpus erythematosus and homonymous hemianopia is presented. A calcarine cortical infarct occurred as a result of a postpartal exacerbation of her disease and was demonstrated by CT scanning of the brain, The neuro-ophthalmic manifestations of SLE are reviewed briefly. The authors propose to include collagen vascular disease in the differential diagnosis of homonymous hemianopia in the young age group. Introduction Lupus erythematosus can produce visual loss hom retinaL retrobulbar, or intracranial involvement i-I: Documented homonvmous hemianopic visual field defects are rare' manifestations of the disease and are presumed to be due to cerebral lesions. 7 ](1 At times, they may be the most prominent and disabling feature of the disease. lo A 23-vear-old woman, with recently diagnosed systemic lupus erythematosus, presented to the ophthalmologist 2 weeks after an abortion with a right homonymous hemianopic visual field defect A CT scan of the brain revealed a recent infarction of the left calcarine cortex. The defect remained unchanged on subsequent follow-ups. Case Report A 23-year-old woman presented to the eye emergency service with acute loss of VISion In the right half of her visual fields. She had systemIc lupus erythematosus, diagnosed dunng her last From the Department' (If Ophthalm(ll(lgy (EIT. AMM, MIA) and Internal Medicine (WSC, RAF), American Umver"ty (It Beirut. Medical Center, Beirut. Lt.'banun. March 1985 pregnancy, which eventuated in an intrauterine fetal death and abortion 2 weeks prior to her present illness. The patient had arthritis, a proteinuria of more than 0.5 g/24 hours, a positive antinuclear antigen tesl, a high anti-DNA titer of 68.4 U/ml, and Raynaud's phenomenon She thus fulfilled the diagnostic criteria for SLE set by the American Rheumatism Association (ARA) in 1982D The general physical examination, including a detailed neurologic evaluation was normal except for the visual field defects in both eyes. The blood pressure on presentation was 130/80 mm Hg. There was no history of migraine. On specific questionning, the patient reported a tightening type of headache and she also noted seeing flashing and distorted nonspecific images on the day that preceded the visual loss. The ophthalmological e:l.amination revealed a vision of 20/70 in the right eye and 20/40 in the left eye; the intraocular pressures were normal; the visual axis was clear and indirect ophthalmoscopy revealed normal maculae and discs in both eyes. There was no afferent pupillary defect and the pupils reacted equally well to direct and contralateral light stimulation. Visual field testing by confrontation and Goldmann perimetry (Fig. ]) revealed a right hl)mlmyn1llus hemianL)pia splitting the macula in both eyes. A CT scan of the head revealed ,1 recent inf.uct of the left calcarine corte:l. (Fig. 2) Nl) llther abnormalities were nllted. A lumbar puncture was done ,lnd revealed nlHmal protein and glucose concentrations ,1nd ,1 white cell Cl)Unt llf 14 Iymphocytes/mm' of cl>rebwspinal fluid with no red billod cells Sl'en. Thl' pdtienl was st,uted on S\'stemic steroids and dischargl'd. On follow-up at 2 weeks, 2 months, and 0 months, repeated Goldmann periml'try showed persistence of the homonymous hemianopia confirming the dic1~nosis of an infarct. The patient dl>vell1ped no further neuwlugic deficts ilnd is still receiving maintenance stewid therapy. 63 \ \ \ \ /' 210\ 22~ Figure 1. Right homonymous hemianopic field defect splitting the maculae of both eves, '" 270 / / ---/' ,/ --- ~~'-L~ 2~~ Figure 2. CT scan of the brain showing a tri,mgul.n art',l of increast'd uptake (arHlwheads) in the region <If distributi<1n of the left po,f<orior cerebra' artery. mostly in th<' cakarine «)rte, (with enhancemt·nt). 64 Discussion Systemic lupus erythematosus is a multisystem disease of unknown cause. Its hallmark is the presence of a number of antibodies to nuclear components. but other immunologic abnormalities exist as well. Viral infections, genetic predisposition. and abnormalities of immunoregulation appear to playa role in the etiology of this disease.' 4 The central nen'ous system is im'olved in 2575% of the cases,' and ,,:as once second to uremia as the most common cause of death in patients with SLE. It OL)\\' accounts for only 10% of deaths mainly because of improved drug therapy.151/> The mllst common neurok)gic abnormalities associated with SLE art' seizure disorders followed bv mt'ntal disllrders. cranial nerve involvement, and transverse nwelitis. 14 The neuro-l1phthalmic manifestations of the dist'ast' have bet'n extensively reviewed by Lessef and can be due to anterior visual pathway, retrochiasmal. or ocular motor nerve involvement. They include (Table 1): Papilledema, a pseudotumor cerebri-like picture, homonymous hemianopia. optic and chiasmaI neuropathy, transient amaurosis, geniculocakarine blindness, visual hallucinations, internuclear ophthalmoplegia, transient ptosis, and diplopia. SLE patients who develop hemianopic field Journal of Clinical Neuro-ophthalmology TABLE 1 Neuro-ophthalmic Manifestations of SLE' ~ llhr.lll1l'~hJ..l' SI'ndwn1l' .2 r'Sl'Udlltllllll..)r lI"-l' picture J Antl'ril..lr Ischl'lnll' t1ptll 11l'Urpp.lth" 4 llplll .ltwplw lll)nhln\'llhlll~ lll'llll,lIlllpl,l t> \',,'u.ll h.l!!ulln.ltlllllS: t"Ill1l'd .llld untlllllll'd - Intl'IIlUlk.H ,'phth.l!mllpl"h,.1 ~ Tr.lIlSll'nl pt",,, '-l Plp!,'pl.l ddl'cls lliten h,1H' ~lgn~ ,md ~ymptom~ th,1t indic, 1tl' lh~iulKtion lli tIll' br,1in ~tl'm ,H1d thi~ indi'~,1tl'~ ImllIH'n1l'nt lli the pll~terior circulatilln \l.1ll1r cerebr,11 ve~~el llcdu~ion in SLE ha~ been demlln~tr,1tl'd by Tre\'lH l't ,11. 1 - Tlwre are, hl)\\·e\·ef. nll hi~tllP,1thlllllgic ~tudie~ oi these types lli llcdu~illn~, ,md tll llur knowledge no CT SC,1n denlllnstr,1tilln l)i ,1 calcarine infarction causing a hllmllnVnlllU~ hemi,H1opia in a patient with SLE In their repl)rt l)f1 the cerebral disorders of \Isilln in SLE. J " Brandt. Lessel and Cohen ~tressed that these disorders are not widely appreciated, that they occur due to cerebral vascuI1tis, and that at times, thev may be the most pmminent and dlsabl1ng iea-ture ,;i the disorder. Thev abo report lln the association of visual hallucinatIOns, iormed and unformed, with the iield defects Lniormed hallucinations consist of spots, I1ghts, patterns, !lashes, or vague shapes? Our patient presented with a total homonymous hemianopia that interfered markedly with her daIly iunctions On specific questi"oning, she reported the unformed hallucinations and the headache. The cortical iniarction in our patient occurred :2 weeks aiter her abortion SLE is known to !lare up dunng the pustpartum period and high dose steroids have been advucatl'd to prevent the exacerbatiun uf the dlsease. I' Our patient did not receive any steroids during ur immediately aiter her abortion and we bel1eve that the cortical infarction is due to an acute exacerbation of her basic disease. It is bel1eved that the cerebral infarcts in SLE are due tu destructive and prul1ferative change~ in arterioles and capIllarie<,. The characteristic lesion is a fibrinoid necrosis of the ves~el wall I1ke that seen in hypertl'nsive encephalopathy, unaccompanied by a cellular infiltration." I fypertension is frequently associated with SLE when there is nephropathy and might be contributory to the encephalopathy Our patient was not note~i to have an elevated blood pressure on repeatl'd measurements. Large vessel involvement in SLE does occur, I although it seems rarer than the smaller ves.,l,1 March 1985 Traboulsi et a!. di~l"lse We believe that a postpartum flare-up of CNS disease in this patient has led to occlusion oi the posterior cerebral artery or its calcarine br,1I1ch in ,1 cortex with compromised circulation bec,Hlse oi the basic small vessel disease and the w.lterslwd area loc.ltion of the calcarine cortex. There are abnormal1ties of regional distribution of oxygen utIlization and blood flow in the great m,1jority of patients with CNS lupus, and this has been demonstrated using l'Oc perfusion studies]" CT scan of the brain al~o shows a diffuse cortical dise,1se in these patients 211 It is thought that older patients with isolated homonymou~ hemianopia have an exceedingly hIgh prubabil1ty of having a vascular lesion whIle younger patients may have either congenital or acquired nonvascular etiologies. c, cc A CT scan is recommended in the workup of the latter group to determine the etiology of the process. Our case demonstrates that a vascular etiology, possibly of the same nature than the one which occurs in hypertension, might lead to an isolated homonymous hemianopia in a young patient with a collagen vascular disease, in this case SLE. The CT scan has again proved to be the best diagnostic tool in the hands of the neuro-ophthalmologist and has provided irrevocable evidence of the responsible intracranial pmcess. The CT scan has been used previously in the delineation of the CNS lesions in SLE.clI The most commonly encountered are small areas of infarction and hemorrhage that correlate well with the clinical findings and corroborate the reported pathologic changes. References 1. Gold, D.H., Morris, DA., and Henkind, r Ocular fll1dings in Systemic lupus ervthemalllsus. Br. f 0l'hthalll/I'1. 56: 800, 1972. ') Dultun, II, Burde, Rr-.t, and "lingek, TG Autllimmune retrllDulDM Uptil neufltis. AliI I Lll'iItha/III"/ 94: II, lllK2 .' Nuwld.l, N.. f1w ululM lll,lnltv,l,ltllll1S Ilf luIL1~,'n di~l',I'l'S ,lnd uf .1~l'nh USL'd In thl'lr trL',ltnwnt Mt'd CIII/. N,,/tll AliI 49: 1.11, ll/h'i 4. W.11sh, FB, ,md Iluvt. \\' L. S\'.,tL'lllll lupus l'1\111L'lll, llusu~ In CIIIII'/il ,\'I'III"-"I'litll,lll11<l/"\I/, 1',,/ 2. Wrlil.1m~ & WrI"ilb, B,l!tll1111rL', I l/bL) lh.;p K, p Ilbh. B,lltinllllL', jl/hl/, lh.lp K, P Ilbh 'i luhn,"n, R. r ,.lnd 1'll~h.lrd"'lf1, E.I' . Tlw nl'ufll!llg1l ·.I1 1ll,1I1ifl"t,11Illns ut ~v~tl'lllil lupus l'rvthem,1Ill~ II~ AI,'''I, III,' (Ha/I) 47: 337, jl/(,K. h Ad,lfn" I, [), ,1I1d Yiltur, M.. CL'reDW\'.1SlU1M disl'. l"'~ In ["1111'11''''' "I Nt'lIn'/"\I/. McGr.1w-Hill BUll" Cu, NL'W lurk Ill77, d1.1~<·2R, P 550. 7' I ,l'........t,L ~ rill' !It'urn llphth~lltnlllpg~' nf s\'stenlic 11II'Ih ,'r\·thl'Ill.lt<I~II~ P,I,' ()/lhtll,lllll"/. 47: 1.1, 11I7') H 11.1l·"dt. I 1\, M.lItinl'l, Rn, LMslln, I'.F., and 1'.IddNln, I\.r-.l . Oplil nL'unti~ 111 svskmil lupus (T\'tl1l'lll,ltu~II". :\/dl. Nt'lIn'/. 31: ll, 1~974. 65 1I Wun~. " .. Ai, E., Vl'rril'r )um'S, J.. .md Ytlun~, D.: Vi~l"ll lu~~ .l~ thl' initi.11 ~Ymptum~ uf systl'mic lupu~ l'r\,thl'm.lhl!'>us. 1\11I. ,. 0//11,,,111I11/. 92: 238, 11IK I. Ill. Br.lIldt. ".P.. 1.1'~~d, S..•lIld Cuhl'n, A.5.: Cl'rd,rdl di~l'rdl'r~ "f \'i~i,,,, in ""~tl'mil' lupus l'rythl'matu~ u~. AIIII. 11111'",. M,','. 83: Ih3, 11175. I I. Cilll'fr,., IU., .lIld Frl'n!.d, M.: Systl'mk lupu~ l'r· \·t111'm.lt,.~u~ pn'!'ol'ntin~ .1S lIptk nl'uritis. 1\,11I. (l/,II"",IIII"I. 10: 5<;q, 11I7K. I:!. Sh'udl'min', A.. Stur!.. M., Sinll'1. D., .lOd HlIupt. 1.1..: Nl'uro-llphth.llmk ,,"slt'mk lupus l'rylhl'm.l· h~u~ mi~i.I~'lU~I'd .I~ hV~!l'ril"ll blindm'ss. 1\11I. /. P~lId,i,,,,,, 139: 11114, IIIH2. J.l Toin. E.tl.i.. Cl.hl'n, A.5.. Frit'S, I.F., l'l .11.: Thl' 1982 rl'\,iSt..,j l"ritl'ri.l fl.r thl' das.'iificatiun uf systl'mic lupus l'rythl'mJtusus. Arll"i'i~ Rllc'um. 25: 127\, IQK:!. I·t tl.1.lI\nkl.. M., .md CiIIilJnd, B.e.: Systl'mic lupus l'r\'thl'mJtllSu~. In HlIrri~",,'~ Prillcil'll'~ Ilf III',.,IIal A"'di"i,,,, (Wth l'd.). McCraw-Hili Buok Co.. New '1lltr!.. 111M3, p. 387. 15. Fl'in~lass. E.)., Arnl'lt, F.e., Dorsch. e.A., Zizic, T.M.. Jnd Stl'\'l'nS, M.B.: Neuropsychiatric manifl'stJtiuns of systl'mic lupus l'rythl'matosus: Diagnl'Sis, dinicJI spt'ctrum Jnd relationship to other fl'Jturl'S of thl' diSl'a§e. Mt'tlidllc' (Ball.) 55: 323, IQ7h. 66 Ih. Oubuis, E.l.. Wil'rzehowiccki, M., Cox, M.B., and Wl'inl'r, I.M.: Duration and death in systemic lupus l'rythl'mJtosus: An analysis of 249 cases. '.A.M.A. 227: 13qll, 11174. 17. Tn'vur. KP., Sundhl'iner, W.F., Fessel, W.]., et .11.: Angiu~raphic dl'monstrations of major cerebral \'l'~sd ucdusiun in systemic lupus erythematosus. N,·/lrll""till/ll,o.:11 4: 202, 1972. I H. Finl', C.L.: Sy.,tl'mic lupus erythematosus in pregnanlY A,,,,. III'a". Med. 94: 667, 1981. III. I'ind;ing, A.I., Trdwrs, R.e., Hughes, C.R.V., et al.: (hygl'n- I 5 brain scanning for detection of (l'n'bral In\'lIln'ml'nt in systl'mic lupus erythematl"' U". L,,,,t"c',I: H9H. 1978. :W Bil.mlUk. L.T.. Patd. 5., and Zimmerman, R.A.: ClImpult'd tllmtl~raphy tlf systemic lupus erythemah... u". Ra,li"I".'':1I 124: 119. 1977. 2I. ~mlth. I L: tfllmunymllu.., hemianopia: a review of IIlCl (a'>l'" Alii. /. O,,',lIlallllll/. 54: 616, 1962. 22. Trobe, J.D., lorber, M.l., and Schlezinger, N.S.: Isolated homonymous hemianopia. Arch. Ophtha'" 1Il1. 89: 377, 1973. Write fClr repri"t!' to: R. A. Frayha, M.D., P.O. Box 113-6044. American lini\"ersit\' of Beirut. Medical Cen-ter. Beirut. lebanon. . )llumal of Clinical Neuro-ophthalmology |