Journal of Neuro-Ophthalmology, December 1986, Volume 6, Issue 4

Acute, severe, symmetric visual loss with cecocentral scotomas due to olfactory groove meningioma.

Cecocentral scotomas are a hallmark of toxic, metabolic, and hereditary optic neuropathies, but are rarely associated with compressive processes. Acute visual loss due to a compressive optic neuropathy by a benign tumor is unusual unless intratumoral hemorrhage or infarction occurs. A case of acute, severe, remitting, symmetrical visual loss with cecocentral scotomas, initially thought to be of toxic etiology, proved to be due to a typical suprasellar meningioma originating in the olfactory groove. A history of olfactory disturbance with visual loss should prompt neuroradiologic investigation despite atypical neuro-ophthalmic features.

"'1/"""", CUI/i'l" N.'rmH,,"rllr,,'I/""",'':'II Itl"': !!"··!!7. ,'IHIt. Acute, Severe, Symmetric Visual Loss with Cecocentral Scotomas Due to Olfactory Groove Meningioma Michael L. Slavin, M. D. Cl) 1986 Raven Press, New York Cecocentral scotomas are a hallmark of toxic, metabolic, and hereditary optic neuropathies, but are rarely asso­ciated with compressive processes. Acute visual loss due to a compressive optic neuropathy by a benign tumor is unusual unless intratumoral hemorrhage or in­farction occurs. A case of acute, severe, remitting, sym­metrical visual loss with cecocentral scotomas, initially thought to be of toxic etiology, proved to be due to a typical suprasellar meningioma originating in the olfac­tory groove. A history of olfactory disturbance with vi­sual loss should prompt neuroradiologic investigation despite atypical neuro-ophthalmic features. Key Words: Cecocentral scotoma-Olfactory groove meningioma-Toxic optic neuropathy. From the Division of Neuroophthalmology, Department of Ophthalmology, Long Island Jewish Medical Center, New Hyde Park, New York, and the School of Medicine, Health Sciences Center, State University of New Yurk at Stony Brook, Stony Brook, New York. Presented at the Frank Walsh Society, 18th Annual Meeting, Seattle, Washington, February 21, 1986. Address correspundence and reprint requests to Dr. M. Slavin, Long Island Jewish Medical Center, New Hyde Park, NY 11042, U.S.A. 224 Visual dysfunction secondary to compression of the intracranial optic nerves and optic chiasm is commonly due to pituitary adenoma and supra­sellar meningioma. Chronic progressive central and peripheral visual loss with ensuing optic at­rophy comprise the typical syndrome. Acute vi­sual loss secondary to anterior visual pathway compression by "benign" tumors is infrequent and usually due to hemorrhage within or infarc­tion of a pre-existing pituitary adenoma (pituitary apoplexy). I report a case of acute bilateral symmetric visual loss secondary to compression of the optic nerves by a suprasellar meningioma originating in the ol­factory groove. In addition, cecocentral scotomas, often attributed to toxic or hereditary optic neu­ropathies, but rare with compressive processes, were detected on perimetry. CASE REPORT A 28-year-old morbidly obese man noted the onset o{a "blind spot" in the left eye in September 1982. Since August 1982 he was a taxicab dis­patcher and attributed the visual dysfunction to exposure to taxicab fumes. He also used a kero­sene heater each night at work with inadequate ventilation. Ocular examination in November 1982 revealed visual acuities of 20/25 in the right eye and 20/30 in the left eye. "Waxiness" of the optic disks and a "hamartoma" near the left disk were described. In December 1982 he reported severe bilateral visual loss over a 2-week period. Neuro­ophthalmic examination revealed visual acuity of 3/200 in each eye. Bilateral cecocentral scotomas were noted on Goldmann perimetry. The pupils measured 7 mm on each side with a 1+ reaction to light, and no afferent pupil defect. Each optic disk (Fig. 1) appeared slightly edematous nasally and VISUAL LOSS WITJf MENINGIOMA 225 Fig. 1. Swelling of the nasal side of each optic disk and temporal pallor of left optic disk (right). Marked tortuosity of retinal venules is noted. An elevated subretinal le­sion in the peripapillary area of the left eye is present (arrow). temporal pallor of the left optic disk was noted. A slightl~' raised peripapillary subretinal lesion was present on the left side. There was marked tortu­osity of \'enules on each side. Fluorescein angiog­raph~' (Fig. 2) failed to show fluorescein dye leakage from the optic disk and the peripapillary lesion seemed compatible with a neovascular membrane. Vitamin 8 12 , folate, serum carbon monoxide levels, and fluorescent treponema I antibody titer were unremarkable. Family history was reviewed and was not contributory. He was asked to stay out of his work environment and return in 2 Fig. 2. Fluorescein angiography of the left eye in early arteriovenous phase. Note venous tortuosity but the absence of superficial capillary telangiectasia. A peri­papillary retinal net is present. weeks for further assessment and neuroradiologic studies. He noted rapid improvement on re-exam­ination and visual acuities were 20/400 in each eye. The patient was subsequently lost to follow-up until June 1983 when vision was reduced to 1/200 in the right eye and 5/200 in the left eye. A history of taste and smell problems was elicited although this was statedly present for at least 1 year. Optic disks showed distinct pallor. Visual fields were similar in confrontation to the earlier study. Al­though computed tomography (CT) of the head was scheduled, it was not performed since several CT centers stated that their scanner would not ac­commodate a 350-lb patient' Skull x-rays were therefore suggested but the patient was again lost to follow-up. The patient telephoned 18 months later stating that CT had been performed. A large, homoge­nously enhancing mass (with surrounding edema) in the olfactory groove (Fig. 3) with extension to the suprasellar cistern, ethmoid sinuses, and nasal cavity was present. Frontal craniotomy was performed and con­firmed the radiologic impression of olfactory groove meningioma. Pathologically, a typical me­ningioma was noted with no features of malig­nancy, cyst formation, or hematoma. Although the optic nerves were surgically decompressed, marked visual compromise remained. DISCUSSION Visual loss with cecocentral scotomas is often at­tributed to toxic or hereditary optic neuropathies. Such syndromes as tobacco-alcohol amblyopia (1), nutritional optic neuropathy [vitamin 8 12 (2), , C1," NCllrtHll'''tllll/",ol, Vol. 6, No.4, 1986 226 M. L. SLAVIN Fig. 3. Computed axial tomography through orbits (left) and suprasellar space (right) shows a large homogenously enhancing lesion in the olfactory groove with extension to the suprasellar space, ethmoid sinuses, right medial orbit, and nasal cavity. Note surrounding edema (arrow). vitamin B1 (3) deficiency), and dominant optic at­rophy (4) typically cause gradual visual loss that is bilateral and often symmetrical. Acute bilateral vi­sualloss with central scotomas may also follow in­gestion of various substances, such as ethambutol (5), methyl alcohol (6), and ethylene glycol (7). In­dustrial exposure to carbon disulfide (8) (rubber and rayon industry) and carbon tetrachloride (9) (cleaning and degreasing industry) have been as­sociated with toxic optic neuropathies. Nitrous oxide and carbon monoxide exposure may cause cerebral blindness but to my knowledge has not been reported to cause optic neuropathy. Acute bilateral visual loss (sequentially within days or weeks) with cecocentral scotomas in a young man suggests the diagnosis of Leber's optic neuropathy (10). "Pseudo-edema" of the optic nerves, peripapillary telangiectasia of the micro­vasculature, and lack of tluorescein dye leakage on tluorescein angiography are characteristic signs. A positive family history clinches the diagnosis. Chronic progressive visual loss with bilateral central scotomas due to compression of anterior visual pathways is uncommon. Gutman and asso­ciates (11) reported three cases of suprasellar mass lesions (histiocytosis, chiasmal glioma, cranio­pharyngioma) that presented with tluctuating vi- I CIIIl Nt'lIrl1-(IphtIIll111lo/, Vol. b, No.4. jq8b sual loss and bilateral central scotomas. Page and Sanders (12) reported four patients with this asso­ciation. One patient with a subfrontal meningioma had a I-.vear historY. of visual loss. There was no perception of smell, and long tract signs were present on neurological examination. Bifrontal as­trocytoma with microcystic degeneration, chromo­phobe adenoma, and craniopharyngioma were the diagnoses in the other three patients. Finn and Mount (13) reviewed 83 cases of meningioma of the tuberculum sellae and planum sphenoidale, none of which were associated with bilateral cen­tral scotoma. Rucker and Kearns (14) reported five cases of in­tracranial meningioma with atypical visual clinical symptoms and signs. One patient suffered visual loss in one eye for 3 weeks with spontaneous re­covery, followed by visual loss in the fellow eye for 1 week again with spontaneous recovery. A second patient presented with acute bilateral vi­sual loss while pregnant, but no associated neuro­logic signs. A third patient was an alcoholic with chronic progressive bilateral visual loss and central scotomas who was thought to have toxic optic neuropathy. My patient presented with acute, symmetrical, remitting, visual loss with cecocentral scotomas, VISUAL LOSS WITH MENINGIOMA 227 all atypical features of a "benign" subfrontal me­ningioma. Toxic, metabolic, and hereditary optic neuropathies were strongly considered. A history of olfactory dysfunction with ul1l'xplained visual deficit (even remitting) should prompt early neu­roradiologic investigation despitt' atypical neuro­ophthalmic features. REFERENCES 1. Traquair HM. Tl',ic .lmbIYl'pi.1 including rl'twbulb.u neu­ritis. T","" lll'h1J"IIIIII" ";"<' 1I1\ IlJJll;:ill:J:iI-I':i. 2. Talbot G. rl'rnil"il'us .1Iwmi.1 with rl'twbulb.lr neuritis. 8r I Op/rtllallll,,1 IlJJt'l;211:t'lllJ-21. 3. Be,1m AD. AmbIYl'pi.1 dUl' tl' dil'tolr\, ddiciency. Alii 10ph­tllllllll,, 1 IlJ~7;311:t'lt'l-711. ~. Kjer P. Intantile "ptk .Itn'ph\' with dl'minant mode of in­heritance: ,I clinical ,1I1d ~t.'netil- study l,f 19 Danish fami­lies. Ada Opllli"I/III,,1 (Kbhl [5uppII1959;5~. 5. Carr RE, Henkind P. Ocular manifestations of ethambutol. Ardt Ophti/llhll,,1 1962;67:566-71. 6. ROt' O. Clinical investigation of methyl alcohol poisoning with special rderences to the pathogenesis and treatment of amblyopia. Acla Mrd Scnwi 1943;113:558-608. 7. Fril'dman EA, Greenberg jB, Merrill jP, Drammin Gj. Con­sequl'nces of ethylene glycol poisoning. Am I Med 1%2;32:1\91 -902. H. Vigiliani EC. Clinical observations on carbon disulfide in­toxication in Italy. IIId""t Mrd SlIrg 1950;19:249-52. 9. Gocher TEP. Carbon tetrachloride poisoning. Northwest M,'d 1944;43:221\-30. 10. Smith )L, Hoyt WF, Susac )0. Ocular fundus in acute Leber optic neuropathy. Arch 0l'hlhallllol 1973;90:349-54. II. Gutman I, Behrens M, Odel ). Bilateral central and centro­caecal scotomata due to mass lesions. Br I Ophthalmol 1984;68:336-42. 12. Page NGR, Sanders MD. Bilateral central scotoma due to intracranial tumor. Br I OphthalmoI198~;68:«9-57. 13. Finn )E, Mount LA. Meningiomas of the tuberculum sellae and planum sphenoidale. Arch Ophtha/mo/ 1974;92:23-7. 14. Rucker CWo Kearns TP. Mistaken diagnosis in some cases of meningioma. Alii I OphthalnI011961;5l:15-9. I Clill Nl'/lYlH'I'htiw/l/loi. Vol. 6. No.4. 1986