| OCR Text |
Show Isolated Trochlear Nerve Palsy Secondary to a Juvenile Pilocytic Astrocytoma GREGORY B. KROHEL, M.D. AHMAD M. MANSOUR, M.D. WILLIAM l. PETERSEN, Ph.D., M.D. BRUCE EVENCHIK, M.D. Abstract A9-yeu-old female developed a fourth nerve palsy which was the first sign of a posterior fossa astrocytoma. The fourth nerve palsy was believed to have resulted from direct invasion of the trochlear nerve rather than compression by hydrocephalus. Sagittal reconstruction of a computed tomogram revealed extension of the tumor into the dorsal midbrain where the trochlear nerve exits. Introduction Trochlear nerve palsies are rarely associated with intracranial tumors. We recently encountered a patient with an isolated trochlear nerve palsy secondary to direct invasion of a juvenile pilocytic astrocytoma extending from the cerebellum. Case Report A 9-year-old white female was admitted to the Albany Medical Center Hospital on March 6, 1981. for investigation of bilateral papilledemd. History revealed that several months prior to admission, she had developed a noticeable right head tilt documented by photographs. She also complained of diplopia on right gaze. Neither the head tilt nor the diplopia were brought to the attention of a physician prior to admission. Two weeks before admission she first noted neck pain which rddiated to both arms. Subsequently, she complained of intermittent tinnitus and generalized mdlaise. Over the weeks prior to admission, the family became alarmed by a rapid personality chdnge, lethargy, anorexia, and decreased intellectual performance. From the Departm('nt of Ophth~lm"I,,):y.Alb.,ny Ml'dll.,1 ("I· lege, Albany. New York. June 1982 Morning frontal headache and vomiting supervened dnd she was brought to medical attention. There was no history of trauma. Physical examination revealed an appropriately developed, well oriented, normocephalic 9-yearold female with a conspicuous right head tilt. The neurologic examination revealed normal sensory, motor, and cerebellar function with normal deep tendon reflexes, Babinski responses, and gait. The cranial nerves were intact with the exception of bilateral papilledema and a left trochlear nerve palsy. Ophthalmic examination revealed a visual acuity of 20/20 in each eye. External examination and pupillary reactions were normal. Measurement of ocular motility disclosed a mild left hypertropia which increased in right gaze and on left head tilt (Fig. 1). Visual fields were full except for enlargement and extorsion of the blind spot bilaterally. Funduscopy revealed advanced papilledema without atrophy in both eyes (Fig. 2). Radiologic examination of the skull revealed demineralization of the dorsum sellae along with didstasis of the coronal sutures. Computed Jxidl tomogrdphy (Fig. 3) showed dn enhancing ringshdped lesion extending from the superior vermis to the dorsal dspect of the midbr.lin. ModerJte enldTgement of the l,lIeral dnd third ventricles resulted from aqueductal stenosis produced by the lesion. Cerebral angiograms were noncontributory. The pdtient underwent d ventriculoperitoneal shunt dnd did well for severdl hours postopera- (~ l )"1,,, 5.1 LHT (lI~htl (Idll Figure J. C~rdin,,1 fil'lds m('~surl'm('nl ~nd h('ad till Ipst. (LHT-left hyperlropi, l, ET-('st>tropi~, 6-diopter) 119 figures 3d and Jb. t,l) Mldlme .lg.ltI.ll rC'...·\.'n~lnjl·tll)n ~hl)\\':' m.dllllC' l VS,th.. I(·~l\.m with thl~J... l~nh.,n\.·I"~ "'.111 The lesh..)n, 2.5 em In dl~m('l("r, 1"- w(',i~("d hl~lw(,,(,1l tht, plne'.,1 r('~llln. ~u.lJri~('mln,J] pl.lll~ .lnJ tht.~ "upt.'r1\.)r \'t.·mll~ :"l~tt." th(' ~r(".)tly ~n[.}r~()d trllrd v(I'otn(]C' ,.~bl,lvC' the 1("'01\111, .lud Ih' hlllrih n~ntndl' b,'ll'\\' II Ie-I (n.l .... .. l.~\,th)n .it tn.... 1..'\'11",1 \." Ih\~ 1t.~nl\'rl.J1 hl.ltU::- :.h 'wIng, the l'nh.lI\(II~~ 1('..lLln ht.'tWC''l'1l Ihl' "'"up('fl...1r ,",,'rm ....Uld Ih(' li,~r...um \'. Irh' nHdbr,HfI lively, but thcn bec,11lle rro~rl'ssiV('ly ll'lh.Hgil A IMgt' den,ity th'll'ght t,l r,'pre·,C'nt l'I'IOJ IV.IS ,letectl'd in Ih{' rf'gi,'n "f 111<"' uprt'r ct'rebdl.H 111.lSS tIn <"<un pUled .1 i.ll ttlnlll~r,'rhy, TIl<' !,.Hi,'nl 1V,1, 1.11<.('11 to ,urgl'ry _'g.1II1, I'rt'ci~l' Stlf~Il·.ll 1,1(.l111.,1t1l111 "I' the ~un1tlr W.l' lI11p",,,ble JUt' I" tilt' prC'sence "f ,1 I.ug(' ht'Ill.1~"m.l. but the .Ht',l il1v"lved gt'l1er, Illy 1l.nf"rnwJ ttl lilt' I"Collil'" "I the 11Im"r 'l','n "n l"mplll'·r17.,'J ,n.I,lllt'l1hlgr.lphv Afll'r Jr.lII,.ll;:t' of Ihe hl'nl,ltllll1,l .>nd fl'rllp\',,1 lIt lilt' tumor. till' p,llil'llt Il.lJ ,I ~I(lW pn'grl'~,ivt' H'llirn I,f ll('lIftll,'gic ft"'1 1"'11. ~Ill' h,.d rt'~(livill~ ~iglls "f .111 upper n,idl>r.,il1 ~vl1dr'''lll' indlldil1g bil.,tl'r.,1 blepll.lr1' 1'1.1.... 1..... I .,Il\'I'I~I·IH I' 11l'-oUttil H'rh:V. ~'rld l'v~t..l~n'lIs retr,lCIl'rius p, stl'per.Hively, Ihe patient had evidel1ce l,f bit.Her.,1 trl'chle.lr nerve palsies. more pr"I1,'unced l'n the left. Ex.lmin.ltion of the p.ltho1,' gic.,1 secti,'ns vielded ., di.lgnosis of juvenile ril,'cytic "strl'cytl m,l (Fig, "). Discussion This ,.,st' is unusu.ll in that the symptoms and SIl;:llS l,f .m isolJted trochlear nerve palsy constilut(' d thE' first manifest,ltions of an evolving intracr. ll1i,1j ,lstrocytoma. The trochlear nerve palsy in thi. ,I was thought to represent direct involvement \'f the nerve by tumor. This direct invasion Journal of Clinic.ll Neufo-ophthalmology (b) Figur~ J l.."nfmucd) without other central nervous system involvement is exceedingly rare, and has not previously been reported with gliomas although pine.llom.ls, I." medulloblastom.ls,"· and metastatic tumors" h.lve presented rarely in this fashion. Thp trochlear nerve is the oculomotor nerve leilst often .lffected by tumors. In il study of 3.033 intr,lcranial tumors associated with extraoculilr muscll' palsies, the trochlear nerve was involved in only 1.3%.~ less lhiln 3% of the trochle.lr nerve pillsic reported in spverallarge series have been attributpd to the intracranial neoplasms:" '" '-I!, Mosltrochlear nerve palsies are secondilry 10 hp"d traum". VilScular disease, or congenital factors. Tumors associated with trochlcilr n(>rve pals;p include brain stem gliomds, 1 , m(>duliobl.1stom.1,,·I. , and meningiomas of the posterior foss.1, pine.llo. mas, I. ~ pituitary ademonas, ilnd mel" tic prPJd from tumors of the n.lsopholrynx. brCilsl, and ovary.'" 14 Gliomas of the br.lin stem and cerebellum account for one-quartpr of "II childhood intr.l· cranial neoplasms, with two-thirds locdtl'd in the cerebellum. and one-third in the brain stem. Diplopia has been encountered in Ipss Ih"n half of these cases and an isolated trochlear n('rve p<llsy has not been reported with th('se gliomil liom.1s June 1982 of the cerebellum <lnd brain stem are r<!rely malignant in children, but Me often lethill due 10 their loc<ltions. Glioblilstom<l multi forme il(Counts for 2% of the gliom<ls in this region. Pilpilledema in dssociation with trochlear flillsy h.ls been reported in prim.1ry hydrocephillus. ,,, leold poisoning. pseudolumor c{'rebri . .lnd with intr,1Cr,lni, ll tumors. In th('se <:,151'5. Ir,,<:hll'.Jr p,llsy .1nd the result,int ocul,H t,'rtic"lis (he.ld tilt) ,1l1d dipl,'pi,l were preceded by inv<llvement "f "tlll'r cr,lni,ll nerves. or by sympt"l11s "f inae.ls('d intr,la.wi,ll pressure. The trochle,H nerve p"lsy in the present <:.1se is felt 10 b(' s('cond,lry t" direct inv<,lvl'menl of thl' nervI' by th(' ".ln1cyh1l)l.L Th,' tn'ehle.'r nl'rvl' IS sIll"II, fll'-..:ibl(' .•1I1d pr,'tt'rtl'd within thl' thie"- tenlori, ll bN! f,'r nllls1 "f its c""rsc. Tuml,lrs molV indir('ctly pwdul'l' tn'ehkJr nnvC' p.,bies by in'. ducing hydr,'cl'ph.,llls with sewnd,lry c<'mpression of th(' nC'rvC'. Thl'rl'fNC'. il1dir(',-lly induced trochleM p.llsi('s .He IIsu.llly prC'<'l'ded· r ,lCcomp.lnied by other symploms of cl'nlr.11 nerv,lus system dispasC'o In the presenl C.1se. there were no othl'r symptoms when the he,ld tilt ,md diplopiil were first not('d, ,lnd none "ppe,Hed for sever,,1 months thC'r(',lfter. This supports the impression that the 121 t ~, (~I J i~ur(' 4.1.1) 1','lhnlp)',ll ,11".'1111011" \1111.,- 1'·... I\It\ J'·tlhH\..'r.'h·... I.H~t' unip",'l.tr Jnd biplllJrceUs. tH&E stdin. reduced from X210.) (b) Ilr. 1111111'1 \\.'.,. 11I1'hl,' \'.1' .• III.H \'\'1111 ,I .. In.n~ .,tlIlHlv h.lr n\·un,~h.,1 ..t.Hns, (Ar~("ntJffin stdin. reduce-d from X200.) Journal of Clinical Neuro-ophthalmology / trochleclr nerve WclS primarily involvl?d .1Ild tl1.l1 th(' hydrocephcllus evolved !.lter .\S thl? tumor gn·w. Furthermore, computerized ll)mogr.\phic.ll irn.lging showed the tumor inv.\ding the postl?rior .1SPl?ct of the midbrain where the fourth nl?rve I? it~ fwm thl? brclin stem. References I. Ruc"er. C. Dvt'r. I.. Smith. n..1I1d T.lUb. R.: Tlw (,lUSeS "f .K'l~ir('d p.lr.lly~i~ ,,( thr ,'nd.lr mu~,I,·s. Am. /. Ophth.lInll'l. -II: ()51-()55. 1%,). , RUl·"er. C.W.: P.lr.lly~is"f tl)(' third. f,'urth .1nd sixth cr.mi.ll n{'["es. Am. I. l)phth.l/nll'l. -10: 7/17-7\14. l()S/I. ~. Ruc"er. C.W.: The '.lU~(,S "f p.lr.llysis ,,( the thiro. fourth .mo ~h.th (r.mi.11 n('rve~. Am. I. Opht/l.J/nl<ll. 6J: 120~-12()1I. I()e-t>. 4. Kh.lw.Jm. E.. SWll. A...1110 '.lmp"lsky, A.: Acquired superi,'r oblique p.llsy: DiJ~n,'sis .Ind m.1I1.Jgement, Arch. OphthJ/nwl. 77: 701-7t>8. (()t>7. S. Cr,'ne. A...1nd Leurio.m. 0.: Les p.lr.Jlysies bil.Jter. lles des ~rJnos ,'bliques. 8ull. Mem. S,'e. Fr. Ophthalmol. 83: -10 0-500. 10 70. o. Burger. L.J .. K.llvin. .H...md Smith. I.L.: Acquired lesions of the fourth crJni.J1 nerve. 8rJ;n 93: 5t>7-574, 1970. 7. Gobin. M.H.: The diagnosis dnd treatment of fourth cranial nerve paralysis. Ophthalmologic.l 173: 292-2°5, 10 70. B. Millieman. D.. ,md Folk. E.: The evaluation and treatment of superior oblique muscle palsy. TrJns. Am. Acad. Ophthd/mo/. Otoldryngol. 81: 893-80 B. 1976. 9. Younge, B.R.. and Sutula. F.: An"lysis of lrochle.1f nerve palsies: Diagnosis. etiology and treatment. Mayo Clin. Proc. 52: 11-18.1977. 10. Wright, H.G.. and Hansotia. P.: Isolated fourth cranial nerve palsies: Etiology and prognosis. Wis. Med. f. 76: 26-28, 1977. 11. Copetto. J.M.. and LesseL 5.: Cryptogenic unil"teral paralysis of the superior oblique muscle. Arch. Oph. thalmol. 96: 275-277, 1978. 12. Neetens, A., and Jansens. M.: The superior oblique: A challenging extra-ocular muscle. Doc. Ophth.1/. mol. 46,2: 295-303, 1979. 13. Harley. RD.: Paralytic strabismus in children. Etiulogic incidence and management of the third. fourth, and sixth nerve palsies. Ophthd/mology 87: 24-43, 1980. 14. Nemet, P.• et .II.: Benign palsy of the superior oblique muscle. j. Pediatr. Ophthalmol. Str.1b;smus 17: 320-322, 1980. June 1982 Krolwl, M.lJ)~llur. Petersen, Evenchik 15. Rush. I...Ind Yllun~r. B.: r.H.Jly~is of crani,,1 nerve~ III, IV, Jno VI: C.IU~e .lnd pro~no~is in 1,000 c.1ses. Anlt. Ophth.llmo/. 99: 7t>-79, (()81. Other References It>. Robert. CM.. Fl'i).;enb.lum. J.A .. Jnd Stern, W.E.: 0, ubr p.ll~y Oll urrin).; with pituit,lry tumor~. f. Neurosur~. 38: 17-1 9 • 10 73, 17. Wr.lY. S.H.: N(·uro,"pth.,lm"I"~i .11 manif('~tali{)n of pituit.lry .lIld p.If.l'{'IIJr I('.. illn~. ('lin. NC'UHl.'urX. 24: ~0-1(7. 1077. III. W,llkrr. A.E., .Ino Hllppl('. T.L.: Br,lIn tumor.. in children. f. red;.ltr. 35: 07-~7. 1049 . 10 . MiIlN. R.H.. Cr.li~. W.M.. ,1nd Kernohan, '.W.: Supr. lt('nt(1ri.l1 tumt'r.. JmonX thddren. Anh. N('uro/. f'svchi,ltry 68: 797-~I<I. 19 52. 20, B,;di.J. M.• .lnd L.lw~on, D.: Th(' IntrdCranial ne,'pl. lstic dise.lses of ,hildhood. Br. j. SurX. 40: 308-392. 105~. 21. Odom. C.L.. Davi . CH.. and Woodhall. B.: Brain tumors in children: C1inicoll olnollysis of 164 Colses. redidtrics 18: 850-809, 19 50. 22. K,ltsura. 5.. Suzuki. I.. olnd Woldd. T: A tatistical study of brain tumor in the neurosurgical clinics in JapJn. /. Neurosurg. 16: 570-580. 1959. 23. Backus. R.E .. and Millichap. J. .: The seizure as 01 manifestation of mtrananidl tumor 111 childhood. Pediatrics 29: 978-9B4. 1902. 24. Gal. A.: erebral astrocytomols 111 childhood: A clinical study. j. Neurosurg. 19: 577-582. 1902. 25. MacKay. E.M.. Jnd Seller. A.H.: M.llign.lnt neo· plilsms of the centr,,1 nervous system. Ontdri cancer foundation clinic (050-04, Can. Med Asso f. 99: 1245-125~, 1908. 20. Gibson, J.L.: Plumbic ocular neuritis in QueenslJnd children. 8r. Med. /. 2(pJrt 2): 14811-140 0. 1()08. 27. Gibson, J.L.: 0 ulJr plumbism in children. 8r. ]. Ophthalmol. 15: 037-042. 1931 2B. Cushing. H.: The intr.lcranial tumors of preadolesencl.'. Am. f. Dis. Child. 33: 551-584. 19 27. 29 , Keith. H.M., Craig. H.M.. and Kernohdn. J.W.: BrJin tumors in children. PediJtr;cs 3: 8~(L8-l4. 1040. Acknowledgment Thi~ p.'p('r \)/.l~ ,urr,'rkd by .111 unn'sl r,,'I,'d ~r.ll1t fr,'m I{(·"'.lr,·h til I'r('\,('nt Hlindn,"s 1'\I"te f,'r r('prints t,,· Cr('~,)ry B. "r"hel. f\ 1.11.. [l,•. p.lrlnll'nt "f ()phth.,II1l"I,,~\'. Alb.II1\' f\ 1<'dk.11 C"lk~,'. Alb.lIlY. Nrw Y,'rl.. I22(l:'. 123 |
