INTRACRANIAL SARCOIDOSIS W I T H OPTIC TRACT AND TEMPORAL LOBE INVOLVEMENT E U G E N E B. M C L A U R I N , Oakland, M.D. California AND D A V I D O. H A R R I N G T O N , San Francisco, Sarcoidosis, a common idiopathic granulomatous disease, has been known for more than 70 years to affect the central nervous system. 1 Over the past two dec­ ades, neurologic sarcoidosis has been rec­ ognized with increasing frequency, 2 and the incidence of the neurologic manifes­ tations of the disease has been reported to be from 1% 3 to a high of 29%. 4 The overall frequency of neurologic sarcoidosis is about 5%. 5 We report herein a unique case which demonstrates a right homonymous hemianopic visual field defect, radiographic evidence of intracranial sarcoid lesion by computed axial tomography, radiographic signs of pulmonary sarcoidosis by chest x-rays, and a positive pulmonary hilar lymph node for sarcoid. Treatment with large doses of prednisone resulted in symptomatic and radiographic improve­ ment, and partial resolution of the visual field defects. M.D. California blurring of vision in both eyes. Examination re­ vealed corrected visual acuity of 6/9 (20/30) in each eye, pupils were equal, round, and reactive to light, there was no Marcus Gunn pupil, and muscle move­ ments were normal. Intraocular pressure by applanation was 12 mm Hg in the right eye and 15 mm Hg in the left eye. Both eyes showed moderate flare and cells in the anterior chamber. Anteroposterior and lateral chest x-rays revealed marked bilateral hilar and paratracheal adenopathy (Fig. 1), which is char­ acteristic of pulmonary sarcoidosis. There were no pulmonary symptoms. Diagnosis was uveitis associ­ ated with pulmonary sarcoidosis. On June 6, 1977, the patient returned complaining of photophobia and decreased vision. Corrected visual acuity was R.E.: 6/12 (20/40) and L.E.: 6/15 (20/50). Visual fields were done by the confrontation method and were normal. Several mutton-fat keratic CASE REPORT A 23-year-old man was examined initially on April 4, 1976, with a one-month history of pain and From the Department of Ophthalmology, Univer­ sity of California, San Francisco, California (Dr. Harrington), and the Naval Regional Medical Center, Oakland, California (Dr. McLaurin). The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Navy or the Department of Defense. Reprint requests to Eugene B. McLaurin, M.D., Department of Ophthalmology, Naval Regional Medical Center, Oakland, CA 94627. 656 Fig. 1 (McLaurin and Harrington). Chest x-ray revealing bilateral hilar and paratracheal adenopa­ thy typical of pulmonary sarcoidosis. AMERICAN JOURNAL O F OPHTHALMOLOGY 86:656-660, 1978 VOL. 86, NO. 5 INTRACRANIAL SARCOIDOSIS 657 Fig. 2 (McLaurin and Harrington). Visual fields showing an incongruous homonymous hemianopia characteristic of optic tract and temporal lobe lesions. precipitates were present in both eyes. The patient's uveitis responded to treatment with topical prednisone and atropine. On Aug. 23, 1977, the patient reported a two-week period of blurred vision in the right eye. Visual acuity when corrected was reduced to 6/60 (20/200) in the right eye and 6/18 (20/60) in the left eye. Intraocular pressure measured 13 mm Hg in each eye. The anterior chamber was clear and the results of ophthalmoscopic examination were normal. Visual field studies on the tangent screen revealed a mark­ edly incongruous right homonymous superior quadrantanopia with sloping margins, even to large stimuli (Fig. 2). Pupils were equal and round, reac­ tive to light, and there was no Marcus Gunn pupil. Except for the visual field defect, the results of a neurologic examination were normal and there were no abnormalities evident on the anteroposterior, lateral, and stereoscopic views of the skull x-rays. The anteroposterior and lateral chest x-rays had not changed from those of April 1976. Computed axial tomography indicated a large lesion in the suprasellar cistern with posterior exten­ sion into the medial portion of the left temporal lobe, along the left optic tract (Fig. 3). The patient was hospitalized for further studies including a search for other neurologic defects. Blood studies were all normal including calcium, phosphorous, alkaline phosphatase, glucose, serum glutamic oxaloacetic transaminase, and lactate dehydrogenase. The patient refused to allow a lumbar puncture to be performed. Pulmonary function tests revealed a mild pulmonary obstructive disease consistent with that of a smoker. The patient smoked a pack of cigarettes a day. He was discharged from the hospi­ tal with a diagnosis of pulmonary sarcoidosis and intracranial involvement. Treatment consisted of 80 mg of oral prednisone daily, and at two-week inter­ vals a complete ophthalmic examination, including visual field studies, was performed. By Oct. 3 1 , 1977, there was marked improvement in the right superior homonymous quadrantanopia with widening of the area of the macular sparing and decrease in the incongruity of the defect (Fig. 4). Corrected visual acuity improved to 6/19 (20/30) in each eye. Repeat computed axial tomog­ raphy showed complete resolution of the left tem­ poral lobe lesion and the suprasellar mass had disappeared, except for a small focus of increased attenuation just lateral to the third ventrical (Fig. 5). The anteroposterior and lateral chest x-rays showed a decrease in the pulmonary lesions (Fig. 6). At this point the patient received 40 mg of oral prednisone every other day, which was slowly decreased over a two-month period of time. The patient was admitted to the hospital on April 3, 1978, for mediastinoscopy to establish a tissue diagnosis of sarcoidosis. Biopsies were done on several mediastinal lymph nodes, and the frozen and permanent section was consistent with sarcoidosis. 658 AMERICAN JOURNAL OF OPHTHALMOLOGY Fig. 3 (McLaurin and Harrington). Computed axial tomography showing large lesion in the suprasellar cistern with extension into the left temporal lobe along the left optic tract. DISCUSSION The central nervous system is not a common site for lesions of sarcoidosis, NOVEMBER, 1978 but Delaney, 5 recently reviewed over 400 cases which had a 5% overall frequency of neurologic involvement. Most patients were between 20 and 40 years old, and racial distribution was primarily black. Central nervous sytem involvement was evident early in the disease, and the pe­ ripheral nervous system and skeletal mus­ culature were involved in the late or chronic stages. About 20 cases of sarcoid were initially diagnosed as brain tu­ mors. 6 - 1 1 A radioisotope study done by Rosen and Wang 9 included a patient with intracranial and pulmonary involvement of sarcoid that improved with corticosteroid therapy. Visual disturbances with sarcoidosis are usually related to uveitis. Visual field defects have rarely been reported. Law­ rence 2 described a patient with a left homonymous superior quadrantanopia with subsequent visual field contraction caused by sarcoid. A biopsy of the right optic nerve was consistent with sarcoid. Another patient 8 had a right homonymous Fig. 4 (McLaurin and Harrington). Improvement in the homonymous hemianopic visual field defect after prednisone therapy. VOL. 86, NO. 5 INTRACRANIAL SARCOIDOSIS Fig. 5 (McLaurin and Harrington). Computed axial tomography after prednisone therapy showing almost complete resolution of the left temporal lesion and the suprasellar mass demonstrated in Figure 3. 659 with bitemporal hemianopia and x-ray evidence of changes in the sella turcica. Intracranial sarcoid lesions are most commonly found in the hypothalamus, the region of the third ventricle, and the pituitary gland. 6 * 13 The normal sarcoid granuloma rarely shows calcification in x-ray studies of the skull. 14 With corticosteriod therapy, the disease may remain in remission for considerable periods of time, but usually recurs when the dosage is decreased. Several of Delaney's 5 patients showed dramatic im­ provement in their neurologic deficits while under treatment. Other patients re­ sponded poorly or not at all. In view of the serious neurologic in­ volvement, we think all patients with intracranial sarcoidosis should receive in­ tensive corticosteroid therapy. SUMMARY hemianopia caused by a large sarcoid lesion in the left occipital lobe. A third patient 1 2 had involvement of the chiasm Intracranial sarcoidosis was diagnosed in a 23-year-old man when he developed a markedly incongruous right homonymous hemianopic visual field defect. The diagnosis was substantiated by radiologic evidence of bilateral hilar and paratracheal adenopathy, typical of pulmonary sarcoidosis. Subsequently, computed axial tomography indicated a large intra­ cranial lesion in the suprasellar cistern with extension into the left temporal lobe along the optic tract. A mediastinal biop­ sy of the lymph nodes was positive for sarcoid. Intensive corticosteroid therapy resulted in restoration of vision, marked improvement of the visual field defect, decrease in the pulmonary hilar adenopa­ thy, and almost complete resolution of the left temporal lobe lesion. REFERENCES Fig. 6 (McLaurin and Harrington). Chest x-ray showing decrease in the pulmonary lesions shown in Figure 1. 1. Jefferson, M.: Sarcoidosis of the nervous sys­ tem. Brain 80:540, 1957. 2. Lawrence, W. P., Gammal, T. E., Pool, W. H., and Apter, L.: Radiological manifestations of neurosarcoidosis. Report of three cases and review of the literature. Clin. Radiol. 25:343, 1974. 660 AMERICAN JOURNAL OF OPHTHALMOLOGY 3. Silverstein, A., Feuer, M., and Siltzbach, L. E.: Neurologic sarcoidosis. Study of 18 cases. Arch. Neurol. 12:1, 1965. 4. Gendel, B.: Sarcoidosis. Review with 24 addi­ tional cases. Am. J. Med. 12:205, 1952. 5. Delaney, P.: Neurologic manifestations in sar­ coidosis. Ann. Intern. Med. 87:336, 1977. 6. Aszkanazy, C. L.: Sarcoidosis of the central nervous system. J. Neuropathol. Exp. Neurol. 11: 392, 1952. 7. Popper, J. S., Bingham, W. G., and Armstrong, F. S.: Sarcoid granuloma of the cerebellum. Neurol­ ogy Minneap. 10:942, 160. 8. Everts, W, H.: Sarcoidosis with brain tumor. 7 Trans. Am. Neurol. As 2:128, 1947. 9. Rosen, J. A., an i g, Y.: Central nervous NOVEMBER, 1978 system sarcoid granuloma monitored by brain scan­ ning. Arch. Intern. Med. 115:336, 1965. 10. Skillicorn, S. A., and Garrity, R. W.: Intracranial Boeck's sarcoid tumor resembling meningioma. J. Neurosurg. 12:407, 1955. 11. Goodman, S. S., and Margulies, M. E.: Boeck's sarcoid simulating a brain tumor. Arch. Neurol. Psychiatr. 81:419, 1959. 12. Coleman, C. C., and Meredith, J. M.: Diffuse tuberculosis of pituitary gland simulating tumor with postoperative recovery. Arch. Neurol. Psychiatr. 44:1076, 1940. 13. Urich, H.: Neurological manifestations of sar­ coidosis. Practitioner 202:632, 1969. 14. Scadding, J. G.: Calcification in sarcoidosis. Tubercle 42:121, 1961. O P H T H A L M I C MINIATURE . . . He was plainly blind, for he tapped before him with a stick, and wore a great green shade over his eyes and nose; and he was hunched, as if with age or weakness, and wore a huge old tattered sea-cloak with a hood that made him appear positively deformed. I never saw in my life a more dreadful-looking figure. He stopped a little from the inn and, raising his voice in an old sing-song, addressed the air in front of him: "Will any kind friend inform a poor blind man, who has lost the precious sight of his eyes in the gracious defense of his native country, England, and God bless King George!—where or in what part of this country he may now b e ? " Robert Louis Stevenson Treasure Island