Journal of Neuro-Ophthalmology, March 2014, Volume 34, Issue 1

Foster Kennedy Syndrome: Now and Then

Foster Kennedy Syndrome: Now and Then Ang-Ting Lai, Shin-Lin Chiu, I-Ching Lin, MD, Michael Sanders, FRCS, FRCP, FRCOpht Editor's Note: There are a number of clinical entities in neuro-ophthalmology having eponymous designations that are frequently discussed but rarely seen. One example is Argyll Robertson pupils. With the advent of improved methods to diagnose syphilis and effective antibiotic treat-ment, it is rare to detect this pupillary abnormality. Simi-larly, Foster Kennedy syndrome is well ensconced in the literature, and a topic we might use to test the fund of knowledge of our residents and fellows. Yet, this syndrome also is rarely seen clinically. No doubt that advances in neuroimaging have contributed to its infrequent appear-ance, with earlier detection of an intracranial mass lesion. What you will read here is a well-illustrated case of Foster Kennedy syndrome by Lai et al, and historical background of this neuro-ophthalmic syndrome by our esteemed British colleague, Michael Sanders. Being the purist, he prefers the term "sign" yet "syndrome" is more frequently used in the literature. In this article, the terms are used interchangeably. CASE REPORT A 36-year-old man reported blurring of vision in his left eye for 1 month and headache for 1 week. The headache was intermittent in the left frontal region and was not worsened by coughing, straining, or changing positions. It was not throbbing and was not associated with any aura. The patient did not have a history of headache or head trauma, and his medical and family histories were unremarkable. He denied anosmia. Visual acuity was 20/20, right eye, and 20/40, left eye. Color vision was intact, and there was a left relative afferent pupillary defect. The patient had normal eye movements. Visual fields showed an enlarged blind spot in the right eye and nasal field loss in the left eye. Slit-lamp examination was normal with normal intraocular pressures. Funduscopic examination demonstrated right optic disc edema and left optic disc pallor (Fig. 1). Magnetic resonance imaging of the brain revealed a left sphenoid wing meningioma, with compression of the left optic nerve (Fig. 2). The patient was treated with preoperative embolization of the tumor via the left middle meningeal artery, followed by a left frontotemporal craniotomy. Histo-pathologic examination of the tumor was consistent with a transitional meningioma (WHO grade I). The patient made an uneventful postoperative recovery and, at 6 months of follow-up, his visual acuity remained unchanged. His right optic disc edema and headache resolved. DISCUSSION Neurologists have always embellished the medical establish-ment, and Foster Kennedy was no exception. He apparently traveled round New York from patient to patient wearing a flowing brown coat and a black homburg in a handsome carriage with fine horses. An engaging, charming, and humorous person, he loved and excelled in his chosen speciality and mixed with the rich and distinguished both as friends and patients. Robert Foster Kennedy was born in Belfast, educated at Queens College, and qualified in medicine in 1906. He joined the junior staff of the National Hospital for the paralyzed and epileptic in 1907. The Hospital, founded in 1860, was one of the earliest neurological institutes and rapidly established a global reputation on account of its outstanding medical staff. Foster Kennedy worked with Sir William Gowers, Henry Head, and Sir Victor Horsley. He attended the Neurological Meeting where he met Sir William Osler (Regius Professor of Medicine at Oxford) and younger neurologists such as Gordon Holmes and Kinnier Wilson. During his 4-year tenure as medical officer, he gained immense experience, sometimes having to write daily notes on 85 patients, and caring for 13 cases of cerebral tumor. He debated with his seniors on diagnostic FIG. 1. The right optic disc is swollen and the left optic disc has temporal pallor. Departments of Family Medicine (A-TL, I-CL) and Ophthalmology (S-LC), Changhua Christian Hospital, Changhua, Taiwan; and Department of Neuro-Ophthalmology (MS), National Hospital for Neurology and Neurosurgery, London, England, United Kingdom. The authors report no conflicts of interest. Address correspondence to I-Ching Lin, MD, Department of Family Medicine, Changhua Christian Hospital, Changhua, Taiwan; E-mail: [e-mail] 92 Lai et al: J Neuro-Ophthalmol 2014; 34: 92-94 Historical Note Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. accuracy, and when he correctly diagnosed a cerebral abscess, he left Horsley humbled. On 1 occasion, he wrote to his fiancée on Horsley, "the operation was mechanically exquisite and most thoroughly unjustified after full consid-eration of the facts." Even at an early stage of his medical career, he showed a strong independence of mind accom-panied by sound judgment. Kennedy met Royalty when King Edward VII visited the Hospital, and he managed to knock over a bowl of flowers on the King's boots. "Don't worry" proclaimed the mon-arch, "they are cleaned almost every day." He wrote fre-quent letters to Isabel McCann, his fiancée, and signed them off with "your boy." They provide a vivid picture of life and medicine at the turn of the 20th century and have been published by his daughter who married a distinguished British medical academic (1). In 1910, Kennedy left the National, and despite the endeavors of his eminent teachers, he failed to find a post in the United Kingdom. He accepted an appointment at the recently formed Neurological Institute in New York. This institute, the first of its kind in America, floundered initially but then became part of the Columbia-Presbyterian med-ical complex. In 1911, he published an article that was to establish his international reputation (2). This will be dis-cussed later. The rumblings of war erupted in 1914, and Kennedy offered his services. As a Lieutenant in the Royal Army Medical Corps, he was asked to set up a hospital in France. During this period, he also joined the Harvard Surgical Unit, which had been established by Harvey Cushing. His war experiences, including the battles of the Somme and Ypres, left a deep impression on him. Returning to New York in 1919, he was appointed as professor of neurology at the Bellevue Hospital, which was the first public hospital in the United States and linked to Cornell and Columbia Universities. As time progressed, he reached the apex of his career with patients and friends from the heights of business and the arts, and a serene domestic environment (Fig. 3). He examined Winston Churchill after he had been hit by a taxi on Fifth Avenue and remained in contact with him. A dinner at his home with Churchill and one other started with Liebfraumilch at 8:30 PM and finished with stronger "ingredients" at 4:00 AM He also treated and knew the President, Franklin Delano Roosevelt. In the early 20th century, 50 years after the advent of the ophthalmoscope, clinicians were attempting to relate visual acuity and optic disc appearances to etiology and, in the process, to construct meaningful diagnostic terminology. Therefore, the article Kennedy published in 1911, in which a patient had optic disc swelling on one side with optic atrophy, on the other in association with a frontal tumor provided a good diagnostic platform (2). Miss Cameron, a 37-year-old Scottish woman, was referred to Foster Kennedy when he was Medical Officer to Sir William Gowers by the ophthalmologist, Marcus Gunn. She had a long history of progressive headaches, fainting attacks, and ultimately vomiting. She had obscurations of FIG. 2. Precontrast T1 axial (A) and coronal (B) magnetic resonance imaging shows a large tumor mass causing left to right shift with ventricular obstruction and compression of the prechiasmal portion of the left optic nerve (arrow). C. Postcontrast T1 axial scan reveals tumor enhancement and left optic nerve compression (arrow). FIG. 3. Foster Kennedy as a young man (left) and in his prime in New York City (right). Lai et al: J Neuro-Ophthalmol 2014; 34: 92-94 93 Historical Note Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. vision in the eye with better visual acuity, described in the right eye by Gowers as "paroxysm of dimness," although Foster Kennedy attributed this to the left eye. Vision was perception of light in the left eye with a pale disc, and 6/6 in the right eye with papilledema (measured as 1.5 mm by Gunn). There was an afferent pupillary defect on the left with a central scotoma. Anosmia was present in the left nostril, and although initially normal on the right, developed later. On October 13, 1908, Sir Victor Horsley loosened the frontal bone and then replaced it, and although the patient briefly regained consciousness, she swiftly passed away. Postmortem examination showed a large olfactory groove meningioma more on the left than on the right. The optic nerve sheath was distended distally on the right side but was normal on the left side, which showed a greater degree of compression. The olfactory nerve on the left had "almost disappeared." The clinicians were pleased with their localization, and Sir William Gowers had never seen such a case. This led him to give a lecture on this case at the University College Hospital, and this was published 8 months later in the Lancet with a photograph (Fig. 4) and discussion of the pathology (3). Gowers used the term optic neuritis for the swollen disc and entitled the article "A case of unilateral optic neuritis from intracranial tumour." Foster Kennedy used the same case in his publication (2) but added 5 similar cases of frontal lesions and indicated that these clinical features were a "sign" of a frontal lesion. His series could be divided into 3 groups: Type 1: optic atrophy on one side with contralateral papilledema Type 2: bilateral papilledema developing unilateral optic atrophy Type 3: bilateral papilledema developing bilateral optic atrophy. In only 2 eyes was good vision retained, and in the remaining 10 eyes, vision was 3/36 or worse. Anosmia was present in 5 cases. Thus, the commonest feature of these large frontal masses was bilateral papilledema, progressing to atrophy on one or both sides. In contrast, in 1972, I reviewed 6 cases of Foster Kennedy sign and found 3 cases of type 1, and 1 case each of types 2 and 3 (4). This heterogenous group included no cases of olfactory groove meningioma but consisted of 2 frontal glio-mas, 1 falx meningioma, 1 sphenoid ridge meningioma, and 1 cavernous sinus meningioma. The sixth case was caused by a cholesteatoma, which conformed more to the type described by Schultz-Zehden in 1905 (5), in which a mass below the chiasm produces asymmetric compression of the optic nerves. Anosmia was absent in all cases. In a review by Watnick and Trobe (6), the rarity of the "sign" was empha-sized: out of 36 cases in the literature, only 8 (22%) had the true Foster Kennedy sign. Today, any patient complaining of progressive headaches or visual loss would undergo neuroimaging, leading to early detection of intracranial lesions. In part, this may contribute to the rarity of the Foster Kennedy sign. For the purist, the Foster Kennedy sign represents optic atrophy and anosmia on one side, with papilledema caused by raised intracranial pressure on the other. However, the majority of cases with these findings occur without anosmia and are due to a variety of intracranial disorders. In Gowers' day, the clinician localized the lesion. Today, the neuroradiologist does it for him! ACKNOWLEDGMENTS The authors thank the Department of Medical Illustration at the National Hospital that provided Figures 3 and 4. REFERENCES 1. Butterfield IK. The Making of a Neurologist. Hertfordshire, England: The Stellar Press, 1981. 2. Kennedy F. Retrobulbar neuritis as an exact diagnostic sign of tumours and abscesses in the frontal lobes. Am J Med Sci. 1911;142:355-368. 3. Gowers WR. A lecture on a case of unilateral optic neuritis from intracranial tumour. Lancet. 1909;2:65-68. 4. Sanders MD. The Foster Kennedy sign. Proc Roy Soc Med. 1972;65:520-521. 5. Schultz-Zehden P. Ein Beitrag zur Kenninis der Genese einseitiger Stauungspapille. Klin Monatsbl Augenheilkd. 1905;43:152-156. 6. Watnick RL, Trobe JD. Bilateral optic nerve compression as a mechanism for the Foster Kennedy syndrome. Opthalmology. 1989;96:1793-1798. FIG. 4. Photograph of Gowers original case with a large olfactory groove meningioma. 94 Lai et al: J Neuro-Ophthalmol 2014; 34: 92-94 Historical Note Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.