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Show ]. Clin. Neuro-ophthalmol. 3: 259-262, 1983. Optic Neuropathy and Cerebral Sarcoidosis MICHAEL L. SLAVIN, MD. JOEL S. GLASER, MD. Abstract An intracranial mass lesion is a rare, but reported presenting sign in sarcoidosis. A 39-year-old woman presented with a frontal lobe lesion (pathologically a noncaseating granuloma) associated with chronic progressive visual loss and optic atrophy on the right side. High-resolution axial and coronal computerized tomography revealed a parachiasmal density compatible with the clinical course. Clinical suspicion of granulomatous processes and appropriate workup in all cases of optic neuropathy may allow for early medical therapy in such cases. Introduction The ophthalmologist is well aware of the association of granulomatous uveitis and systemic sarcoidosis. However, intracranial involvement of the visual pathways by this disease process is not often appreciated. Symptoms of chronic progressive visual loss, bitemporal or homonymous hemianopia, and signs such as progressive optic atrophy or disc swelling are, as a rule, rarely construed as evidence of sarcoidosis. We report a patient presenting with a frontal lobe mass, biopsy confirmed as neurosarcoidosis, who subsequently developed unilateral progressive visual loss and optic atrophy secondary to suprasellar granuloma. Since medical treatment is often successful in halting visual loss due to sarcoidosis, every ophthalmologist should be aware of the manifestations of intracranial sarcoidosis. Case Report A 39-year-old, obese black woman was admitted to the orthopedic service of Jackson Memorial Hospital on January 22, 1978, for evaluation of increasing pain on motion in the right knee. She From the Department of Ophthalmology, Long Island ]ewishHillside Medical Center, School of Medicine. Health Sciences Center. State University of New York at Stony Brook. Stony Brook. New York (MLS); and Bascom Palmer Eye Institute. University of Miami School of Medicine, Miami. Florida (JSG). December 1983 had previously undergone knee surgery for traumatic arthritis. In addition, occasional right-sided frontal headaches had occurred for 2-3 months. There were no visual complaints. A broken surgical clip was discovered on x-ray of the right knee. Routine chest x-ray showed nodular, and possibly cavitating, densities in the right upper lobe, with some fullness of the azygous node region. Bronchoscopy showed no evidence of tumor or granuloma, but did show focal obstructive lipid pneumonitis with lymphocytic infiltrate. Smears and sputum cytology for acid fast bacilli (AFB) of the bronchial washings were negative. A skull film showed possible enlargement of the sella turcica and, therefore, a computerized tomographic (CT) scan was done. This serendipitously showed an enhancing large mass lesion of the right frontal lobe. Cerebral angiography confirmed the presence of an avascular intra-axial, deep right frontal mass. The patient refused any operative procedure and signed out against medical advice. Six months later, the patient was readmitted with severe right frontal headaches and slight weakness of the left arm and leg. Neurological examination showed a mild left hemiparesis with a slight decrease to pain stimuli of the left side of the body. Chest x-ray showed no change from the study done 6 months earlier. CT scan showed an enlarging lesion in the right frontal lobe, with a large amount of surrounding edema. This enhancing lesion extended minimally across the midline, with compression of the right frontal ventricular horn. Lumbar puncture was deferred. First- and secondstrength protein-purified derivative (PPO) were weakly positive. The differential diagnosis at this time included: tuberculoma, fungal lesion or other granuloma, astrocytoma, or metastatic tumor. A right frontal craniotomy on August 9, 1978, with subtotal removal of the lesion revealed the cerebral cortex, subcortical white matter, and the overlying leptomeninges to be infiltrated with noncaseating granulomas consistent with cerebral sarcoidosis. Cultures for tuberculosis were negative. Postoperative CT scan showed minimal residual edema of the right frontal lobe and follow-up assessment showed resolution of the left hemiparesis. 259 Sarcoid Optic Neuropathy (c) Figures la -Ie. (a) Marked bilater.ll frontal lobe involvement with bowing of the falx to the right. (b,c I Coronal sections. (Figure lb reproduced with permission from Post. M.jD.. Quencer, R., .lnd Tabei. S.Z.: CT demonstration of s.lrcoidosis of the optic nerve, frontal lobes, and falx cerebri: C.lse report and literature review. Am. ./. Neuroradiol. September/October 1982.) In January, 1979, the patient had several grand mal seizures for which phenobarbital was begun. One month later, blurred vision in the right eye was first noted. On March 27, 1979, the patient was readmitted for uncontrolled seizures, persist- ~60 ent frontal headaches, and progressive visual loss in the right eye. CT scan showed an enhancing mass in the left frontal lobe, with increased irregular enhancement of the falx with only minimal right-sided changes. Unfortunately, no basal sec- Journal of Clinical Neuro-ophthalmology tions were demonstrated on this study. Eye examination showed visual acuity of 20/200 on the right and 20/15 on the left, with a right-sided afferent pupillary defect and pallor of the right optic disk. Visual fields demonstrated a dense central scotoma with depression of the temporal periphery in the right eye, and a normal field for the left eye. Optic canal tomograms were unremarkable. Appropriate axial and coronal basal CT studies were scheduled, but the patient signed out against medical advice and was lost to follow-up for several months. The patient was once again admitted on August 21, 1980, for uncontrolled seizures due to poor anticonvulsant compliance. Visual acuity was 1/200, right, and 20/15, left. Visual fields showed a dense central scotoma in the right eye and a normal left field. Nodular infiltration of the left cheek and a plaque with central darkening of the left shin were noted; biopsy of each was compatible with dermal sarcoidosis. CT scan with axial and coronal sections (Figs. 1a -Ie) showed bilateral frontal lobe involvement with a large enhancing lesion on the left, displacement of the falx to the right, and marked edema on the right side. In addition, an increased density in the right parachiasmal area with extension to the level of the intracranial right optic nerve was seen, with invasion into the sellar region (Figs. 2a and b). The patient was discharged on Decadron 4 mg p.o. every 4 hours, as well as antiseizure medication. No further follow-up examination was possible and the patient expired without medical attendance; no autopsy was conducted. Comment Central nervous system (CNS) involvement by sarcoidosis occurs in approximately 5% of all cases of the systemic disease. I It is not unusual. however, for neurologic symptoms to herald the presence of sarcoidosis. The most common intracranial site of involvement is at the base of the brain, with the cranial nerves, pituitary gland, third ventricle, and the hypothalamus being targeted preferentially.~ However, no portion of the CNS is immune. The facial nerve is not only the most commonly involved cranial nerve, but facial palsy is the most common CNS disorder seen in sarcoidosis.;] The optic nerve is the next most frequently involved cranial nerve.' CNS sarcoidosis may present either as an infiltrative granulomatous process or as discrete tumoral mass or masses. About 20 cases have been reported of sarcoid granulomas presenting as intracranial masses." 2. 4 Powers and Miller" reported a case of a 29-year-old with sarcoidosis who presented with seizures and was later discovered to have a temporal lobe avascular mass, with a CT picture consistent with grade III or IV glioma. December 1983 Slavin. Glaser Figures 2a and 2b. (a) Axial section showing density in area of right optic nerve (arrows). (b) Coronal section with parachiasmal involvement (.nrow). (Figures 2.1 and 2b reproduced with permission from Post, M.ID., Quencer, R., ,md Tabei, S.Z.: CT demonstr.Jtion of s.ncoidosis of the t>ptic nere, frontal lobes .Jnd falx cerebri: Case report and liter.Jture review. Am. ,. I\:eurorJdiol. September/October 1082.) Prednisone therapy, however, resulted in marked improvement in both the clinical ,1Od radiologic picture, with the impression that the lesion was granulomatous in nature. Optic nerve involvement at all levels (intraocular6 - H intraorbital,~ intracanalicular,'" and intracranial l !-IC') is well-recognized. Sarcoid granulomas may involve the optic nerve head itself as a solitary process, or may be associated with more charac'teristic fundal features such as perivascular exudates (candle wax dripping), neuroretinitis, or grayish- white preretinal lesions in the inferior part of the fundus, as described by Landers. lli Optic neu- 261 Sarcoid Optic Neuropathy ritis, papilledema, and secondary optic atrophy have been described. l7 · IH Aszkanazi reported two patients with bitemporal hemianopia; one showed granulomatous infiltration of the chiasm, perichiasmal area, and base of the brain at postmortem exam; and the second showed a "tumor of the optic chiasm with third ventricular extension" seen at exploratory craniotomy. Moraxll reported a sarcoid granuloma involving both optic nerve and chiasm. The latter case, as well as the two of Aszkanazy, were evaluated in the pre-CT era; therefore, radiologic procedures were not useful in delineating these lesions. Decker1:) recently reported a patient with sarcoidosis who had bilateral progressive loss of vision, with temporal visual field loss in one eye. Skull films showed clouding in the sphenoid sinus and a normal sella turcica. CT showed an enhancing suprasellar mass. At surgery via the transphenoidal approach, inflammatory changes were observed in the mucosa of the sphenoid sinus and in the pituitary gland. A suprasellar granuloma was removed. Visual acuity and visual fields improved bilaterally. Our patient showed unilateral visual loss with CT evidence of a suprasellar mass, contiguous with a frontal lobe infiltrate known to be due to sarcoidosis. Another CT-documented case was reported by McLaurin,14 who demonstrated a suprasellar enhancing mass in a patient with sarcoidosis presenting as visual loss and an incongruous homonymous hemianopia, suggesting optic tract and posterior chiasmal encroachment. Steroid therapy increased central acuity bilaterally, and improved the pretreatment field defect. A right homonymous hemianopia was reported by Everts5 in a patient with a large left occipital lobe lesion caused by sarcoidosis, which also responded to corticosteroids. Therefore, the visual system may be involved by this granulomatous process at virtually any level, from the eye to the occiput, by either an infiltrative or compressive process. Visual loss in a patient with sarcoidosis must be evaluated thoroughly, including thin-section computerized tomography of the base of the brain (axial and coronal views) with contrast enhancement. The demonstration of a mass lesion in a patient with sarcoidosis warrants a trial of systemic corticosteroids when there is progressive visual loss. Surgery may be useful in those cases resistant to steroids or where the diagnosis is in doubt. A more diffuse granulomatous inflammation at the base of the brain acting as a basal arachnoiditis, associated with visual loss but with a negative CT scan, should be evaluated with repeat CT after injection of intrathecal metrizamide. 16 Again, a trial of medical therapy should be instituted. 262 References 1. Delaney, P.: Neurologic manifestations in sarcoidosis. Ann. Intern. Med. 87: 336, 1977. 2. Aszkanazy, e.L.: Sarcoidosis of the central nervous system. r Neuropathol. Exp. Neurol. 11: 392-400, 1952. 3. James, D.G., and Sharma O.P.: Neurological complications of sarcoidosis. Proc. R. Soc. Med. 60: 1169-1174,1967. 4. Popper, rS., Bingham, W.G., and Armstron, F.5.: Sarcoid granuloma of the cerebellum. Neurology 10: 942-946, 1960. 5. Powers, W,J., and Miller, E.M.: Sarcoidosis mimicking glioma: Case report and review of intracranial sarcoid mass lesions. Neurology 31: 907-910, 1981. 6. Jampol, L.M., Woodfen, W., and McLean, LB.: Optic nerve sarcoidosis. Arch. Ophthalmol. 87: 355360,1972. 7. Laties, A.M., and Scheie, H.G.: Evolution of multiple small tumors in sarcoid granuloma of the optic disc. Am. r Ophthalmol. 74: 60-67, 1972. 8. Kelley, J.5., and Green, W.R.: Sarcoidosis involving the optic nerve head. Arch. Ophthalmol. 89: 486488, 1973. 9. Rush, J.A.: Retrobulbar optic neuropathy in sarcoidosis: Ann. Ophthalmol. 12: 390-394, 1980. 10. Anderson, W.B., Parker, J.]., and Sondheimer, F.K.: Optic foramen enlargement caused by sarcoid granuloma. Radiology 86: 319-322, 1966. 11. Statton, R., Biondi, F.e., and Hanigan, J.: Sarcoidosis of the optic nerve. Arch. Ophthalmol. 71: 834-836, 1964. 12. Morax, P.V.: Les localisations Neuro-oculaires de la Reticulo-endotheliose de Besnier-Boeck-Schaumann. Ann. Oculist. 189: 73-91, 1950. 13. Decker, R.E., Mardayat, M., Marc, J., and Rasool, A.: Neurosarcoidosis With computerized tomographic visualization and transphenoidal excision of a supra- and intrasellar granuloma. r Neurosurg. 50: 814-816, 1979. 14. McLaurin, E.B., and Harrington, D.O.: Intracranial sarcoidosis with optic tract and temporal lobe involvement. Am. r Ophthalmol. 86: 656-660, 1978. 15. Everts, W.H.: Sarcoidosis with brain tumor. Trans. Am. Neurol. Assoc. 72: 128, 1947. 16. Landers, P.H.: Vitreous lesion observed in Boeck's sarcoid. Am. r Ophthalmol. 32: 1740-1741, 1949. 17. Bruntse, E.: Ocular sarcoidosis. Danish Med. Bull. 5: 217-227,1958. 18. Blain, J.G., Ley, Riley, W., and Logothetis, J.: Optic nerve manifestations of sarcoidosis. Arch. Neurol. 13: 307-309, 1965. 19. Post, M.JD., Post, R.M., and Tabei, S.l.: CT demonstration of sarcoidosis of the optic nerve, frontal lobes, and falx cerebri: Case report and literature review. Am. r Neuroradiol. 3: 523-526, 1982. 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