Journal of Neuro-Ophthalmology, June 1985, Volume 5, Issue 2

Association of bilateral internuclear ophthalmoplegia and myelomeningocele with Arnold-Chiari malformation, type II.

We describe the unusual association of long-standing repaired myelomeningocele, hydrocephalus, and Arnold-Chiari malformation with bilateral internuclear ophthalmoplegia in a young man. A rarely reported association, this case emphasizes the need for further neuroradiologic evaluation to determine the possibility of third-ventricle enlargement with worsening hydrocephalus.

,. Oill. N"III'lJ-jl'I'II'IIl'llt",. 5: 12-l - 1211, IYK'i (u 1985 RauclI Prcss, New York Association of Bilateral Internuclear Ophthalmoplegia and Myelomeningocele with Arnold-Chiari Malformation, Type II ROBERT C. WOODY, M.D. JAMES D. REYNOLDS, M.D. Abstract We describe the unusual association of long­standing repaired myelomeningocele, hydroceph­alus, and Arnold-Chiari malformation with bilat­eral internuclear ophthalmoplegia in a young man. A rarely reported association, this case em­phasizes the need for further neuroradiologic eval­uation to determine the possibility of third-ven­tricle enlargement with worsening hydrocephalus. Myelomeningoceles are frequently associated with the Arnold-Chiari malformation. This central nervous system malformation may vary In anatomical and clinical severity. Arnold­Chiari malformation, Type II, the most common form, consists of pontine, medullary, and cer­ebellar ~istortion and protrusion into the spinal cord. It IS nearly always associated with mvelo­meningoceles. I In addition to the other b'rain­stem anomalies, the aqueduct of Sylvius may be obstructed in this malformation, leading to' hv­drocephalus with distention of the lateral and third ventricles. . Bilateral. internuclear ophthalmoplegia is a disorder ot oculomotor function in which con­jugate horizontal gaze is impaired secondary to bra~nstem I~sions involving the medial longi­tudInal faSCiculus. Such bra.instem pathology is frequently seen In adult patients with neuroll)g­! Cal disease. We report the unusual association of a .Iong-standing surgically repaired myt'lo­menIngocele and Arnold-ChicHi malformatil)n, hom Division of Pediatric Nl'uwlogv. Uni\'l'rsitv "f Ar, kansas for Medic<ll Sciences and ArLl;,s,lS Childn·i,·s II"s, pita!, LittlL' Rock, Arkansas. Writ!' (III' r!'prillts til: R. C. Wo"dv, M.D., l1i"ision "f Pl'­diatric Neurology, Dl'pMtml'nl "f 'Pl'di,llrics, Unin'rsilV "f Arkansas for Medica) Scil'nn's, ·1301 Wl'sl Mdrkh,lm SliL'l'l, Llttll' Rock, AR 722llS, USA. 124 Type II, with bilateral internuclear ophthalmo­plegia. Case Report The patient is now a 20-year-old white man. He was born with a large lumbar myelomenin­gocele that was surgically repaired shortly after birth, FoIlO\,ving surgery, the patient demon­strated a flaccid paraparesis, a neurogenic bladder, and an L--l sensory level. At about 15 years of age, he began complaining of mild bi­frontal headaches and his family first noted that at times his eyes "jiggled." He was examined b~' a neurologist who confirmed the diagnosis ot bIlateral Internuclear ophthalmoplegia. No turther evaluation was performed. Over the next 5 ~'ears, his headaches worsened, and he and his famil~' continued to note disconjugate eye movements and nystagmus on horizontal versIOns. On recent examination, the patient's mus­cular de\'elopment in the upper extremities was good, but his lower extremities were tlaccid and atrophic. The head circumference was 59 cm (95'!(). The mental status examination was Ol)rmal. Visual acuit~· was 20/20 in each eye. Funduscop~' revealed mild, chronic disc edema bilaterally, Cl1nfrontational visual field testing was nl1rmal. A motilitv examination revealed that, on lateral gaze, a' clear-cut bilateral inter­nuclear ophthalml1plegia was present, with pa­rests l1t the adducting eye and nystagmoid ml1\'t'ments l~t the abducting eye. Upward gaze was slIghtly hmited and vertical nystagmus was present; downward gaze was also slightly lim­Ited. There was a small A-pattern intermittent eXl)tropla eVlLient, ~easuring 10 prism diopters 111 pnmary, 20 pnsm dlOpters in downward gaze, and 5 prism diopters in upward gaze. Conve~~ence was normal. Pupillary size and reachvny to light and accommodation were normal. Lower cranial nerve function was Journal of Clinical Neuro-ophthalmology normal. The remainder lli the neurologic e:>.­amination coniirmed an L--t St'nsorv ,md nllltor level consistent with tlw surgical iiridings ,lt lhl' time oi 111\'e1l1mt'ningoct'1l' c1llsurl'. ComputeriZt'd tonlllgr,lphic sc,ms of the br,lin revealed massi\"l' dil,lt,ltilln oi till' 1,1 It'r,l I ,lnd third ventridl's with nll l'nl.lrgl'ml'nt of llll' iourth ventricle, suggl'sting ,In ~lbstructi\"t· I1\'­drocephalus ,lt till' It'vl'lllf the ,h]ueduct oi Svl­\' ius (Fig. 1). TIll' iwnt,ll hllrl1s of the I,lll,i',ll ventricles h,ld a Cl1niigur,ltilln tq~iC,ll of I\r­11l1Id- Chi.ui malfllnn,ltilln. TIll' p,ltienl under­went surgt'n' fllr ,1 H'ntricull1pnitollt',11 shunt through thl' right 1.1lt'r,11 H'ntriciL' ,1Ild h'ld ,1Il uneventful pl1stopl'ratiH' ClHlrSl' with gradu'll resolutilln oi till' Ill'adaclws. Aflt'r 2 months, ree,aminatilln rt'\'l',lkd th,lt the intl'rnucle,H llphthalmopkgia \V,lS unch'lngl'd. Computl'r­ized tlll11llgraphil' scans del11llnstratl'd signifi­cant impwvenll'nt in \"l'ntricuIM size (Figs. 2 and 3). Discussion Naidich et al. 2 recentlv re\'ie\Ved the mesen­cephalic neuroanatomy 'and neuroradiology oi the Arnold-Chiari T~'pe II maliormation. The simultaneous occurrence oi the Arnold-Chiari maliormation and bilateral internuclear oph­thalmoplegia has rarel~' been reported. Cogan,:1 listing \'arious causes oi internuclear ophthal- Figure 1. Computerized tomographic scan 01 brain betore ventriculoperitoneal shunt. June 1985 Woody, Reynolds Figure 2. Computerizl'd tL1mographic scan oi brain .liter shunt. moplegia, described two patients with Arnold­Chiari maliormation. One of these patients was an ~-year-old boy. Houtman4 also noted one case. 'Other reviews oi the neuro-ophthalmo­logic findings in Arnllid - Chiari maliormation Figure 3. ComputL'r1/l'd lomllgr,lphic SC,111 oi nwsenceph­. lIon aitl'r shullt. 125 Internuclear Uphtlhllnll)plq~ia in Arnllid-Chi,lri reported no confi rmed in it'rn uclear oph thal­moplegia. ~ 10 Well-described causes of bilateral or unilateral internuckar ophthalmoplegia in­clude multiple sclerosis, infiltrative tumors, neurosyphil is, and ,lrtt'riosclerotic or in fla m­matory vascular diseilse. Occasionally, myas­thenia gravis m,ly have a neum-ophthalmologic finding suggestive of inh.'rnuclear ophthalmo­plegia. All of these disorders are uncommon in the pediatric patient, while the Arnold-Chiari malformation and hydrocephalus are seen quite often. Clinically, internuclear ophthalmoplegia con­sists of a characteristic syndmme of paresis of adduction of one eye and horizontal nystagmus of the contralateral abducting eye on attempted versions to the left or right or both. Conver­gence and light reflexes generally are well pre­served. Upward gaze is associated with vertical nystagmus. Subjective visual complaints by the patient are few, if any, in long-standing cases of internuclear ophthalmoplegia. Internuclear ophthalmoplegia clearly can be localized to lesions of the medial longitudinal fasciculus. This tract is an associative fiber system running throughout the brainstem ven­tromedially to the aqueduct of Sylvius and ex­tending into the cervical spinal cord. The medial longitudinal fasciculus receives input from the vestibular and other proprioceptive systems and coordinates this input with the oculomotor and abducens motor complexes, thus permit­ting conjugate horizontal eye movements. Our patient also demonstrated an A-pattern exotropia. This is a well-recognized condition associated with spina bifida and mvelomenin­gocele. 11 - 13 Its occurrence is probabfy indepen­dent of the diagnostic findings present in inter­nuclear ophthalmoplegia. The case described here points out that bilat­eral internuclear ophthalmoplegia may be a pre­sentmg feature of the Arnold-Chiari malfor­~ ati~n associated with hydrocephalus in pe­dIatriC myelomenmgocele patients. The pathophysiologic cause of internuclear ophthal­moplegia in our patient may be the disruption of ~he medi~1 lo~gitudinal fasciculus owing to brall1stem dIstortion or the presence of priman' embryologic malformations of this tract. The iri- 126 ternuclear ophthalmoplegia, however, may signal compression of the medial longitudinal fasciculus by third-ventricle enlargement, indi­cating worsening hydrocephalus, which would require further neuroradiologic evaluation and surgical therapy. However, in long-standing hydrocephalus such as in this case, shunting may not relieve the internuclear ophthalmo­plegia. References 1. Swaiman, K. F., and Wright, F. S.: The Practice of Ped1l1tric Nellroluxy. C. V. Mosby, SI. Louis, 1982, P 414. 2. Naidich, T. P., Pudlowski, R. M., and Naidich, J. B.: Computed tomographic signs of Chiari II malformation II: Midbrain and cerebellum. Ra­dioloXy134: 391- 398, 1980. 3. Cogan, D. G.: Internuclear ophthalmoplegia, typical and atypical. Arch. Ophthalmol. 84: 583­589, 1970. 4. Houtman, W. A., Meihuizen-DeRegt, N. J., and Rutgers, C. H.: Strabismus and meningomye­locele. Doc Opht/1I1111101 59: 255-261, 1981. 5. Collard, M.. Strubel-Streicher. D., et al.: Oculo­motor disorders associated with Arnold-Chiari malformation. Rt'l, . .'",'lIr"I. (Paris) 136: 531-538, 1980 6. Harcourt, R. B.: Ophthalmic complications of meningom\·elocele and hvdrocephalus in chil­dren. Br. /. Opht/Itlllllol. 52: 670-676, 1968. 7. Clements. D. B., and Kaushal. K.: A study of the ocular complications oi hydrocephalus and men­Ingomyelocele. Trail". Ophthalmol. Soc. U.K. 90: 383-390. 1970. 8. Rothstein. T. B.. Romano. P.. and Shach, D.: Meningonn·elocele-associated ocular abnormal­ities. Trail". Alii. Oplzthalllll11. Sl1(. 71: 289-295, 1973. 9. Goddard, C. K.: Ocular changes in hvdroceph­alus. Br. Orthl1pt/c 1.22: 72-80. 1965.. 10. Z.acharv. R. B.: Ophthalmic problems in spina bi­lIda ,lnd hvdrocephalus. Br. Orthoptic /. 22: 70­71. 1%5. II. H,ucourt, B.: Str,lbismus aiiecting children with multiple h.mdicaps. Br I. Ophthalmol. 58: 272­2~ O. 1974. 12. Fr.mce. T. D.: Strabismus in hvdrocephalus. Am. Ortlzl'pt/( I. 25: 10 1- 105. 1975.' 13. l\lalole\". A. .. Weber. S.. and Smith, D. R.: A and V p.llterns oi str,lbismus in meningomyelocele. :\//1. l)rlhtl/,ti( I. 27: 115-118. 1977. . IlIurn,ll oi Clinical Neuro-ophtha]mo]ogy