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Show Journal of CliniCilI Neuro- ophlhalmology 10( 3): 188- 192, 1990. © 1990 Raven Press, Ltd..: York Pseudo- Foster Kennedy Syndrome in a Patient with Anterior Ischemic Optic Neuropathy and a Nonbasal Glioma Suresh R. Limaye, M. D., and Jonathan Adler, M. D. A 49- year- old woman with a 6- year history of headaches was found to have a pale right optic disc with narrowed retinal arterioles and a congested left optic disc. Her visual acuity was 20/ 20 in each eye with normal visual fields in May 1983. These findings were attributed to a previous attack of nonarteretic anterior ischemic optic neuropathy ( AlaN). She had a normal neurologic examination and a normal head computed tomographic ( eT) scan performed 2 years prior to her initial ophthalmologic evaluation. She was followed over the next 2 years without change in her fundus examination. In December 1987, after a generalized tonic- clonic seizure, she was found to have a large right frontoparietal mass without direct impingement on the optic nerves, or chiasm on neuroradiological studies. At this time she developed marked papilledema in the left eye with a pale optic disc in the right eye remaining unchanged. Histopathological diagnosis of malignant glioma was made. Two diseases, ischemic optic neuropathy and glioma, in one patient represents a bizzare example of the pseudo- Foster Kennedy syndrome. Key Words: Anosmia- Anterior ischemic optic neuropathy- Astrocytoma- Pseudo- Foster Kennedy syndrome- Oligogendroglioma. From Ophthalmology Service, District of Columbia General Hospital ( S. R. L.) and Center for Sight, Georgetown University Medical Center ( J. A.), Washington, DC. Address correspondence and reprint requests to Suresh R. Limaye, M. D., Chief, Ophthalmology Service, D. C. General Hospital, 19th Street and Massachusetts Avenue, S. E., Washington, DC 20003, U. S. A 188 In 1911, Foster Kennedy published six cases of expanding frontal lobe lesions, demonstrating ipsilateral optic atrophy with contralateral papilledema. He considered these signs pathognomonic for a space- occupying lesion in the region of the basofrontal area on the side of the optic atrophy ( 1). In a paper published in 1916, Foster Kennedy added ipsilateral anosmia to his previously described signs ( 2). The Foster Kennedy syndrome then became a triad consisting of ipsilateral optic atrophy, contralateral optic disc edema, and ipsilateral anosmia. Since these first descriptions, nontumor causes of ipsilateral disc pallor and contralateral disc edema have been reported, the most common of which is anterior ischemic optic neuropathy ( 3). Other nontumor causes of the Foster Kennedy syndrome include occult trauma, optic neuritis, syphilis, and severe arteriosclerosis of the internal carotid arteries ( 4). The noncompressive causes are termed pseudo- Foster Kennedy syndrome. Two diseases, ischemic optic neuropathy and meningioma, in one patient have been reported by Gelwan et al. ( 3) and termed the pseuopseudo- Foster Kennedy syndrome. Our case represents another example of two disease processes in one patient resulting in a pseudo- Foster Kennedy syndrome. CASE REPORT On May 21, 1983, a 44- year- old Bolivian woman presented to one of us ( S. R. L.) with complaints of tearing of the right eye for 2 years and difficulty reading small print. She was followed by a neurologist for a 2- year history of daily throbbing parietooccipital headaches. Her headaches were relieved by standing and by taking one to four acetamino- PSEUDO- FOSTER KENNEDY SYNDROME 189 phen tablets. The headaches were exacerbated by lying supine. Head computed tomographic ( CT) scan taken in 1981 was normal. Her headaches were considered to be muscular in nature, and her neurologic and ophthalmologic history was otherwise unremarkable. She had no other neurologic symptoms and her neurologic evaluation was normal. She had hypertension that was controlled by hydrochlorthiazide. On ocular examination, Snellen acuity without correction was 20/ 20 in both eyes. She required a + 1.25 add in both eyes to see Jaeger 1+. External examination revealed right epiphora with regurgitation on pressure over the right punctum. Lacrimal irrigation was performed and the nasolacrimal duct was found to be patent. The pupils were 4 mm in both eyes, reactive to light and accomodation, without an afferent pupillary defect. Extraocular muscles showed full range of motion with orthophoria in primary gaze. Slit lamp examination was normal with applanation tonometry readings of 16 mm Hg in both eyes. Ophthalmoscopic examination of the right eye showed a pale disc with narrowed arterioles and a normal macula; the left disc appeared slightly congested with normal macula, vessels, and periphery ( Fig. 1). Goldmann perimetry revealed full visual fields in both eyes without scotomata. The pale right disc with narrowed arterioles was thought to be due to an old ischemic event; that is, anterior ischemic optic neuropathy. The patient was prescribed Vasocon A ( IOLAB-- Pharmaceutical) for her right eye. The pa-tient was seen again in November 1984 for chronic allergic conjunctivitis with the remainder of the ophthalmic examination unchanged. She returned 1 year later for a follow- up examination without complaints. Her examination was unchanged. Fundus examination revealed a pale right optic disc with narrowed arterioles and a congested left optic disc with normal macula, and periphery in both eyes. Fundus photos were repeated ( Fig. 2). Goldmann kinetic perimetry showed a full field in the left eye and constriction to the 14e isopter in the right eye ( Fig. 3). The blind spots were enlarged in both visual fields. She continued to have headaches and her neurologic examination continued to be normal. On November 21, 1987, she experienced a generalized tonic- clonic seizure preceded by a darkening and blurring of the visual fields of both eyes. She was taken to a local hospital for evaluation. A review of systems showed an increase in the intensity of her headaches over the preceding 6 weeks. She had experienced a temporal visual field defect in the right eye and decrease in hearing of the right ear 2 weeks prior to the seizure. She also complained of bilateral lower extremity weakness for six weeks prior to the seizure. Her family reported that she had increased difficulty recalling recent events. Her neurologic examination revealed a visual field cut inferiorly in the right eye and decreased recall. She was noted to have optic atrophy in the right eye and papilledema in the left eye. Olfaction 1A B FIG. 1. Fundus photographs of May 21, 1983, showing pale disc with narrowed retinal arterioles in the ( A) right eye and congested disc in the ( 8) left eye with absence of cup. J Clin Neuro- ophthalmol, Vol. 10, No. 3, 1990 190 S. R. LIMAYE AND J. ADLER 2A B FIG. 2. After 30 months. pale disc with narrowed retinal arterioles in the ( A) right eye and congested disc in the ( 8) left eye remained unchanged. was intact bilaterally as tested with coffee. The remainder of the neurologic and physical examination was normal. Head CT and magnetic resonance imaging ( MRI) ( Fig. 4) revealed a mass in the right fronto- parietal region not directly involving the optic nerves or chiasm. There were no calcifications or hydrocephalus present. She was placed on 400 mg Dilantin ( Parke- Davis) q. h. s. and 4 rng Decadron ( Merck Sharp & Dohme) q. i. d. She refused further treatment at this time. The patient was then admitted on December 7, 1987, for further workup and treatment. Neurologic examination remained normal. An arteriogram revealed a large right fronto- parietal mass with tumor stain and a suggestion of small tumor vessels, consistent with a glioma. On December 12, a right frontal craniotomy was performed and the lesion was excised. It consisted mostly of oligodendroglioma with a foci of grade 2 astrocytoma ( Fig. 5). She had an umemarkable postoperative course and was discharged on 30 mg phenobarbital t. i. d. and 2 mg Decadron b. i. d. She received postoperative radiotherapy of 6,000 rad over a 6- '" IB ----',"-- "-. ' h, 1') 0. ''''''' I ... -- I 3A > CON \ \!- ," /:.:,./ .~ m -'/"<!--' c-.:~ S::-;"' 1' 1'.",' ~';...~ .:. ,. ~:~.. H; :-;' .-\-'-~/ , It • 2~! 1 110 ll~ ., J ,' I 0 IV " ': ~ ...::..;; ....;;;,:.:- .. ~.../ . -' .. ~ FIG. 3. Goldmann visual field of November 30. 1985. showing mild constriction to 14e isopter with enlarged blind spot in the ( A) right eye. full field with enlarged blind spot in the ( B) left eye. I Clin Neuro- ophthalmol, Vol. 10, No. 3, 1990 PSEUDO- FOSTER KENNEDY SYNDROME 191 4A s FIG. 4. A: MRI of November 1987 shows right frontoparietal tumor. B: Another view of MRI shows inhomogeneous signal of proton density with effacement of right sulci and leftward shift of third ventricle. Note that the right side of the optic chiasm is depressed downward by adjacent brain tissue ( arrow). week period. During her hospital course she was not examined by us or any other ophthalmologist. The patient presented for ophthalmologic evaluation in April 1988. Her examination revealed Snellen visual acuity of 20/ 20 in both eyes. Pupils were 4 mm in both eyes with equal reaction to light and accomodation without an afferent pupillary defect. Extraocular movements were full. Fundus exam revealed continued pallor of the right disc and edema with blurred disc margins of the left disc ( Fig. 6). DISCUSSION The Foster Kennedy syndrome is extremely rare. It was first described in association with frontal SA : 8 . FIG. 5. A: Astrocytoma component of the tumor consisting of small cell bodies, large nuclei, nuclear pleomorphism and hypercellularity without mitotic figures [ hematoxylin and eosin ( HE) x120j, B: Oligodendroglioma component composed of small nuclei, dense cytoplasm, hypercellularity, nuclear pleomorphism, and calcification ( HE x230). I Clin Neuro- ophtlullmol, Vol. 10. No. 3, 1990 192 S. R. LIMAYE AND]. ADLER SA B FIG. 6. A: Fundus photograph of May 7, 1988: optic atrophy in the right eye and papilledema in the B: left eye. lobe parenchymal tumors. Since the initial description there have been many noncompressive causes of ipsilateral disc pallor and contralateral disc edema reported. Causes include bilateral nonsimultaneous optic neuritis ( younger) or ischemia ( older), arachnoiditis ( 5), papilledema of any cause with an old optic neuropathy, and papillitis of any cause with an old optic neuropathy. The noncompressive causes are termed pseudo- Foster Kennedy syndrome. This hypertensive patient presented with a clinical picture of narrowed retinal arteries in her right eye, and a pale right disc. This was consistent with a previous attack of nonarteritic anterior ischemic optic neuropathy ( AION), especially since both discs appeared to be small in size without cups. Subsequently she developed rightfronto- parietal oligodendroglioma that reached sufficient size to act as a mass lesion and produced increased intracranial pressure and papilledema, but only in the contralateral eye. A pale optic nerve associated with 20/ 20 vision and a full visual field should have many nerve fibers left to swell with elevated intracranial pressure. It is believed, therefore, that the lack of swell-f Clin Neuro- aphtha/ mol, Vol. 10. No. 3. 1990 ing in the right optic disc was not due to atrophy but was caused by some anomaly of the anatomy and physiology that produces unilateral papilledema in otherwise normal patients with increased intracranial pressure. It has been speculated that a congenital nerve sheath anomaly may obstruct transmission of pressure such that the disc remains flat despite increased intracranial pressure. This patient had two precesses that subsequently led to a pseudo- Foster Kennedy syndrome. REFERENCES 1. Kennedy F. Retrobulbar neuritis as an exact diagnOStic sign of certain tumors and abscesses in the frontal lobes. Am I Med Sci 1911; 142: 355. 2. Kennedy F. A further note on the diagnostic value of retrobulbar neuritis in expanding lesions of the frontal lobes, with a report of this syndrome in a case of aneurysm of the right internal carotid artery. lAMA 1916; 67: 1361. 3. Gelwan J, Seidman M, Kupersmith MJ. Pseudo- pseudoFoster Kennedy syndrome. I Clin Neuro Ophthalmol1988; 8: 49- 52. 4. Schatz N}, Smith JL. Non- tumor causes of the Foster Kennedy syndrome. I Neurosurg 1967; 27: 37. 5. Gupta SR, Biller}, Frenkel M. Yarzagaray L, Fine M. Foster Kennedy syndrome due to Optochiasmatic arachroniditis. Surg Neurol 1983; 20: 216- 20. |
