Giant cell (temporal) arteritis. The differential diagnosis

SURVEY OF OPHTHALMOLOGY

VOLUME 23- NUMBER 4-JANUARY-FEBRUARY 1979

CLINICAL CHALLENGES PAUL HENKIND, EDITOR

Giant Cell (Temporal) Arteritis . The Differential Diagnosis NEIL R . MILLER, M .D., JOHN L. KELTNER, M .D ., JOHN W . GITTINGER, M.D ., AND RONALD M. BURDE, M .D. The Wilmer Ophthalmological Institute, Baltimore, Maryland, Department of Ophthalmology, University of California at Davis, Sacramento, California, Department of Ophthalmology, Tufts-New England Medical Center, Boston, Massachusetts, and Department of Ophthalmology, Washington University Medical Center, St . Louis, Missouri

Abstract . A patient was referred to the neuro-ophthalmology unit with a diagnosis of Foster-Kennedy syndrome ; "papilledema" had been noted in the right eye and optic atrophy in the left . Results of radiographic examinations and lumbar puncture had been normal . The considerations and procedures leading to a correct diagnosis of giant cell arteritis are discussed . (Sun Ophthalmol 23 :259-263, 1979) words. Foster-Kennedy syndrome • giant cell arteritis • ischemic optic neuropathy • papilledema • temporal arteritis Key

Case report : Neil R . Miller, M .D ., The Wilmer Ophthalmological Institute .

65-year-old white man was referred to the Neuro-Ophthalmology Unit of Johns Hopkins Hospital with a diagnosis of Foster-Kennedy syndrome . The patient's past medical history was positive for mild hypertension . Three months previously, the patient had noted a severe decrease in the vision of his left eye but had not sought medical attention . One month previously, he noted the sudden onset of blurred vision in his right eye . He had been seen by an ophthalmologist who noted "papilledema" in his right eye and optic atrophy in his left eye . The patient was admitted to another hospital where skull x-rays, computerized tomography, cerebral angiography and lum-

A

bar puncture were performed . All studies were within normal limits . The patient was then referred for further evaluation . On examination, the patient's visual acuity was 20/50 in the right eye and 20/400 in the left eye . There was a left afferent pupillary defect . Slitlamp examination and intraocular pressures were within normal limits . Visual fields were as shown in Fig . I . Ophthalmoscopic examination showed an edematous, pale right optic disc with several peripapillary hemorrhages (Fig, 2) . The left optic disc was uniformly pale (Fig . 3) . Subsequent history disclosed that the patient suffered from episodes of sharp, temporal headaches and occasionally developed an aching sensation during mastication . What is your differential diagnosis?

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V4E also f

LEFT

RIGHT

FIG. 1 . Right and left visual fields demonstrating bilateral inferior altitudinal field defects involving fixation .

Comments Comments of John L. Keltner, M .D ., University of California at Davis .

Ftc . 2 . Right optic nerve head demonstrating pallid disc edema with superficial splinter hemorrhages .

FIG. 3 .

Atrophic left optic disc.

The patient described in Dr . Miller's case was initially thought to have a FosterKennedy syndrome - probably one of the most over-diagnosed syndromes in neuroophthalmology . The most common cause of the Foster-Kennedy syndrome is the so-called pseudo-Foster Kennedy syndrome, which is secondary to ischemic optic neuropathy . Thus, a patient has an attack of ischemic optic neuropathy in one eye and several months or years later has an attack in the second eye . The ophthalmologist, seeing a swollen disc in one eye and optic atrophy in the other eye, may misdiagnose the condition as a FosterKennedy syndrome . The true Foster-Kennedy syndrome is characterized by a tumor which causes direct pressure on one optic nerve, producing optic atrophy and a choked disc on the opposite side secondary to increased intracranial pressure . The causes of the true Foster-Kennedy syndrome have been olfactory groove meningiomas, sphenoid wing meningiomas, frontal lobe tumors, and an assortment of other non-tumorous conditions . However, the true Foster-Kennedy syndrome is very rare . The patient presented by Dr . Miller represents a rather typical case of giant cell arteritis . First, the patient had suffered from episodes of headache and aching sensation during mastication . As was pointed out in a

CLINICAL CHALLENGES

recent review article by Huston,' in a 25-year epidemiologic study of temporal arteritis, over 90% of patients had headache as a presenting symptom . Jaw claudication, as evidenced in this patient, was a symptom in 69% of patients . Any patient over 60 years of age who presents with sudden onset of visual loss in one eye should be questioned carefully regarding the history of headache, tender temporal arteries, claudication of the jaw, weight loss, and polymyalgia rheumatica . Once the diagnosis is suspected, a sedimentation rate should be performed, and the patient immediately started on prednisone in high doses to prevent visual loss in the second eye . A temporal artery biopsy can be performed at a later date . Corticosteroids given for a short time will not affect the biopsy . A long biopsy (3 to 5 cm) with multiple histologic sections will minimize "skip areas ."' , " If Dr . Miller's patient did not have temporal arteritis, the most likely cause for his problem would have been arteriosclerotic ischemic optic neuropathy . With arteriosclerotic ischemic optic neuropathy the patients lack headache and other symptoms of temporal arteritis, and have normal sedimentation rates . The patient's altitudinal field loss characterizes the typical field loss as seen in ischemic optic neuropathy . Most patients with Foster-Kennedy syndrome have bizarre field defects, rather than sharp altitudinal field defects as seen in this patient . The patient's visual loss in his second eye is characteristic of patients with temporal arteritis . According to previous authors,2 ' 8 "'a the chance of blindness is greatest in the first 1-10 days, and the patient is still at great risk for blindness in the second eye for 6-8 weeks after involvement of the first eye . In summary, pseudo-Foster-Kennedy syndrome is caused by either ischemic optic neuropathy or temporal arteritis, and is a fairly frequent neuro-ophthalmologic condition which is often misdiagnosed . The patient is often inappropriately evaluated, giant cell arteritis is not considered early in the course of the illness, and the patient develops severe visual loss in the second eye . Unfortunately, while the ophthalmologist may be better educated about this condition than is the internist or general practitioner, these patients are often not seen by the ophthalmologist until they have had attacks of blindness in one or both eyes .

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Comments of John W. Gittinger, M .D ., Tufts-New England Medical Center .

In 19) 1, Kennedy described six patients with optic atrophy and central scotomata in one eye, papilledema in the opposite eye, and neoplasms or abscesses in the frontal lobe on the side of the optic atrophy .' Ipsilateral optic atrophy and contralateral papilledema has been well-imbedded in the medical mind as the Foster-Kennedy syndrome . Unfortunately, as Dr . Miller's case exemplifies, this combination of findings is not specific to basal frontal tumors . Schatz and Smith" called attention to a series of 169 cases of the Foster-Kennedy syndrome collected by Francois and Neetens, 32% of which were not caused by tumor and an additional 17% of which were the result of tumors not located in the frontal area . Schatz and Smith" found that, in their own experience, bilateral ischemic optic neuropathy with the events in the two eyes separated by time was the most frequent clinical condition confused with Foster-Kennedy syndrome due to tumor . In the present case, the examination of the eye with the swollen disc allows the distinction from true Foster-Kennedy syndrome to be made . In this eye the presence of decreased acuity and an altitudinal field defect is strong evidence of ischemic optic neuropathy . Papilledema from increased intracranial pressure of recent onset produces decreased acuity only in the exceptional case . The field defects seen with true papilledema are an enlarged blind spot and, in chronic papilledema, constriction of the field, especially nasally . The correct diagnosis, I assume, was eventually made in this patient because of his physician's knowledge of the association between giant cell arteritis and ischemic optic neuropathy in older patients . Most patients with giant cell arteritis also have elevated sedimentation rates, although this is variable . Once the diagnosis is made, treatment is usually initiated with systemic corticosteroids to try to prevent involvement of the second eye . Both eyes were already affected here, but one eye had relatively good acuity and both had significant preserved field, so one purpose of the corticosteroid treatment was presumably the prevention of second infarctions . This is a reasonable course, although most patients with optic nerve ischemia do not have second episodes in the same eye . The question



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in patients with known giant cell arteritis is usually not whether to treat but rather how long treatment should be continued . The disease appears to burn itself out over time, and various parameters have been suggested for determining whether this has occurred, including repeat sedimentation rate and rebiopsy . My own thinking on this point is influenced by Hollenhorst's observation that of the patients followed at the Mayo Clinic none have lost vision after four months of treatment ." Unless there are other compelling reasons for corticosteroid treatment, I would try to take this patient off corticosteroid therapy as soon as possible to avoid the complications of long-term treatment .

patient carefully questioned with respect to a history of headache, scalp tenderness, jaw pain or any of the protean manifestations of polymyalgia rheumatics .' If the patient has any of the above symptoms, or if the sedimentation rate is elevated, the patient should be given 100 mg of prednisolone, or its equivalent, by mouth immediately in the office and then admitted to the hospital for a medical evaluation . A confirmatory biopsy of the temporal artery should be obtained (3-5 cm) since you are committing the patient to longterm, high-dose systemic corticosteroid treatment, and the aid of an internist should be enlisted in order to minimize any of the systemic side effects that might arise from the corticosteroid therapy . The dosage should slowly be reduced (10 mg/day/week) while the sedimentation rate is monitored . The patient should be kept on corticosteroids for a minimum of six months and should not be weaned unless the sedimentation rate can be kept stable . If the patient does not relate a positive history and the sedimentation rate is normal, the presumptive diagnosis of idiopathic arteriolar sclerotic ischemic optic neuropathy should be made . Because of the high prevalence of diabetes mellitus and hypertension in this group of patients, a judicious course of action is to refer the patient to his or her family doctor for a general medical evaluation . Findings, Management and Outcome. The erythrocyte sedimentation rate was 65 . A temporal artery biopsy showed a diffuse inflammatory process with numerous giant cells and destruction of the elastic lamina . With the diagnosis of giant cell arteritis, the patient was placed on 80 mg of prednisone, resulting in rapid relief of his headaches . His erythrocyte sedimentation rate fell to the mid-20 range over the next five months . Visual acuity and visual fields remained stable, and the right optic disc gradually became pale.

Comments of Ronald M. Burde, M.D., Washington University School of Medicine .

The clinical finding of a swollen disc associated with contralateral optic atrophy immediately raises the spectre of a frontal mass lesion that has destroyed one nerve and is now compromising the contralateral nerve, either by contiguity or pressure .''' This scenario comprises the well-known, but extremely rare, Foster-Kennedy syndrome and immediately sets the clinical trap for an allout search for the offending lesion . From the information provided to us in the protocol, the diagnosis of a true FosterKennedy syndrome should be excluded . First of all, the patient has central visual loss in both eyes with an inferior altitudinal field defect . Papilledema, as a rule, does not affect central visual acuity, and it does not produce inferior arcuate defects but progressive constriction, more often accentuated in the nasal field .4,7 Altitudinal field defects are the hallmark of circulatory insufficiency of the optic nerve in the juxta lamina cribrosa regions .` The two most likely diagnoses on this basis are arteriolar sclerotic ischemic optic neuropathy and giant cell arteritis . Secondly, the description of the optic nervehead as being pale and swollen" is typical of ischemic insult and not compatible with papilledema secondary to raised intracranial pressure . It is true that the disc in chronic papilledema may appear pale, but this type of secondary pallor should not be confused with the pale disc swelling of ischemia . At this point, an immediate erythrocyte sedimentation rate should be drawn and the

Conclusion All that is swollen is not edema . EDITOR'S NOTE : This article and the following

one deal with giant cell arteritis . The first case demonstrates that even acute ischenic optic neuropathy can be confused with other causes of a swollen disc unless the correct possibility is entertained . Misdiagnosis led to the performance of unnecessary neurologic testing, In the second case,

CLINICAL CHALLENGES the widespread manifestations of giant cell arteritis are well demonstrated. The cases are juxtaposed to reemphasize that giant cell arteritis is a relatively common disease that is likely to be seen by all ophthalmologists . The performance of an adequate history combined with an erythrocyte sedimentation rate (ESR) is likely to provide the correct diagnosis in the vast majority of cases . PAUL HENKIND References 1 . Albert DM, Ruchman MC, Kellner JL : Skip areas in temporal arteritis . Arch Ophthalmol 94 :2072-2077, 1976 2 . Andrews JM : Giant cell arteritis . Neurology 16 :963-971, 1966 3 . Barber HS : Myalgic syndrome with constitutional effects . Polymyalgia rheumatic, Ann Rheum Dis 16 :230-237, 1957 4 . Harrington DO : Visual Fields . A Textbook and Atlas of Clinical Perimetry. St Louis, CV Mosby, 1971, ed 3 5 . Hayreh SS : Pathogenesis of visual field defects . Role of the posterior ciliary circulation . Br 3 Ophthalmol 54 :289-311, 1970 6. Hollenhorst RW, Brown JR . Wagener HP, Shick RM : Neurologic aspects of temporal arteritis . Neurology 10 :490-498, 1960 7 . Huber A : Eye Signs and Symptoms in Brain Tumors (translated, edited and updated by F . C . Blodi) . St Louis, CV Mosby, 1976 8 . Huston KA, Hunder GG, Lie JT et al : Temporal arteritis : A 25-year epidemiologic,

263 clinical and pathologic study . Ann Intern Med 88 :162-167, 1978 9 . Kennedy F : Retrobulbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal lobes . Am J Med Sci 142 :355-368, 1911 10 . Klein RG, Campbell RJ, Hunder GC, Carney JA : The existence and significance of skip lesion in TA . Mayo Clin Proc 51 :504-510, 1976 11 . Meadows SP : Temporal or giant cell arteritis . Ophthalmic aspects, in Smith JL (ed) : Neuroophthalmology . Symposium of the University of Miami and the Bascom Palmer Eye Institute, Vol IV . St Louis, CV Mosby, 1968, p 148 12 . Russell RUR : Giant cell arteritis : Review of 35 cases . Q 3 Med 28 :471-489, 1959 13 . Schatz NJ, Smith JL : Non-tumor causes of the Foster-Kennedy syndrome . J Neurosurg 27 :37-44, 1967 14 . Symposium on Neuro-Ophthalmology . Transactions of the New Orleans Academy of Ophthalmology . St Louis, CV Mosby, 1976, p 282 15 . Walsh FB, Hoyt WF : Clinical Neuroophthalmology . Vol 1 . Baltimore, Williams & Wilkins, 1969

Requests for reprints should be addressed to Ronald M . Burde, M .D ., Department of Ophthalmology, Box 8096, Washington University School of Medicine, 660 S . Euclid Avenue, St . Louis . Missouri 63110 .