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

Adult-onset cyclic esotropia and optic atrophy.

A 37-year-old man developed periodic esotropia with 4-day cycles. He was unique because of his age, negative family history for strabismus, bilateral optic atrophy, and absence of light perception in the deviating eye. Adult-onset cyclic esotropia in a blind eye is reported. This afferent defect may lead to secondary changes in the central nervous system, and fusional potential is not necessary for cyclic esotropia to occur.

Adult-Onset Cyclic Esotropia and Optic Atrophy Ronald E. P. f"rl'nkcL 1\\.\)., l\Iil'h,ll'\ C. Brodsky, I\1.D., and ThlH11,lS C. Spl)lH, \\.\)., 1.\.l.S. \ ,;--ll',H-l,ld 111,111 dl'l l'1,'p,'d pl'nll,hc l'~,'ln'f'I,l 1I'llh -i,d,11 clcil" f it' II ,1' Ul1lqUl' bl',',lU'l' "I h" ,1gl'. l1l'g,l­IlIl' 1,1111111 hl,I,'rl I,'l' ,lr,lbl~I11U~. bil,lkr,ll llplic ,11­r,'~' hl ,111,1 ,1["l'I1Cl' III lighl pl'rclTll"11 III 11ll' dl'I'1<1ll1lg l'll' ,\dulh'l1'l'l ,'Iclic l"l'lrllpl,l 111 ,1 blll1d l'I'l' I, rl'­~',' rkd fhl' ,ltkrl'111 dl'll'd 111,11 Il',ld III '<'<'llnd,HI' eh,ll1gl" III till' 'l'l1lr,lll1lTII'U~ ~1'~kl11. ,111d IU"""I1,ll pll­knll, lll' Ill'l I1l'Cl'--,HI t\lr Cl'cl1l' l,~,'tn'pl,l III ,'ccur, hum Ihl' Dl'p.ul n1l' 11 I "I ()f'hlh,illlllll"hl'. "n"hl' 11'1' 111,11' lull' of Wal'nl' St,l!l' LIlI\l·r"tl'. Ikln,,!. \111 hlh"" Addrl'''' (orrl"pund"I1'" dnd n'prllli rl'qlll"h lu Dr I l Spour al Dl'p.utml'nt uf (lphth,ilrlltd"hl', "n"h" II'\' In,llllItl' of Wal'ne Slate Unll'l'r'lll', l'Jl/4 I"hll I'''dd. 11,'lr"l!. \ll,HCIII, USA :!7 Cvclic <.,trabi<.,mu<., i<., a rarl' di<.,prdl'r pi pcular mp­tilitv pi unknpwn l'liologv; it j<., rl'porkd tll pccur in 1 in 3,()(}(1-::;,()()() ca<.,e<., pi <.,trabismu<., (1.2) Cvclic strabismu<" i<., u<.,ualh' an acquired cpnditilm, com­mencing at agl' 3-4 Vl'ar<., and charackrized bv 24-h periods oi prthpphoria or micrp<.,trabi<.,mu.., al­lern, lting with 24-h period.., lli a largl·-angle de\'ia­tiun, usualI' esutropia (3). On orthophpric davs, the patient has iusiun with amplitudes and mal' have an esuphuria. Thi<., diiierentiates c\'Clic esu­tropia irom usual intermittent esutrllpia in that the tropic pha<"l' i.., unrelall'd to the inkrruptiun ui iusiun and is nllt bnllight un b\' \'i..,ual acti\'it\', ac­cUmn1l1d, 1 tilln, llr i,l tigul'. It m,l\' alsu be diiieren­tiakd irom c\'dic oculun1l1tur par'lh'sis in which there are briei. in\'lliuntar\, sp,lstic cuntractil1l1S lli the l'\.tr'1l1cular muscles ..,upplied b\' the third ner\'e, tugl'lher with millsis and Cl1l1tractilm lli the ciliar\' bud\' (4) C\'clic strabismus is usualh' acquired in child­huud. The patient describl'd herein is unique in that Ill' 11',h I1lUdl ulder than patients prl'\'illusl\' repllrtl'd, h,ld bil,lll'r,llllptic 'ltrophl'. and ,lbsence pi light pl'rH'ptipn in the dl'\i,lting l'H' CASE REPORT A 37-\'e,H-llld 111,111 cllmpl,lil1l'd ui c\'clic eSll­trupi, l ui the right l'\'l' lli 3 \'ears' dur,ltiun that ,11­tl'rn, ltl'd l'\l'n' 41" h, IIi.., l'\'l' wlluld be llrtl1l1phllric luI' 2 d,1\'<" (Fig. I) ,md then l'Slltropic iur 2 l1,1\'s (Fig. 2), Wlll'n l'Slltnlpic, Ill' h,ld a hl',ld,lChe and Clll1lpl'linl'd lli blurrl'd \'isilln in till' left eve. He gr'ldu'llh' Illst \'isilln in his right l'W, beginning 9 Yl'ars pn'\'illuslv ,1I1d progrl'ssing tu nu light per­cl'ptilln 4 \'l'ars prl'\'illUsh', Ill' h,ld a past histmy lli ,lkpl1l11 ,lbusl' but stuppl'd drinking 4 years agu. Ill- Ull1lpl'lilll'd ui iatigm', slight ata\.ia, and llcca­siun, ll part'stlll'sias in buth hands upon awakening. R, L. fl, fRtNf\U fJ AL FIG, 1, Patient on an orthophoric day Tht're \\"lS nll hi"tun' uf preYit1u" "trabi"mu" (lr du"t'd ht'ad tr,lum,l, There \\',1" nu famih' hl"tun' llt "trabi"mu", Ocular e\aminatiun rt'\'ealed \'i"ual dcuity of III1 light perceptitln OD and 202:;-2 OS, C\'Cluplegic rt'lractiun \\'a" a" tullll\\''': 00. - 1,7:; ~ Illll . :;ll', ,llld as, - 0,7:; + 1.00 . 1-111, Tht're \\',1'> Jl(l differeJlce in hi" \'i"ual acuit\' ur rt'tr,lctlllJl OJl orthllphlHic and e"lltrtlpic d,l\''' , Hi" left l'\'t' h,ld an amplitude uf accummodation uf 0,2:; dillp!L'r'>, a near point of accommodation (l\:PA) llf I:; cm and a near point of convergence (NPC) l1f Y cm The right pupil was 4,5 mm and amaurtltic The left pupil was 4,5 mm and was 3 + reactin' tu light. Light-near dissociation \\'a" present OS E\­traocular motilit\' examination re\'ealed a cumitant right esotropia of 35 prism diopters, The dulliulb and versions were full, The visual fields shtl\\'ed an arcuate scotoma as, Slit lamp e\aminatil)ll \\'as \\'ithin normal limits and intr'lllcular pressurt'" b\' applanation tonometry \\'ere Ymm 00 and 13 mm as, The fundus e\amination re\'ealed cup-tu-disc ratios of 0,8 00 and 0,9 as, \\'ith pale rim" OL' (Fig, 3), Neurologic examinatil)ll re\'ealt'd llnh' ,1 mild peripheral neuropath\" ' The evaluation included tht' follll\\'ing, tht' rt" suits of which were all normal: lllmpult'd tllllHlg-raph\' ot the brain, orbit. and optic canals; skull \-ra\' film", optic canal tomograms; VORL; fluores­cent trt'Punemal antibody absorption test (FTA­ABS). platelet aggregation; C3 test; sedimentation rate; and electroretinogram (ERG), The cerebro­spinal fluid glucose le\'~1 \\'as 72 mg 100 ml. and the cerebrtlspinal fluid protein le\'el \\'as 58 mg 100 ml (normal 15--1:; mg 100 mIl The spinal fluid \\'as other\\'i"e cumplelt'h' normal. including anti­m\' e!in basic pwtelll le\'els, :\ll etiology for bilat­erall1ptic atrtlph\' \\'as found The patient ,taled that hi" right eye \\'lluld "tr,lighten e\en -1S h, Thi5 \\'a5 \erified objecti\eh' un "t'\'t'ralt)cc,lS1l1ll", His appearance on an ortho­phllric da\' 15 depicted in Figure I, Figure 2 depicts hi" appearance l)ll an e50tropic da\', PupillarY re­actil) llS did Illlt differ l)n orthophoric and esotropic daY" (Figs, 1 and 21 DISCUSSION C\'Clic stral'isl1lus is u5ualh' ,lll acquired condi­tllln in children, \\'lth the l1lbt't at 3--1 nO'ars of age (31 Our ca5e is unique becaust' the patient \\'as an adult \\'ithl)ut pre\'il)US eye surgery, had bilateral l1ptiC atrtlphY, \\'ith nl) light pt'rceptil1n in the de­Yiating e\'e, and had nll family historY of stra­bisl1lll", Ht''''estl1n (3\ repl1rted' the ca5~ of a 32- FIG. 2. Patient on an esotropic day, L'H'IK ISl )ll':'ll/'/\\\I) l >I' lit ,\ lI\l J/'I f) FIG. 3. OptiC atrophy In right (top) and left (bottom) eye \'ear-old patient with altt:rnate e..,utropia; huw­e\' er, no ca"e hi..,tuT\' wa.., gl\'t'n. Truu..,t ,lnd co-worker" ('i) repurkd the C,l"'l' ul ,1 h2-Yl'M-uld woman whu develuped a cyclic L'..,utropi,l I \'l',U following repair uf a "puntallL'uu" rdinal ddach­ment. Parlato and uthL'r.., (h) dL'"cribL'd ,1 13-yeM­old boy \vith cvclic e"otropia that became wn"t,lIlt within a relatively "hurt periud uf time. Wind"ur and Berg (7) reported a ca..,L' of cvclic nuncumi­tant hypertrupia and l'xlltrupid dl'\'l'lupi ng in ,1 1(1­year- old who had "u"tdilll'd tr,lLImd in till' regiun of the left truchlL',l. Cyclic strabi"mu" W,l" fir..,t IllL'ntiulll'd in )lJc;K (8) and then de..,cribed in yuung children by eu,,­tenbader and Mou"el (I) in 1%4, Since then, dp­proximately 50 Gl"e" have been repurkd, Till'sl' reports indicate that the devidtiun is ,1Imo"t dl\v,lYS convergent and noncomitant, demonstrating fu­sional abilities on alternatL' days. Cvcks lwtwl'l'n I and 5 days have also been reportL'~i (1,7, lJ), M,lIlv pdlienh dl..,u h,lVl' congl'nit,ll strabislllus, There is IHI rl'purkd Sl'\.Udl prl'dikrtiun or relationship to rl'lrddi\'l' l'rnlr or ViSUdl acuity, Il'llust dnd ulhl'r'" (C;) have po"tUI,ltL'd that the ,ldull lurlll ul dCl\uirl'd l'"utrupi,l i.., pdrtially due to "l'l'lllldMY n'ntrdl repn lll'""ing dnd dLLlptatiun to a periplll'l'di ,1IIl'l'l'nl dl'll'rt ("Udl ,1" ,1 rl'linal de­l, ldlllll'nt). TIlL'ir pdtil'nt initially h<1d l'\.cdlent vi­" u,ll ,1l'uitv in buth l'Yl'" ,md "uflt'red a "ponta­Ill'OUS rdindl ddachnlL'nt in lhe right l'YL'. One Yl',U Idtn "Ill' dl'vl'!uped l'velid ,lml pupillMv ab­norlll< 1litil''', followed ,1 VL'M IdtL'r bv dn alternatL'­d, lV cvclic l'"otrupia. l'erh,lp" till' precipitating f,1l'tor was the "',lmL' ,1S in our p,ltient-an ,lfferent dl'lect-and thus the unknuwn diulugv uf the uptic ,1trophv is not itself prilll<1rily re"'pon"ible fur the cvclic L'sotropia. If tlwrL' is a cummun mecha­nism bL'lwL'L'n our case and that of Troost et .11. ('i), it certainly does not lead to the ..,alllL' periodicit\'. The rea..,on fur this periodicity is quite "'pecula­tive, It is t\'pical for all patients with cvelic stra­bislllus to develop a con..,tant L'sotropia if fullowed for a sufficient iL'ngth uf tillle. Thi.., may indicate that these patients are basicalh' strabismic with cvcles uf remission rather than orthophoric with periods of esotrupia (3). It ma\' be the result ot an aberration in the biologic clock (1,2) or ,1 wmbina­tion of defects in the clock, the oculunwtur nuclei, superior wlliculi, or other nuclei ,1" ..,ugge..,ted by Richter (2). Roper Hall and Y,lpp (W) pruposed that the mechani..,m is a manifestatiun ot inClml­plete cerebral dominance, with rivaln' between the cerebral hemispherL'''', Reimann (II) reported more than 200 diseases that haH' perilldic recur­rences. C\'c1ic ..,trabismus differ.., from llther r1wthmic di..,ease states in th,lt end-org,ln manipu­lation unitormly elimin,ltL'.., the prublt'm, RichtL'r ..,t<1tL'd that "these opt'r,ltions probably lu..,t rt'ml)Yt' the 'hand' of till' clock, ,lnd dl) nl)t ,let on the clock ihelt-tlll' clock must ..,till bt' running" (2), Windsur and Berg (7) proposed th,lt the cyclic l)C­currence m,1\' gin' tlw p,ltients ,m t'\.cellent fusion potL'ntiaI. !'v1o..,t patients fuse aftt'r surgery (12), ,1nd surgL'n' dllt's not produce an l1\'erCorrectiLln un the Illlllstr,lbi..,mic d,1\'. Hl1\\'l'\'l'r, "'t'Yl'ral chil­dren h,1\'l' de\'l'loped tilt' condition ,1flL'r stra­bislllus surgl'ry (13,14). The unly utlwr C,l"l' rl'port of ,1 cyclic l)culomotor probll'lll ,1"suci,1 tL'd wi th l)ptic a truph \' W,lS made bv Le\'\' (I'») ,lbout ,1 14-\'l',lr-old boy with cvclic ocululllotor p,lrah'sis. He had onset llf intermittent Idt OCUll)mutor p,lr,lh'..,is limited to the iris and cil­iMv budv IllUSCUI,ltUrt' ,1t ,1gL' :; months clnd at age 7 dl'veloped ,ltruphy of the Idt disc. No etiology for the optic ,1truphv was established. The cerebru- I ClIIJ NClIrtHI/11/tI/Il/lIIl1/. Vol, b, No.1, 1986 f' f/'fNf\.U fl Af. ]i..Ih .... F' ~h2 I)",rI1lIhl11' ,In,i cil"'''''' 1,\\1 '\ 183: '" "j" 'II I Ij ,'nci II url"I, P D CleliC \,1;'''' 11!',:;t.lU;'I'!"/ 15: )]."2h, 11..1S' ,lI,,1 Ikr~, t F C,rc,,,i'.\I) hdefl)lr,)!',a ( .. 'I<'nl1,,,l.-r 1- I J, ,)/,,1 \l"t"",I_ D " C\,cllC ",,,twpid, -\I(f: f 1:'i/tI:,i/P:,'! 71: IHIJ, Illh..J .. "II J1tt'f ( /' (1IIlJ.-lllt,dl.lnl..,rn ... t""lItrt 1pl,1 In chtldren. ,11· I<'rll,lI,'-d", "IUlilt /"ill!' /I"I'!."" ,11.-.1 I 122: 2L~, l'Jhil, 11,,1\ \· .... II'n I \1 l \', III .... trdbl ... ll11h '\m l)Jfhopllt I 23: IS I'/~' Bu""n II \1 "nJ \,,,n ,\Ikn, \1 II' (1','I,c "(ul"m,,I,)r f1drclh· .... r.... \II! I (J;//u!,t,/t'!,!.,j 55: =)2\..J, Il/h~ [f('ll ..·d, B '\l'cl. I ,'\.'\lft'IL1, J {'Jnd C,l..'n(I\'t'Sl', F. t\.1 /\~qtl]n.'d t,\', III ("'dltrll~'I,' In dn ddu!t .. \1.'1 I t)J 'hth,llll1(ll 91: , 1'/'< J h [',lfl,lt( I, ( .... lr,lbJ .... niu .... ~ \\ Ind'ilr, ( ,"':' bllrl~ln II .\1 [,"und (dl,] ... · dl ..... lu .......... lIln In 5trilh-dJlll':' ":,;::,,,,',,;; ':,"",,,.;','" iI I H .-\Ile-n, I J C \' \I'''b\ _5t 11Il/1 ..... Iq;.... f' --l:....... lj t .,.....knl...dlkr I [I .Hhi l ) ' .... l·dl. I ~ l \ 1I1( .... trab, ..... mu..::.. REFERENCES (ill.. t.· .....,ltr\I}... I,1 ; i\·';!li~' i..lf":tl:.;!ll~,,: 1-1: 2-'" lQ-~ l~ \lu","", r, " ....,Hltll"", ~ In,i f)unl,'f' E .-\ C"I" ... ·,·,,'tr'l~""'.) t..1 ... ,\ l'III~'Jn~ .1!h::- ..... :r<ll,l ..... nHI-.. .... tlr~l'n· "1'~ '! l-l:,j .... :.: .... : '. 4-l: --l;U 1l.J~ro I" L,'" \1 1\ l\ ,:,,' ,1,"1;"])"':," f','Ll!\ 'I' ,) Ith 'Ttll Jt-fl'f" IH .. ,1)' '-..'!: .::':.;,'''. 03: -["1(""'1 \Yto:"- .-{ ": i I, :::,,~ :!tl: .... - ] '-;- r'l 1'\ L"IIHlr;, 1 T,m::" \1 ,H"i L,n,,".. In i... ''':~' 1I!!'·";'l; \ 'l'i:';:,ii/':",',,·;!{ f-{,IIl:I!lJi': -:';1 )(1'1,'111"1 /)'fkc­! i,f," I l, B"II ..", F,j II lilian" ~~ II iI"III", B,dllm"r,' Ilj~~ f'" 4~2 I,' I, .. f" r I!.,[ I \ 1 '-.l!LJlnt In"- \1\ '-" t"'1 \ '"'I j I'lll" II R"I111,)flII H '\ ,-\-I..j ]L4(1.; olhl'r.., (::;) thaI central n:procl's~ing occurs, re­.., ulting in Ihl' inabililv to (onlrol a previouslv la­knt d,'\'iatil)n, ~I'III," Illllei \1',1' ",~"III],"1I IIIIlll1,Ji In "lIr f'dlll'nl, \I',' c',111 ,,1111' ,1~~IIIIl\' 111,11 Ih,' "I)llc' ,1lr,,!)hv 1)1'('­ll,. k.i till" I', II, 1,lll'III"I1"11,1 I ,'I I' (I;) W,l~ 1IIlelbl" tl' "\1,1,1111 1,,,111 lilt' 'lc'llc 1",>1,1,'111 ,llld till' "f'tlC ,I I Il '1,111 I\"th \\'illll"IIr ,111L1 1"'1,1.:, (7) ,ll1d ",Hlell" ,Ind ,)llll'l" (ll) "lIp~,'~1 Ih,ll il1 cnll" ~lr,lbl"Jllll" Ilw ,,1r.ll'I"IlIU~ I~ 111"[',1"" /'r"bklll, 1\'llh "I"d,-Ill'" h,I' 1l1~lll IU~lcll1 ~llf)"1I111I"h,'d I h,'lr b"II""',11'\' b,l"nJ }'1'I1ll.1rih' "11 till' h,,,,d "III'hll'ell n'''f)''11~'' wilh"lIl ,'\','rt")!T,'dlllll ,llld "b",'1'\ ,1111(1)'-, Ih,11 Illl)llll','r,l!l'd d"\'I,III"Il" ulllll1,ltl'h' bn"1III " 1(111"1,1111 (11ll' ,1(111-. 1\ll1d~ d,'\l'I1) Ihl" 1'(I"tlll,l!l' \\'"uld Iii Illir t',l",' .h 1\',' II lIw rL'lln,ll lil'l,llhnll'nl l,I"l' "I [r",I"t vt.1I (;, •lnd clur t'.1:--1' h,ld till' ,1n"I't "l Ih,' l'l't'lll' v",'tnlf'l,l illlt'r 1":--,, "I \'I"II1n In tlw d"\'I,Itlll,~ V\'" II11' /',1­tl,' nl ,,' Tn),)~l ,lnd (\Ih,'r:-- (')) r,'co\"')',,d \'i~iOI1, "UI' p,ltl"l1t did ntll. Sub~,'qu"l1th'. if th,' ,ltkl'('nt \1­~ u,ll dl'f"d I" till' "\t'itlll,~ l'\','nl ,lI tlw ,-\'t'lll ,'",1­tropl'l, then tlw d"\'iilli(\11 Illil\' rv 111 ,1 I 11, \\Ith ,II' withtlut ,ln ,literl'nt dl'il'(l Fu<;i"I1,ll 1'(lll'nll,ll I" not lleCt'~~arl' for tilt' C\,t'IIt' l'h,'nol1l"lllll1 10 ''It'ur We b"lie\',' th,lt till' ,llln,'nt \hU,ll dded \\'cl" the III I t Iatin hI'" 1'1 I' h to' I' ,11 V\'Ie' 11 t, k ,1 d i 11,~ Ill"" t'­() n d aI' v (,' n t I' aI n erv () u s <; \' SIt' ITl ( h ,1 ll,~ ,'" II1 t Ill' ()eultl1l10t(lr C(lI1tn,l SI'<;lL'1ll a~ p(\QulcllL'd b\' Irllo"t and lIther,;; (::;), 1111'11' patiellt lllc1intain"d her t'\lli( t',;;otwpia illlL'r rehc1ining \'i~I,'n, and (lUI' p,lllt'11I mall1tained his (vclic de\'iation in the blind C'I',' Thi<; supports till' p,,~tlll,lk 01 other" (h,:-) that till' strabismus i~ the ba~i( ~'roblem in tlw cI'(he e:--,'­tropia, and ';;lI~'porb til<:' po,;;lulak 01 Tr')lht ,md