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Show Journal of C1illlcal Neuro- ophtiullmology 9( 4): 254- 257, 1989. Bilateral Internuclear Ophthalmoplegia Associated with Fourth Ventricular Dermoid Tumor Ismail H. Tekkok, M. D., Giyas Ayberk, M. D., Tulay Kansu, M. D., and Suleyman Saglam, M. D. © 1989 Raven Press, Ltd., New York We report an unusual case of bilateral internuclear ophthalmoplegia occurring in association with fourth ventricular dermoid tumor and we review the current literature. Key Words: Bilateral internuclear ophthalmoplegiaDennoid tumor- Fourth ventricle. Hacettepe University School of Medicine, Ankara, Turkey Address correspondence and reprint requests to Ismail H. Tekknk at 2 nei Caddp No 1- 6. Kivanc Apt, Bahcelievler, Ankara 06500, Turkey. 254 Bilateral internuclear ophthalmoplegia results from bilateral lesions of the medial longitudinal fasciculi and is usually considered to be a pathognomonic sign of demyelinating disease ( 1). However, many other causes have been defined in recent years. To our knowledge, our case report is the only one in the literature published in English that shows an association with a fourth ventricular dermoid tumor ( Table 1) ( 2- 23). CASE REPORT A 7- year- old girl presented with unsteady gait and impairment of her vision. She was known to be late in reaching her developmental stages. Her unsteadiness had been profound for the last 2 years and she had been vomiting quite frequently for - 6 months before admission. Her teacher observed a progressive impairment of her concentration during the last 3 months. She was always complaining of headaches and neck pain. On admission her head circumference was 54.5 em. She was fully alert, cooperative, and had no disorientation. Neurological examination revealed limited adduction bilaterally. Nystagmus was present in all directions; it was most marked with lateral gaze and in the abducting eye. Her visual acuity was 20/ 25 bilaterally and the visual fields were full on confrontation. Funduscopic findings were normal. Facial sensation and corneal reflex were diminished bilaterally. She had minimal leftsided facial paresis. Gag reflex and other lower cranial nerve functions were normal, as well as the power, tone, and sensation in her limbs. The cerebellar functions were impaired, most markedly on the left. Gait was ataxic, and she had a tendency to fall to left. BILATERAL INTERNUCLEAR OPHTHALMOPLEGIA 255 a BIND, Bilateral internuclear ophthalmoplegia. TABLE 1. Summary of previously reported causes of bilateral internuclear ophthalmoplegia The plain skull x- rays showed digital impressions, A computed tomographic ( CT) scan demonstrated a cystic tumor within the fourth ventricle that distorted the brain stem forwards and obstructed the CSF passageways, hence causing triventricular hydrocephalus ( Fig. 1). Results of routine laboratory tests were found to be within the normal range. A suboccipital craniectomy was performed on March 22, 1988 with the girl in the sitting position. The vermian portion of the cerebellum was hypoplastic with a bulging midline lesion. Small cheesy particles were flowing freely in the cisterna magna. The surface of the tumor was also cheesy and fragile and could be easily cleaved from the cerebellar hemispheres, It was about 6 cm in diameter. The central part of the tumor was quite solid, As soon as the tumor was separated from cerebellar hemispheres, it fell off ( Fig. 2a, b). There was only one feeding vessel coming from the anterior aspect. The CSF passageway was then free of obstruction. The postoperative period was uneventful. On the fifth postoperative day her ophthalmoplegia was significantly improved and her gait was more steady. The histopathological diagnosis was a dermoid tumor ( Fig. 3). A postoperative CT scan showed no residual tumor ( Fig. 4). FIG. 1. Preoperative enhanced CT- scan demonstrating a huge mass within the fourth ventricle that distorts the brainstem. DISCUSSION Dermoid tumors account for 0.1- 0.7% of all intracranial space- occupying lesions; they arise from displacement of epithelial tissue early in the intrauterine life. Dermoids are more frequent in children and the middle- aged ( 24). Their rate of increase in size is slow and the clinical course of the disease is rather swift, especially for dermoids occurring within the posterior fossa, where the average duration of symptoms is < 1 year ( 24). Only a limited number of fourth ventricular dermoids have been reported in large series ( 24,25). None of these cases reported bilateral internuclear ophthalmoplegia, as exhibited in our patient. The vast majority of other fourth ventricular tumors reported in the literature to have bilateral internuclear ophthalmoplegia were malignant, i. e., medulloblastomas ( 4,5) and a choroid plexus papilloma ( 5), whereas only one case of a benign tumor was reported in 1981 to exhibit bilateral internuclear ophthalmoplegia, which was histologically confirmed as an epidermoid ( 18). Although the mechanism of bilateral internuclear ophthalmoplegia in the presence of an extrinsic lesion is far from clear, it is best explained by the stretching of the basilar paramedian arteries resulting in selective ischemia of the medial longitudinal fasciculi and/ or actual brainstern compres- Author( s) Weiner et al. 1967 ( 2) Ford et aI., 1983 ( 19) Rizzo and Corbett, 1983 ( 20) Bodziner et aI., 1983 ( 21) Donhowe, 1984 ( 22) Inosencio and Ballecer, 1985 ( 23) Herishanu and Lavy, 1969 ( 3) Cogan and Wray, 1970 ( 4); Fotzsch, 1971 ( 5) Wallace & CulebrasFernandez, 1970 ( 6); Gonyea, 1974 ( 7) Cogan, 1970 ( 8) Cogan, 1970 ( 8); Nishikazi et aI., 1985 ( 9) Gonyea and Heilman, 1972 ( 10) Schmidt and Zimmerman, 1973 ( 11) Rich et aI., 1974 ( 12); Baker, 1979 ( 13); Beck and Meckler, 1981 ( 14), Keane, 1987 ( 15) MacDonald and Sher, 1977 ( 16) Deveraux et aI., 1979 ( 17) Deveraux et aI., 1979 ( 17) Schraeder et aI., 1981 ( 18) Cause of BIND · Branched chain ketoami-noaciduria Subdural haematoma Basilar artery aneurysm Fourth ventricular epider-moid tumor Carcinomatous meningitis Narcotic overdose Meningoencephalitis Doxepine overdose Tuberculosis granuloma in midbrain Angiokeratoma corporis diffusum ( Fabry's Disease) Head injury Neurosyphilis Arnold- Chiari malformation with hydrocephalus Cryptococcal meningitis Occlusive cerebrovascular disease Brainstem tumors Spinocerebellar degeneration Vasculitis I Clill Neuro- ophthalmol. Vol. 9, No, 4, 1989 256 1. H. TEKKOK ET AL. FIG. 2. A and B: Peroperative photograph showing complete removal of the tumor. sion ( 3,6). The rapid return to normal eye movements in our patient supports the view that brainstem compression was the cause of bilateral internuclear ophthalmoplegia. FIG. 3. Photomicrograph of the tumor specimen showing skin appendages and a hair follicle ( arrow). Hematoxylin and eosin stain. x148. FIG. 4. Postoperative CT- scan showing no residual tumor. REFERENCES 1. Smith JL, Cogan DG. Internuclear ophthalmoplegia. A review of fifty- eight cases. Arch Ophthalmol 1959; 61: 687-{ j94. 2. Weiner LP, Konigsmark BW, Stoll J. Hereditary olivopontocerebellar atrophy with retinal degeneration. Report of a family through six generations. Arch Neural 1967; 16: 3~ 376. 3. Herishanu Y, Lavy S. Transient bilateral internuclear ophthalmoplegia of vascular origin. A case report. Ophthalmologica 1969; 159: 324-- 327. 4. Cogan DG, Wray SH. Internuclear ophthalmoplegia as an early sign of brainstem tumors. Neurology 1970; 20: 629-{) 33. 5. Fotzsch R. Die internukleare ophthalmoplegie. Ophthalmologica 1971; 162: 331- 342. 6. Wallace TW, Culebras- Fernandez A. Posterior cerebral artery embolism: hemianopsia and median longitudinal fasciculus syndrome. Cleve Clin Q 1970; 37: 157- 160. 7. Gonyea EF. Bilateral internuclear ophthalmoplegia. Association with occlusive cerebrovascular disease. Arch Neurol 1974; 31: 168- 173. 8. Cogan DG. Internuclear ophthalmoplegia, typical and atypical. Arch Ophthalmol 1970; 84: 58J...- 589. 9. Mishizaki T, Tamaki N, Nishida Y, Matsumoto S. Bilateral internuclear ophthalmoplegia due to hydrocephalus. A case report. Neurosurgery 1985; 17: 822-- 825. 10. Gonyea EF, Heilman KM. Neuroophthalmic aspects of central nervous system cryptococcosis. Internuclear ophthalmoplegia and supranuclear ophthalmoplegia. Arch OphthalmoI1972; 87: 1~ 168. 11. Schmidt D, Zimmermann H. 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I Neurosurg 1981; 54: 403- 405. 19. Ford CS, Cruz J. Biller }, Laster W, White DR. Bilateral internuclear ophthalmoplegia in carcinomatous meningitis. I Clin Neuro- ophthalmol 1983; 3: 127- 130. 20. Rizzo M, Corbett}. Bilateral internuclear ophthalmoplegia reversed by naloxone. Arch NeuroI1983; 40: 242- 243. 21. Bodziner RH, Singer W, Hedges TR. Bilateral internuclear ophthalmoplegia in meningoencephalitis. Oev Med Child Neurol 1983; 25: 819- 820. 22. Donhowe SP. Bilateral internuclear ophthalmoplegia from doxepin overdose ( letter). Neurology 1984; 34: 259. 23. Inocencio FP, Ballecer R. Tuberculosisi granuloma in the midbrain causing wall- eyed bilateral internuclear ophthalmoplegia ( Webino). I Clin Neuro- ophthalmoI1985; 5: 31- 35. 24. Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. I Neurosurg 1981; 47: 403- 405. 25. Love } G, Kernohan } W. Dermoid and epidermoid tumors of central nervous system. lAMA 1936; 107: 1876- 1883. 1Clin Neuro- ophtha/ mol. Vol. 9, No. 4, 1989 |
