Journal of Neuro-Ophthalmology, March 1986, Volume 6, Issue 1

Optic neuropathy secondary to lymphoma.

Optic neuropathy secondary to lymphomatous infiltration of the optic nerve developed in a patient who was in complete remission from treatment of a non-Hodgkin's lymphoma. This is the first reported case of an isolated optic neuropathy occurring in a patient in clinical and laboratory remission of non-Hodgkin's lymphoma. Recognition that such a complication can occur is important in that prompt treatment may lead to reversal of visual loss, as it did in this patient.

"_. lYH6 Rawn Pre's, New York Optic Neuropathy Secondary to Lymphoma Marilvn C. Kay, r-..1.D. Optic neuwpathv ~eC,)nd,ln' t,) Ivmph,l[n,ll')lI~ infiltra· li,'I1 ,)f the ,)ptic nern' dl,,·t'l,)ped III <1 patil'nt who was 111 c,)mplete remi~~i,)n (r,)m tre,ltment "f a non­H" dgkin's Ivmph,'m,l This i~ the tirst reported case of an i5"latt'd ,'ptic neufl)p,lllw ,)ccurring in a patient in clinical and I,lbl)rat,)rv remis~i,)n "f non-Hodgkin's Iym­ph,' ma. Rec,'gniti,)n th,lt such a complication can occur is important in that pr,'mpt treatment mav lead to re­wrsal" i "bUdl 111ss, ,15 it did in this patieni. From the Department of Ophthalmology, M,'di,',)! ColIl'g<' of Wisconsin, Milwaukee, Wisconsin. Address correspondence and rl'print rl''1ul'sh to M. C. Kay, M.D., Department of Ophthalmology. Ml'dical Collt-gl' 01 Wi,­consin, Milwaukee, WI 53226, U.S.A 3/ Optic neuritis secondary to infiltration of the optic nerve by lymphoma usually occurs in a re­currence of systemic lymphoma or as part of the initial presentation of the disease. Often, other cranial nerves are involved as well. Recently, a pa­tient in clinical and laboratory remission of non­Hodgkin's lymphoma, whose only complaint was decreased vision in one eye, was examined. The visual loss was found to be secondary to a lesion of the optic nerve that represented an isolated re­currence of lymphoma. Prompt treatment, after recognition of this etiology, led to recovery of vi­sion. CASE REPORT A 55-year-old Japanese-American woman re­ported that for 1 week vision had been decreased in her left eye. Her only physical complaint was that of mild fatigue related to the cyclic treatment of undifferentiated histiocytic lymphoma. She had undergone her most recent intravenous chemo­therapy 4 weeks before, She was in clinical remis­sion. Examination revealed a visual acuity of 20/20 in the right eye and 20/400 in the left eye, with an afferent pupillary defect on the left. Extraocular motility was unrestricted. No proptosis was evi­dent. Goldmann perimetry revealed a dense infe­rior altitudinal field cut with a cecocentral scotoma of the left eye (Fig. 1). The right visual field was normal. Examination of the fundus showed marked disc edema on the left, The patient underwent a computed tomography scan, which showed mild irregularity of the left optic nerve intraorbitally as well as some mild contrast enhancement of the left optic nerve intra­cranially just as it entered the chiasm (Figs. 2 and 3). No other parenchymal involvement of the brain was seen. The patient was placed on predni­sone therapy, 60 mglday, but on repeat evaluation 3 days later visual acuity had declined to hand movements and she reported a mild headache. .»'- M. C J(I1Y LEFT FIG. 1. Visual field defect In left eye at first examina­tion. Visual acuity. 20;400 Spinal fluid examinatipn re\'ea!l'd an llpening pressurt' uf 310 mm pf w<lkr, a pWkin iL'\'l'1 pf ]().J mg/ml, a glucuse levt'luf 22 mg/ml, and nUlllt'rpus lvmphuma cells, Radiatiun therapv tu tlw r\.'tw­bulbar optic nerve and suprasellar uptic nerve was begun immediateh', Further workup fur S\"stemic recurrence of lymphoma was performed and was entirely negative, including gallium scan, com­puted tomograplw scan of the abdumen and chest, bone marrow biopsv, chest x-ra,' film, he-mpgram, and liver function tests. After the sewnd treatment of radiation therapy, the patient re­pprkd impwvement of vision. By the fourth day pI radiatiun therapy, visual acuity was 20/60 on the Idt An Ommaya reservoir was placed and five cuurSl'S uf intrathecal methotrexate, Solu-Medrol, <lnd ara-C were given. The patient's vision con­tinued tp impwVl' following initiation of whole­brain radiatilln, Urll' mpnth after the initial examination visual acuJlv was 2(}/2::; in the left eye, with moderate cunstrictilln of the I-2e isopter on Goldmann pe­rinwtrv (Fig . .J) Further follow-up 3 weeks later Shll\\'l:d cum plete resplu tion of disc edema, The patient's \'isual acuity was 20;20 in both eyes. DISCUSSION Leptomeningeal or parenchvmal central nef\'llUS svstem "'mphoma was once thought to bt' extremeh' rare, pwbabh' because most patients "uf\i\ed onh' a shurt time after the initial diag­nusis uf h'mphuma, Leptomeningeal or paren­ch,' mal spread in Hodgkin's disease is still quite rare, but it occur" much more frequentl~- in non­Hudgkin'" h'm phuma (l-.J), In 1971, Griffin et al. (:;) di"cu""ed 21 autop"ied patient", examined oyer a period of -; \"t'ar" , who had de\'eloped central ner\'llU" "\',,tem non-Hodgkin'" I~'mphoma. In lY7b, Bunn et al. (.J) repl1rted a stud,' of:;2 patients FIG. 2. Computed tomography scan of brain, Note increased contrast enhancement of left optic nerve at Its intracranial end (arrow­head) IYtvI['l!l )tv1/\[l JUS l J['IIl' NUI/,( )['/\/1 I'r 33 FIG. 3. Computed tomography scan of brain. coronal view. Note Increased contrast en­hancement of Junction of left optiC nerve and chiasm (arrowhead) ~utferin~ twm hi~tiocYtIL- l\'mpllllmel in whidl l-l (2lj'; ) de\'t,'ll)ped central IWrn1LI"; ,,;\'..;!L'm 1\'111­phoma, Fi\'t, of the..;e patienh were in remi..;..;iun l)ther\\'l~e, Thirteen l)t the l-l peltIl'n h held Il'ptl)­meningeal IYmphuma, Of Inll're..;t I"; the fact that 10 Llf the..;e 13 patient..; held e\'Idl'ncl' uf bunl' marww lIl\'ul\'ement either priur tu the develup­ment uf the cen tra I nen'Llus ..;vsll'm Ivm phOmel Llr at the tIme Llf the original diagnu..;i..; uf l\'mphuma, In this series uf patients, decrea~l'd mentatiun, headache, and CLlma were cumnllln finding..;, Fuur of the patIents had multIpll' cranial IlL'r\'l' pal..;ie..;, specificallv IIl\'ul\ing cranIal Ill'n'l'" III, IV, elnd VI, but nLl mentiun wa..; made ut till' dlllilell prl'''l'ntel­lions of the"e patients, \:0 uptic nl'Urupeltll\' I\'el"; reported, In all patienh frum whum "pinal tluid was obtained, cvtulogv wa..; pU..;itiVL' tor lym­phoma cell..;, Bunn et al. theurized that the high correlation of bone marrow invul\'l'ml'nt with ..;ub­sequent central nervous ..;yskm invulYement Wel"; the result of the direct ..;pread of the Iymphumel from the medullary cavity along perlorelting vessels and nerves through the dura intu the (l'll­tral nervous system, They recomml'nded thelt in evaluating a patient with previuus Iymphumel will> develops neurological com plain h, el cum pUll'd tt,­mography "can must be pertorJlll'd lirst tu ruk uut a mass lesion or hydruCl'phellus, HUI\'l'\'l'r, tlll'Y emphasized that a spinal tap i..; nl'Cl''';Sclf\' tu luuh­for abnormal cells as well as to documellt prull'ill and glucose levels, Others have reporll'd clbllllr-mally high cerl't)ru..;pinal fluid l\'sozvme le\'els as indicator..; llf central ner\'l)US sv~tem Ivmpllllma, although leve!>. are elevated in multiple ..;clero~is, lue~, and other ct>ntral nen'ou~ ~v~tem malig­nancies (11), Venab1l's et al. (3) reported a ~imljar group ot patients in Ilj7lj in which there was a 12( ( incidence of central rll'rvou~ sYstem l\'mphl)ma in LEFT FIG. 4. Visual field of left eye 1 month after the initial examination Visual acuity, 20/25 I ellll NCllfl1-nphflllllll1o/, \'01. 6, No.1, 1986 3.J ,\ I. }\;\ ) p,llil'llh \\'jlh 11<'111 todg"ill'" 1\'llll'hOIll", lilt' 111<,,,1 l'lllll111011 I'rl'''l'IlI"lillll ill 111.11 "I'rll'" \\''''', ,lg,lI 11 , l11ullIl'l,' IT,1I1i,,1 Ill'r\'!' 1',11"11''', 110 l'l'lil Ill'U­n'I', llh\' \\,," rq','rkd 111 111\ "1l0\\ll'dg", "11 I"ol,lll'd 01'111 Ill'urlll',llh\' "1'I',"hLll'\ h' ",'n'"d III Illlil Illldg"IIl'" l\'Illl'holll" ill ,1 1',11 il'll I l\'Illl i" illllillil,1I "'ld 1,11'01',1101'\' 1'1'1111"­" illil h,h 11111 [ll'l'll rq'llrll'd 1'I'l,\'jou"h' :\lth'lugh I,tlll'r 1'1',1111,11 Ill'rn' 1',,1"11'''' 1'''l'n'l'llh' 01 IT.lIlI.l1 1ll'I'\I'" III. 1\', \'1. ,lIld \'11. h.l\'l' 1>1'1'11 I'l'l'0rlnf. thl'\ h,l\'I' ,11\\',1\'" l1ll'UITl'd ,II 11ll' 111111' III till' Illiti.ll di,lglll l"I" 111 h'I11I'I1<II11,l or durillg ,I "I'''ll'lllll rl'­l, ll'''l' l'l"l'wlll'rl' (I h) I \\',1" ,lbk 10 lind 111 111l' IIt­l'r, llurl' tIl'll 1',l"l'" III 111'111 Ill'urol',ltln' "I'llllld.ll'\ III nl ln-III,dg"ln'" 1\'I))phl1l11,l (h,7), In 011l' 1""1', rl'pllrll'd b\' 1\.1',111" ,1nd U'Rl1Ur"l' (h) in II/h\, llplll 11l'Urllp,llln' 1lllUrrl'd III ,I p,llil'nl 1\llh unllln­Iwlit'd h'l11phl'''.Hll1l11,1. 11ll' p,llil'n!'" llplll Ill'U­rop, 1Ih\' \\',1" rl'n'r"l'd \\'lth inlr,l\'l'nOU" "drl'I1<'­cllrlicl, l fl Iphi n Ihl'r,I pI' ,1 nd prl'd 11 i"IIII 'Ill' Hl'l,l U"l' l,llhi." I\.r,HI" ,1I1d U'Rour"I' "uggl'''ll'd Ih,ll dl'I11\'­l'Iinalilln l11ighl bl' 11ll' I,W"l' "I thl' 111'111 nl'lIrllp­, lIlli', lhi" h,b ,1"'0 bl'l'n hinll'd ,II in !'l'p"rh 01 "pin,11 1l1l't,hl,1"i., "I 1\'I))ph"I11,l ill \\'hll'h "Il'rlnd" rl'I't,'r"l'd Ihl' ,1bnl'rl11,1Iilil'" I'), IIlI\\'I'\'I'r. \\'ilh Ihl' ,1dn'nl l,l CIl(llPUll'd l"I11"g­raph\', \\'1' .HI' morl' li"l'h' t" "1'1' ,11l1l,11 "lrllllllr,ll changl''' "l'c"ndal'\' Il' 1l1l'1,1"1,1"i", ,I" "llllrrl,d III m\' p,llil'nl, \\'hich "lIggl'''t-, Ih,ll Ihl' inliltr,llllln "I thl' nl'l'\'l' is morl' likl'h' Ihl' I,W"l' "I thl' 111'111 1ll'1I­wpattw, Wil"ins and S,lIl1h"urI (2) 11/7lJ, rq'orll'd ,I Ihird-Ill'rn' pals\' "clurrin,~ In ,1 (',llil'nl \\'111' h,ld a prl'\'I"U" hi"lor\' 01 n"n-lI"dg",n'" I\'Ill I'Iw111 ,1 and \\'hll did n,,1 rl'sp"nd 10 corllcll"ll'nlld Irl',ll­ml'nl. Un 'lUlops\' Ihl' Ihird-nl'rI'I' l,bCicll'" In thl' midbr'lin \\'1'1'1' dirl'llh' innl!ll,d b\' 111111'11<1111,1 Likl'l\' i"l', in ,1 ca "l' ot ,111 "rbi 1,1 I ,lpl'\ "\' nd n Inll' "l'L"nd<lr\' to non-Hodgkin's lymphoma reported bv Bullol:k l'I .11. (7), at autopsy the patient's optic Ill'rn' \\,.1., inlillr,lll'd bv Ivmphomil and was not dl'm \'t'l in,l It'd, !\ggfl'""in' Irl',lln1l'nl \\'ith radiation therapy can rl'\'l'r"l' 1111' \,j"udl 10,,'" in Ihl'sl' caSl'S but because dl'n1\'l'lin,llioll i" probabl\' Ilut the etiology of the optl( 1l1'U 1'01',1 t Ill', (llrlicu.,ll'ruids probably do not h,1\'I' "n I 111 port,lIlt roll', Rddialion Iherapy to the rl'lrobulb,H l'pll( Ill'r\'l' and intracranial optic lll'rI'l' ,,/wuld ldU"l' ,m imml'diate rl'sponse if the d,lmdgl' IfI'l11 11ll' Inli/trilll\'l' tumor is not too gn',ll, :\llhl'Ugh "nt' mu,,1 ,11\\'a~'s think of sys­Il'm Il rl'l,lp"l', In IhI' "l't II ng III nt'\\' nl'U rological (ompl,lInh, I hd\l' ducurlll'nll'd in Ihi.., case that h"l,lll'li llplll [1l'ufl'path\' "l'cundary to I\'mphoma can occur In ,1 p,llll'nt 111 clinical and laborator\' re­1111"" I,,n REFEREJ\jCES 1.1" I I' I 'I, ~ I " IlI"m I ,111.1 Ikr~"\In, r R In­\ IIh t:lllt.'nl Ilf tilt' 'l'lltr,d Ilt'r\ \Ill'" ... , "'(l'n1 In I1llr1-thlJglo..ln' .. hm~""h"rn., "-.;,:,, ~b: 22;-:-;1 l~-; ~ 1\ i1~If" I' f ,If"! '-.1mh"u" .\ .\1 1,,>I.lteJ bliatl'ral l'",'U]t'1l1"tl 1r F'·lrl· ... l ... dUl' :,, 1\ r,.'~. h,'m ..l \t"I.rt1/\,\'U 29; 14:;-142.... ]lj-1.4 \ ,'n.,I'''"- t, '- I'r,',t,'r ... I H,lt", D CartbJht', :\ f I .lnd '-h.tI, [) '\ Inlr.ldanlal J"",hl' In n,'n­H" d~~J1)' "ml'h"Ill,' t...' i \1,.: .J'l: J II-l~1 14~lI, ~ Ilunn 1' ..\ '-,Ill'II) I' '- 1',lI)Io." r.\1 ,InJ Dt'\"la, \ I l t'ntr.ll I1l'r\,llI'" ... ' ... tl'1l1 \.'\l(l1r~h\.-~1th~n... In r,jttt'nt~ \\'llh d'Il,'"'nt,,,!>',1 h"t"", t" ,lIhl unJ'll,',,'ntt.Hl'J "'m­~"" htln1.1 Il'lI~l·fl'll.1 rl'\ I"'ltl.,j !'," ".: 4:"": ,":._ hi 1\J~h (,"ttln I 1\ I h"IllI",'n I, II .\lit'h'n','n,.\1 I JC'- 1-..1,'" I,'r I l ,If"! \\ ,,11.1I,,1 ~ Ii L, ml'h,'mal"ll' It'pt,,­nh'n][ h~ltl"'\"" r \1.',; 51: 2\111-21 1..... , ]4:-"1 " I-..r.lLI' \ .\1 ,IThi l ) ",'ur~,' I I, Illl'h"m,)l,'u, ,'pt" nC'lI­ntl'" '\" i,' l ):';:1',',;,":,'. 70: 1-,":.- J-; l~t<, - I\ull",~ I (1 ) .1f'" H 1-..,,11, .\1 dnd .\kD,'nald, L \.l~,n',ti~l\.i~h.ln ... 1\ 111~""hlln1.l )[1\\,1\ 111.~ tht.' ll~"'tl"'" nl'n't' AmI l':"::':,;,":.': II: I~-- I~"l' 1"-4