sign in sign up
<<
Home , Synkinesis

1026 CLINICAL NEURO-OPHTHALMOLOGY

(greater than 2 msec)and increased amplitude, and they are Microvascular decompression carries the risk of trochlear polyphasic, with a spontaneous discharge rate of approxi- palsy (244,800–802). mately 45 Hz. Spontaneous activity is absent only with large SOM spontaneously resolves in some patients (791); oth- saccades in the ‘‘off’’ (upward)direction and is less affected ers tolerate it and do not need treatment. For those whose by vestibular eye movements. Some firing units show an symptoms are distressing, both medical and surgical thera- irregular discharge following muscle contraction before sub- pies are available. Individual patients may respond to a num- siding to a regular discharge of 35 Hz (792,798,799). Elec- ber of drugs, including carbamazepine, baclofen, gabapen- tromyography findings are similar to those of tin, and topically or systemically administered ␣-adrenergic with hemifacial . The electromyogram suggests that blocking agents (791,803). Those who do not respond the etiology of SOM is neuronal damage and subsequent to drugs may benefit from extraocular muscle regeneration. SOM is only occasionally preceded by an ipsi- (791,792). The procedure used by most surgeons is a supe- lateral palsy, but the possibility remains that rior oblique tenotomy combined with myectomy of the ipsi- mild chronic damage to the trochlear nerve could trigger lateral inferior oblique muscle, but nasal transposition of regeneration (792,798,799). Vascular compression of the the anterior portion of the affected superior oblique tendon, trochlear nerve by the posterior cerebral artery may be etio- thereby weakening cyclorotation, is also performed. The role logic in some cases and may be disclosed by thin-slice MRI. of neurovascular compression has yet to be determined.

SYNKINESIS INVOLVING THE OCULAR MOTOR AND OTHER CRANIAL A synkinesis (‘‘moving together’’)is a simultaneous patients (804). One child had her ptotic lid elevate whenever movement or a coordinated set of movements of muscles she sat up, perhaps from a synkinesis between the levator supplied by different nerves or different branches of the same and the neck muscles (805). One woman experienced diplo- nerve. Normally occurring cranial synkineses are exempli- pia every time she swallowed, because of intorsion and fied by sucking, chewing, conjugate eye movements, and depression of one eye. She appears to have had a synkinesis Bell’s phenomenon. between the superior oblique muscle and one of the muscles Abnormal cranial nerve synkineses occur most commonly of deglutition (806). Some patients with the Marcus Gunn in DRS (discussed earlier)and in the Marcus Gunn jaw- jaw-wink phenomenon have more extensive synkineses, winking phenomenon (trigemino-ocular motor synkinesis) with movements of both the eye and the lid related to jaw (see Chapter 24). Similar synkineses involving various facial movement (807). The Marcus Gunn phenomenon and DRS and neck muscles and the occur in rare may coexist (808).

NEUROPATHIC TOXICOLOGY OF THE OCULAR MOTOR SYSTEM Exogenous agents sometimes cause peripheral ocular case with the development of and limitation of upgaze motor neuropathies. They can also cause supranuclear dam- bilaterally, with and paresthesias. The patient had anti- age, extrapyramidal reactions, neuromuscular blockade, and AChR antibodies. The antibodies and the ophthalmoplegia myopathic damage. disappeared within 10 weeks of discontinuing the drug. The Toxic ocular motor neuropathies are rare. They may be product monographs for three other HMC-CoA reductase confined to a single nerve or involve some or all of the ocular inhibitors (lovastatin, simvastatin, and fluvastatin)list ‘‘im- motor nerves. They may be associated with other signs and pairment of ocular movement’’ as an adverse effect (811). symptoms, either neurologic or systemic. There are no char- A man industrially exposed to carbon tetrachloride, an acteristic features that clearly identify them as toxic; unless organic cleaning solvent, developed bilateral abducens pare- the patient is rechallenged with a drug or poison and devel- ses associated with evidence of systemic toxicity (812). ops the ophthalmoplegia again, diagnosis may have to be Subtenon carboplatin has been use as chemotherapy for tentative. intraocular retinoblastoma. Ten consecutive patients devel- In most cases of toxic ophthalmoplegia, the mechanism oped restricted ocular motility as a result of fibrosis of orbital is unknown. In a few, there is an immunologic attack. Enzy- soft tissues. This does not appear to be specifically neuro- matic processes may be blocked. Some patients have idio- genic. It may represent fibrotic change after fat necrosis pathic reactions because of their own genetic makeup. Some (813). reported cases may be pure coincidence. Chloroquine diphosphate, a quinoline derivative, is some- Before the availability of penicillin, organic arsenicals times used to treat malaria and a number of rheumatic disor- were used extensively to treat syphilis. An ders. A patient who had undergone long-term treatment with was observed in one patient with chronic arsenic chloroquine developed bilateral abducens palsies (814). poisoning (809). Cocaine abuse has been implicated in several cases of A transient palsy developed in a 2.5- myasthenia gravis. While the associated ophthalmoplegia is year-old child who consumed a large amount of aspirin and not caused by a nerve lesion, it is easy to confuse with one. then developed convulsions and coma. We wonder whether One case was associated with pseudotonic pupils (815). A this was a sign of herniation from cerebral (810). midbrain hematoma from smoking crack cocaine caused a Atorvastatin, a lipid-lowering agent, was associated in one bilateral INO (816). A small wound infection from cocaine NUCLEAR AND INFRANUCLEAR OCULAR MOTILITY DISORDERS 1027 injection caused ophthalmoplegia and descending weakness mg/kg/day), but they occur frequently with progressively from botulism. A patient experienced diplopia from a tension larger doses. Optic neuropathy is the most dreaded complica- orbital pneumocele, caused by nasal obstruction from co- tion. A patient who ingested 7,500 mg became comatose caine abuse (817). Prenatal exposure to cocaine may have and developed a bilateral abducens nerve paresis (828). resulted in a case of Mo¨bius syndrome (524). Isotretinoin (Accutane, 13-cis-retinoic acid)is a vitamin A patient developed a reversible bilateral abducens nerve A analog used for the treatment of cystic acne. Used during palsy and cerebellar dysfunction while being treated with pregnancy, it is teratogenic. A child exposed in utero was high-dose cytosine arabinoside and mitoxantrone for acute born with congenital restrictive ophthalmoplegia, which may myelogenous leukemia (818). have been congenital ocular fibrosis syndrome, and gusta- Dichloracetylene is known to cause trigeminal neuropa- tory epiphora, a misdirection syndrome involving the sev- thy. Two men exposed to monochloracetylene and dichlor- enth cranial nerve. Both these conditions could have arisen acetylene fumes developed trigeminal neuropathy, followed because of loss of neurons in brain stem nuclei, around the days later by herpes zoster of the lips. One of them developed 5th week of gestation. In addition, the child had stigmata of transient trigeminal and abducens palsies, perhaps related to retinoic acid embryopathy (craniofacial and ear abnormali- the herpes zoster (819). ties, micrognathia, and a tall forehead)(829). Diphenylhydantoin was introduced as an anticonvulsant Systemic lead poisoning can cause a wide variety of visual by Merritt and Putnam in 1938. Nystagmus and reversible and ocular disturbances and a peripheral neuropathy. Bilat- generalized ophthalmoplegia occur with increasing dosages eral abducens nerve pareses occurred in 60% of a group of of diphenylhydantoin. We have seen patients with therapeu- 75 soldiers who developed acute lead poisoning from eating tic levels of Dilantin referred for presumed abducens nerve chili powder contaminated with lead chromate (830). This paresis who actually had a decompensated esophoria. Pa- may have resulted from increased ICP; however, cases of tients may also exhibit divergence insufficiency. This break- of the oculomotor and trochlear nerves have also down of fusional ability is reversible (820,821). been reported (824). Ecstasy (MDMA)abuse has been associated with one case Prenatal exposure to misoprostol has been associated with of bilateral abducens nerve palsy (822). Mo¨bius syndrome (525,526). Ethylene glycol (antifreeze)is usually drunk by mistake. The nitrofurans nitrofurantoin (Furadantin)and furalta- It is oxidized in part to oxalic acid. Two men developed done are antibacterial agents that cause both cranial and gen- transient horizontal diplopia from bilateral abduction weak- eralized polyneuropathies. Toxicity is related to dosage and ness during recovery from ethylene glycol poisoning. Inter- length of treatment. Impaired renal function hinders their estingly, both patients could see clearly in primary position excretion. The polyneuropathy is reversible if the drug is at near, suggesting that they may actually have had diver- withdrawn, but recovery may be quite slow. Abducens nerve gence paralysis rather than true abducens nerve palsies palsy appears together with other cranial nerve palsies and (823). A man developed left abducens paresis and bilateral recovers in 2 weeks after the drug is withdrawn (831). optic disc swelling after drinking 3 or 4 ounces of ethylene Orthoclone OKT3 is a monoclonal murine immunoglobu- glycol (40). lin G used to treat acute cellular rejection of allografted or- Gelsemium sempervirens, the yellow jasmine flower, gans. It can cause increased ICP with optic disk swelling yields a mixture of alkaloids used in the folk treatment of and bilateral abducens palsy (832). and neuralgias. Severe systemic reactions to the Phenylbutazone is an anti-inflammatory agent previously mixture are characterized by bradycardia, hypothermia, dys- widely used in the treatment of a number of conditions, in- phagia, weakness, and ocular motor paralysis with diplopia cluding gout, rheumatoid arthritis, and bursitis. Two patients and ptosis. Involvement of the abducens nerve is most com- developed unilateral abducens nerve palsies within 3 weeks mon. In nonfatal cases, the ocular symptoms always disap- of beginning the drug. The palsies resolved in both patients pear within several days (824). after the drug was stopped (833). Black tar heroin is injected subcutaneously or snorted. It Once used in the treatment of gout, piperazine citrate, a has caused outbreaks of wound botulism with ophthal- phenothiazine derivative, is employed as a base for medica- moplegia (825). tions used for parasitic diseases in humans and animals (e.g., A 13-year-old boy newly diagnosed with diabetes devel- ascariasis). In humans, overdosage produces diarrhea, urti- oped ophthalmoplegia and while receiving caria, weakness, extrapyramidal , and disturbed coor- an insulin infusion of 0.1 units/kg/hr. One dose of intrave- dination and equilibrium. A 4-year-old child developed a nous mannitol reversed the cerebral edema. The ophthal- unilateral abducens nerve paresis after he was given pipera- moplegia improved immediately and was completely gone zine syrup for treatment of intestinal parasites. The paralysis in 2 weeks (826). recovered within several months but recurred 5 days after ␣-2a-Interferon is used in the treatment of hepatitis C. A the drug was restarted (834). woman developed a left abducens nerve paresis 3 days after A 50-year-old man with erectile dysfunction developed a beginning treatment with this drug. The abducens nerve pa- pupil-sparing third nerve palsy 36 hours after the use of resis cleared within 6 weeks after treatment was stopped sildenafil citrate (Viagra)(835). (827). Ophthalmoplegia is a very common manifestation of en- Isoniazid (INH)is used primarily to treat tuberculosis. venomation by Russell’s viper, the Sri Lankan krait, the Pap- Toxic reactions rarely occur when small doses are used (3 uan taipan, and some other Asiatic snakes. If the patient does 1028 CLINICAL NEURO-OPHTHALMOLOGY not die of respiratory paralysis, rhabdomyolysis, coagulopa- 17. Lotufo DG, Smith JL, Hopen GR, et al. The pupil in congenital third nerve misdirection syndrome. J Clin Neuro Ophthalmol 1983;3:193–195. thy, or hemolysis, the ophthalmoplegia resolves in about 2 18. Wilbrand HO, Saenger A, Die kongenitalen augenmuskellahmungen (kernpla- weeks (836,837). sie). In Neurologie des Auges. Munich, JF Bergmann Verlag, 1921:179–182. 19. Mellinger JF, Gomez MR, Agenesis of the . In Vinken PJ, Bruyn Thalidomide, a nonbarbiturate hypnotic, is a teratogen, GW, eds. Handbook of Clinical Neurology. New York, Elsevier, 1977:395–414. causing phocomelia and sporadic occurrences of facial, pala- 20. Norman MG. Unilateral encephalomalacia in cranial nerve nuclei in neonates: tal, and abducens nerve palsies. Mo¨bius syndrome was re- Report of two cases. Neurology 1974;24:424–427. 21. Freedman HL, Kushner BJ. Congenital ocular aberrant innervation: New con- ported in a patient whose mother took the drug during her cepts. J Pediatr Ophthalmol Strabis 1997;34:10–16. pregnancy (523,838). 22. Brown HW, Congenital structural muscle anomalies. In Allen JH, ed. Strabismus Thallium, a heavy metal, is highly toxic, causing periph- Ophthalmic Symposium. St Louis, Mosby, 1950:205–236. 23. Engle E. Applications of molecular genetics to the understanding of congenital eral neuropathy, brain swelling, and visual system dysfunc- ocular motility disorders. Ann NY Acad Sci 2002;956:55–63. tion. In a few cases, thallium has injured the ocular motor 24. Engle E. The genetics of strabismus: Duane, Moebius, and fibrosis syndromes. In Traboulsi E, ed. Genetic Diseases of the Eye: A Textbook and Atlas. New nerves, either a single nerve or multiple nerves. If ocular York, Oxford University Press, 1998:477–512. motility is affected, there is always a polyneuritis involving 25. Engle E, Goumnerov B, McKeown CA, et al. Oculomotor nerve and muscle the extremities (839). abnormalities in congenital fibrosis of the extraocular muscles. Ann Neurol 1997; 41:314–325. Trichloroethylene (Trichloroethene)is a nonflammable 26. Gillies WE, Harris AJ, Brooks AM, et al. Congenital fibrosis of the vertically volatile liquid used as a solvent. Toxic exposure to the fumes acting extraocular muscles. A new group of dominantly inherited ocular fibrosis causes cranial polyneuropathy. One patient had incomplete with radiologic findings. Ophthalmology 1996;103:1517–1519. 27. Doherty EJ, Macy ME, Wang SM, et al. CFEOM3: A new extraocular congenital bilateral external ophthalmoplegia. Neuropathologic exami- fibrosis syndrome that maps to 16q24.2-q24.3. Invest Ophthalmol Vis Sci 1999; nation revealed pronounced degenerative changes of the 40:1687–1694. 28. Guirgis MF, Wong AMF, Tychsen L. Congenital restrictive external ophthal- brain stem nuclei, fascicles, and peripheral cranial nerves. moplegia and gustatory epiphora associated with fetal isotretinoin toxicity. Arch Another had facial , trigeminal motor weakness, Ophthalmol 2002;120:1094–1095. numbness, loss of taste on the anterior two thirds of the 29. Miller MT, Stromland K. Ocular motility in thalidomide embryopathy. J Pediatr Ophthalmol Strabis 1991;28:47–54. tongue, ptosis, and left abduction weakness (840). 30. Ramsay J, Taylor T. Congenital crocodile tears: A key to the aetiology of The vinca alkaloids, vincristine and vinblastine, are de- Duane’s syndrome. Br J Ophthalmol 1980;64:518–522. rived from the periwinkle plant. They bind to tubulin, pre- 31. Burian HM, Cahill JE. Congenital paralysis of medial rectus muscle with unusual synergism of the horizontal muscles. Trans Am Ophthalmol Soc 1952;50: vent microtubular formation, and arrest cells in metaphase. 87–102. Vincristine is used intravenously as cancer chemotherapy. 32. Cruysberg JR, Mtanda AT, Duinkerke-Eerola KU, et al. Congenital adduction palsy and synergistic divergence: A clinical and electro-oculographic study. Br Abducens nerve palsy may occur as a complication of vin- J Ophthalmol 1989;73:68–75. cristine therapy, usually as part of a generalized axonal poly- 33. Shivaram SM, Engle E, Petersen RA, et al. Congenital fibrosis syndromes. Int neuropathy (841–843). Ophthalmol Clin 2001;41:105–113. 34. Phillips PH. Strabismus surgery in the treatment of paralytic strabismus. Curr Opin Ophthalmol 2001;12:408–418. REFERENCES 35. Jimura T, Tagami Y, Isayama Y, et al. A case of synergistic divergence associ- ated with Horner’s syndrome. Folia Ophthalmol Jpn 1983;34:477–480. 1. Miller N. Solitary oculomotor nerve palsy in childhood. Am J Ophthalmol 1977; 36. Wilcox LMJ, Gittinger JWJ, Breinin GM. Congenital adduction palsy and syner- 83:106–111. gistic divergence. Am J Ophthalmol 1981;91:1–7. 2. Victor DI. The diagnosis of congenital unilateral third-nerve palsy. Brain 1976; 37. Miller NR, Kiel SM, Green WR, et al. Unilateral Duane’s retraction syndrome 99:711–718. (type 1). Arch Ophthalmol 1982;100:1468–1472. 3. Balkan R, Hoyt CS. Associated neurologic abnormalities in congenital third 38. Hotchkiss MG, Miller NR, Clark AWea. Bilateral Duane’s retraction syndrome: nerve palsies. Am J Ophthalmol 1984;97:315–319. A clinical pathologic case report. Arch Ophthalmol 1980;98:870–874. 4. Keith CG. Oculomotor nerve palsy in childhood. Aust NZ Ophthalmol 1987; 39. Wagner RS, Caputo AR, Frohman LP. Congenital unilateral adduction deficit 15:181–184. with simultaneous abduction. A variant of Duane’s retraction syndrome. Oph- 5. Schumacher-Feero LA, Yoo KW, Soleri FM, et al. Third cranial nerve palsy in thalmology 1987;94:1049–1053. children. Am J Ophthalmol 1999;128:216–221. 40. Brodsky M. Hereditary external ophthalmoplegia synergistic divergence, jaw 6. Good WV, Barkovich AJ, Nickel BL, et al. Bilateral congenital oculomotor winking, and oculocutaneous hypopigmentation: A congenital fibrosis syndrome palsy in a child with brain anomalies. Am J Ophthalmol 1991;111:555–558. caused by deficient innervation to ocular muscles. Ophthalmology 1998;105: 7. Hamed LM. Associated neurologic and ophthalmologic findings in congenital 717–725. oculomotor nerve palsy. Ophthalmology 1991;98:708–714. 41. Scassellati-Sforzolini G. Una sindroma molto rare: Diffeto congenito monolater- 8. Ing EB, Sullivan TJ, Clarke MP, et al. Oculomotor nerve palsies in children. J ale della elevazione con retrazione del globe. Riv Oto Neuro Oftalmol Cir Neurol Pediatr Ophthalmol Strabis 1992;29:331–336. Sud Am 1958;33:431–439. 9. Parmeggiani A, Posar A, Leonardi M, et al. Neurological impairment in congeni- 42. Pruksacholawit K, Ishikawa S. Atypical vertical retraction syndrome: A case tal bilateral ptosis with ophthalmoplegia. Brain Dev 1992;14:107–109. study. J Pediatr Ophthalmol 1976;13:215–220. 10. Patel CK, Taylor DS, Russell Eggitt IM, et al. Congenital third nerve palsy 43. Kommerell G, Bach M. A new type of Duane’s syndrome. Neuroophthalmology associated with mid-trimester amniocentesis. Br J Ophthalmol 1993;77: 1986;6:159–164. 530–533. 44. Weinacht S, Huber A, Gottlob I. Vertical Duane’s retraction syndrome. Am J 11. Prats JM, Monson MJ, Zuazo E, et al. Congenital nuclear syndrome of the Ophthalmol 1996;122:447–449. oculomotor nerve. Pediatr Neurol 1993;9:476–478. 45. Khodadoust AA, von Noorden GK. Bilateral vertical retraction syndrome: A 12. Flanders M, Watters GB, Draper J, et al. Bilateral congenital third cranial nerve family study. Arch Ophthalmol 1967;78:606–612. palsy. Can J Ophthalmol 1989;24:28–30. 46. Rampoldi R. Casuistica clinica. II. Singolarissimo caso squilibrio motorio oculo- 13. Al-Gazali LI, Sztriha L, Punnose J, et al. Absent pituitary gland and hypoplasia palpebrale. Ann Ottal 1884;12:463–469. of the cerebellar vermis associated with partial ophthalmoplegia and postaxial 47. Loewenfeld IE, Thompson HS. Oculomotor paresis with cyclic : A criti- polydactyly: A variant of orofaciodigital syndrome VI or a new syndrome? J cal review of the literature and a new case. Surv Ophthalmol 1975;20:81–124. Med Genet 1999;36:161–166. 48. Fells P, Collin JRO. Cyclic oculomotor palsy. Trans Ophthalmol Soc UK 1979; 14. Papst W, Esslen E. Elektromyographischer beitrag zum Mobius syndrom. Klin 99:192–196. Monatsbl Augenheilkd 1960;137. 49. Bateman DE, Saunders M. Cyclic oculomotor palsy: Description of a case and 15. Tsaloumas M, Willshaw H. Congenital oculomotor palsy: Associated neurologi- hypothesis of the mechanism. J Neurol Neurosurg Psychiatry 1983;46:451–453. cal and ophthalmological findings. Eye 1997;11:500–503. 50. Parlato CJ, Nelson LB, Harley RD. Cyclic strabismus. Ann Ophthalmol 1983; 16. Sun CC, Kao LY. Unilateral congnital third cranial nerve palsy with central 15:1126–1129. anomalies: Report of two cases. Chang Gung Med J 2000;23: 51. Frenkel REP, Brodsky MC, Spoor TC. Adult-onset cyclic esotropia and optic 776–781. atrophy. J Clin Neuro Ophthalmol 1986;6:27–30.