| OCR Text |
Show JOl/ TIlal of Climeal NCl/ fo- ophthalmology 8( 1): 49- 52. 1988. Pseudo- Pseudo- Foster Kennedy Syndrome Mark J. Gelwan, M. D., Mitchell Seidman, M, D., and Mark J. Kupersrnith, M, D, .(: ] 988 Raven Press, Ltd., New York An SO- year- old woman presented with a classic story and findings of an anterior ischemic optic neuropathy in her left eye. Her right eye had slow and progressive decreased vision, ostensibly secondary to a cataract. However, the right eye showed slight temporal pallor of the optic disc and a superior temporal field defect was found. Her radiologic exam showed a tuberculum sella meningioma extending into the right optic canal compressing the right optic nerve. Two diseases, ischemic optic neuropathy and meningioma, in one patient may be termed a pseudo- pseudo- Foster Kennedy syndrome. Key Words: Meningioma- Foster Kennedy syndrome - Ischemic optic neuropathy. From the Departments of Ophthalmology ( M. J. G., M. S., M. J. K.) and Neurology ( M. J. K.), New York University Medical Center, New York, New York. Address correspondence and reprint requests to Dr. M. J. Kupersmith. 530 First Ave, Suite 36, New York, NY 10016, USA. 49 The syndrome of optic atrophy in one eye and optic nerve swelling in the other eye can be caused by a tumor growing at the base of the frontal lobes. This was first pointed out by Paton ( 1) and was further elaborated on by Foster Kennedy ( 2). Since these first descriptions, nontumor causes of similar syndromes have been reported, the most common of which is anterior ischemic optic neuropathy. An acute event occurs in the eye with the swollen optic disc. A history of a prior episode of visual loss in the other eye with optic atrophy is elicited. Other reported causes include occult trauma, optic neuritis, syphilis, and severe arteriosclerosis of the internal carotid arteries causing compression of the optic nerves by the carotid arteries ( 3- 5). The noncompressive causes have been termed the pseudo- Foster Kennedy syndrome. Our case paints a similar picture, but careful clinical evaluation with appropriate radiologic examination indicated two diseases in one patient. We call this a pseudo- pseudo- Foster Kennedy syndrome. Case Presentation An 80- year- old white woman presented to New York University Medical Center complaining of a precipitous decrease in vision in her left eye. She had no associated scalp tenderness, ocul~ r pain, or jaw claudication. She had no other common neurological complaints. She indicated that the vision in her right eye was diminished over the past few years. She was known to have a cataract in her right eye. Her past neurologic and ophthalmologic history were otherwise unremarkable. Her medical history was noncontributory. On physical exam, her corrected ' Snellen acuity was 201100 in the right eye and 10/ 400 in the left eye, with eccentric fixation in the left eye. She saw only 1 Ishihara color plate with the right eye and none with the left eye. Tangent screen perimetry 50 NAME, ' 0' M. f. CELWAN ET AL. DATE ' 0' TARGET SIZE TAIl: GET COLOR TESTING 015TANCE FIG. 1. The right eye shows a superotemporal visual field defect to a 2 mm white ( solid line) and 5 mm white ( dashed line) objects. The left eye shows a superior altitudinal defect with central scotoma to a 10 mm white object. ( Fig. 1) at 1 m revealed a superior temporal field depression to a 3 mm and 5 mm white test object in the right eye. In the left eye, fixation was poor, but it showed a superior altitudinal and central scotoma field defect to a 10 mm white test object. Only a small area of inferior nasal field remained to a 5 mm white test object. The pupils were 5 mm in both eyes, reactive to light and accommodation, with a marked afferent pupillary defect in the left eye. The right fundus, viewed through a nuclear sclerotic cataract was not clear and showed an optic disc with a 0.2 cup and mild temporal pallor. The nerve fiber layer was suggestive of thinning in the papillomacular bundle. The retinal vessels, macula, and retinal periphery were normal. The left eye showed an optic disc with no physiologic cup, diffuse elevation, splinter hemorrhages, and gray yellow discoloration of the disc inferiorly. The arterioles were slightly narrowed. The macula and the remainder of the retinal exam was normal. FIG. 2. Contrast enhanced CT scan: A mass is seen in the area of the planum sphenoidale on the right side ( arrow), The borders are irregular, and there is slight enhancement of the lesion. PSEUDO- PSEUDO- FOSTER KENNEDY SYNDROME 51 FIG. 3. MRI: T1 sagittal view shows an isodense lesion ( arrow) in the area of the planum sphenoidale. The slit lamp exam was normal except for a 3+ nuclear sclerosis in the right eye and 2+ nuclear sclerosis in the left eye. The remainder of her neurologic evaluation was normal. The hemogram, VORL, FTA, ANA, latex fixation, and chest x- ray were normal. Her sedimentation rate was 30. A computed tomography ( CT) scan of the head with intravenous contrast showed a small rounded enhancing mass arising near the medial sphenoid wing tuberculum, primarily on the right side. The right optic nerve appeared involved as it passed intracranially through the optic canals. The lesion extended beneath the right anterior clinoid ( Fig. 2). A magnetic resonance imaging ( MRI) of the head showed a low signal intensity lesion, on T1 and T2 spin resonance images, in the same location as on CT scan ( Fig. 3). A presumptive diagnosis of meningioma was made. Radiotherapy ( RT) of 5,500 rads was delivered to the tumor over a 6 week period. Near the completion of RT, she noted improvement in the vision and her acuity in the right eye was 20170 + . She was able to see 4/ 10 color plates in the right eye and her tangent field perimetry showed less depression of the temporal field. The left eye was unchanged except the disc was flat and atrophic. DISCUSSION This elderly lady presented with a classic history and clinical findings of a non- arteritic anterior ischemic optic neuropathy in the left eye. She had a cataract in the right eye, with acuity of only 201100, worse than expected from the cataract alone. The right eye optic nerve was pale temporally with corresponding nerve fiber layer loss. If one evokes a single disease process, the well- reported pseUdo- Foster Kennedy syndrome comes to mind. This syndrome is often due to bilateral nonarteritic anterior ischemic optic neuropathy: swollen disc- new, pale disc- old. The importance of this case presentation lies in the clinical history and findings concerning the visualloss in the right eye. The patient's story is of a chronic, mildly progressive visual loss in the right eye, not an acute event as occurred in the left eye. The atrophy of the disc without narrowing of the arterioles in typical of a compressive rather than an ischemic lesion ( 3). Most importantly, the superior temporal field depression is typical of intracranial anterior visual pathway compression. These findings led to a radiologic evaluation that showed that the patient's decreased vision in the I Oi" Neuro · ophthalmol. Vol. 8, No. 1. 1988 52 M. J. GELWAN ET AL. right eye was due to a tuberculum sellae meningioma compression of the right optic nerve. The meningioma was treated with radiotherapy because of the patient's age, and a good short- term result was seen ( 6). The patient's problem in the left eye was a true nonarteritic anterior ischemic optic neuropathy, unrelated to the problem in the right eye. It is the philosophy of medicine to unite all the clinical findings as being indicative of one pathological entity. This is not always the case as demonstrated by our pseudo- pseudo Foster Kennedy syndrome. REFERENCES 1. Paton L. A clinical study of optic neuritis in its relationship to intracranial tumors. 8mill 1909; 32: 68- 91. 2. Kennedy F. Retrobulbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Am J Med Sci 1911; 142: 355- 68. 3. Schatz NJ, Smith JL. Non- tumor causes of Foster Kennedy syndrome. , NellroslIrg 1967; 27: 37- 44. 4. Yaskin HE, Schlezinger NS. Foster Kennedy syndrome associated with non- neoplastic intracranial conditions. Arch Ophtha/ l/ l0/ 1949; 28: 704- 10. 5. Yaskin HE, Alpers BJ. Foster Kennedy syndrome with post- traumatic arachnoiditis of the optic chiasm and base of the frontal lobes. Arch Ophtha/ mol 1945; 34: 399- 401. 6. Kupersmith MJ, Warren FA, Newall J, et al. Irradiation of meningiomas of the intracranial anterior visual pathway. AIIII Nellro/ 1987; 2l: 131- 7. |
