Optic neuropathy in sarcoidosis - BMJ

Journal of Neurology, Neurosurgery, and Psychiatry 1986;49:756-763

Optic neuropathy in sarcoidosisELIZABETH M GRAHAM, CJK ELLIS, MD SANDERS, WI McDONALD

From the National Hospitalfor Nervous Diseases and the Medical Eye Unit, St Thomas' Hospital, London

SUMMARY Five patients with isolated optic neuropathy and sarcoidosis are discussed. The spectrumof clinical disease was variable but two groups could be identified: patients with chronic progressivevisual loss which was associated with thickening of the optic nerve and was refractory to steroidtreatment, and patients with acute or subacute optic neuropathy in which the visual loss respondedrapidly to steroids. In the latter group steroid dependence developed in all three of the patients. Innone did the clinical picture resemble that of the optic neuritis associated with multiple sclerosis.

Sarcoidosis is a multisystem disease in which noncaseating epithelioid cell granulomata occur indifferent sites producing variable clinical features.Ocular involvement occurs in 25% of patients' andinvolvement of the central nervous system has beenassessed as occurring in 1-16% of patients.2Involvement of the optic nerve is unusual and occursin only 5% of cases.3 Clinical manifestations of opticnerve disease in sarcoidosis are diverse and includeuveitis or papilloedema but optic neuropathy, granu-lomata of the optic nerve and optic atrophy second-ary to compression by an intracranial sarcoid masscan occur.4 Isolated optic nerve disease associatedwith neither intraocular inflammatory nor extensivedisease of the central nervous system is rare. Wereport five patients with isolated optic nerve diseaseand sarcoidosis whom we have seen recently at eitherthe National Hospital for Nervous Diseases, QueenSquare, and Maida Vale or the Medical Eye Unit, StThomas' Hospital.

Case IA 43-year-old West Indian woman who came from Jamaicain 1962, presented in May 1976 complaining of loss of visionin her right eye. This had occurred five years previously in1971 when she had noticed sudden loss of vision associatedwith some discomfort in the globe. Initially vision appearedas a "white fog" but deteriorated over six weeks until shecould just count fingers. Vision had remained unchangeduntil in August 1975, further deterioration occurred withloss of light perception in the right eye though in her left eyevision was normal.

Address for reprint requests: Dr EM Graham, Department of Neuro-Ophthalmology, The National Hospital, Queen Square, LondonWCIN 3BG, UK

Received 6 September 1985 and in revised form 4 November 1985.Accepted 9 November 1985

She was admitted to the Maida Vale Hospital in 1976 forassessment. General examination revealed an obese ladywith hypertension (BP 180/130 mm Hg). Visual acuity in theleft eye was 6/5 with no perception of light in the right. Theleft visual field was full. There was no pupillary constrictionon direct light stimulation of the right eye. There was noproptosis and eye movements were full. Slit lamp exam-ination was normal. The right optic disc was atrophic withhypertensive retinal vascular changes and two smallaneurysmal dilatations of the disc vessels (fig 1). The leftoptic disc was normal. Neurological examination was other-wise normal. Investigation at this time showed a normalblood count, negative WR and an ESR of 30 mm/hour.Radiographs of the abdomen, chest, optic foramina andskull were all normal. CT scan showed normal intracranialand orbital views. It was considered that she had had a rightcentral retinal artery occlusion in 1971 and that there was noclear history of documented progression since that time. Shefailed to attend for follow up but reappeared in January1980 when she noticed that colours seemed less bright thanusual in her left eye. Although the vision in her left eye con-tinued to deteriorate, she again failed to attend until Sep-tember 1980 when she was admitted. At that time she com-plained that her blind right eye had become uncomfortableand itchy. Her general health was good although she rarelytook her anti-hypertensive therapy.On examination the visual acuity in her left eye had deteri-

orated to perception of light with hand movements in thenasal quadrant. There was 6 mm proptosis of the right eyewhich was divergent but eye movements were full. The rightdisc was pale but now swollen and infiltrated with dilatationof superficial capillaries (fig 2). The left optic disc showedtemporal pallor. A fluorescein angiogram of the right eyeconfirmed the extensive dilatation of the capillaries on theoptic disc and marked leakage of dye from the disc and sur-rounding infiltrated retina. Fluorescein angiography of theleft eye was normal. Investigation showed a neutrophil leu-cocytosis of 13,000, ESR 35, raised plasma protein and cere-brospinal fluid (CSF) protein of 69 mg/dl with 19lymphocytes/mm.3 Oligoclonal bands were not detected.Hypocycloidal polytomograms of optic canals and bilateralcarotid angiograms were normal but a CT scan showed

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Optic neuropathy in sarcoidosis

Fig 1 Case 1. (1976) The right optic disc is atrophic with two small aneurysmal dilatations ofthe optic disc vessels (left).The left optic disc and retina are normal (right).

thickening of the right optic nerve (fig 3).A lateral orbitotomy (Mr J Wright) was performed and a

cross sectional biopsy of the swollen right optic nerverevealed infiltration with epithelioid and giant cells formingtypical non caseating granulomata of sarcoidosis (fig 4).Treatment with Prednisolone 80 mg daily did not produceany visual improvement. Follow up in the subsequent twoyears showed no further visual deterioration.

Case 2A 50-year-old West Indian woman who had lived in theUnited Kingdom since 1963 and had a history of maturityonset diabetes mellitus, presented in February 1982 withprogressive, painless visual loss in the right eye. The visiondeteriorated over four weeks to no perception of light. Sheattended her local doctor who could not find any cause forthe loss of vision. Four months later she noticed progressive

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Fig 2 Case 1. (1980) The right optic disc is swollen, infiltrated with dilated superficial capillaries (left). The left optic disc isatrophic, with loss ofnervefibres (right).

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Graham, Ellis, Sanders, McDonald

Fig 3 Case 1. Axial CT Scan showing a thickened rightoptic nerve.

deterioration of vision in her left eye. She had lost two stonein weight during the previous three months, but otherwiseher general health was good. General examination wasunremarkable.

Visual acuity in the left eye was 6/36 corrected with noperception of light in the right eye. The left visual field wasconstricted with an inferior altitudinal defect to a 3 mm

CJ 22 6.82Fig 5 Case 2. Visualfield on presentation showed a leftinferior altitudinal defect. Visual acuity was no perception oflight in the right eye.

white target (fig 5). Colour vision was absent in both eyes. Aright relative afferent pupillary defect was present. Slit lampexamination was normal. The right optic disc was atrophicand the left showed some temporal pallor. There was noevidence of diabetic retinopathy. Investigation showed nor-mal blood count, ESR, electrolytes and negative WR. Uri-nary calcium 8-31 mmol/24 hours (<6-00 mmol/l). Serumangiotensin converting enzyme (SACE) was 74 nmol/min/ml(16-53 nmol/min/ml). CSF protein was 95 mg/dl with 34

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Fig 4 Case 1. Right optic nerve is infiltrated with non caseating granulomata (H andE x 33).

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22-11-83Fig 6 Case 3. The right visualfield isfull. There is a densetemporal and infero-nasalfield defect in the left eye to an I4isopter.

white cells/mm,3 (80% lymphocytes, 6% polymorphs). Nooligoclonal bands were detected. Chest radiographs showedhilar lymphadenopathy and skull radiographs showed thatthe right optic foramen was expanded. Orbital CT scanshowed thickening of both optic nerves. Pattern reversalvisual evoked potentials (VEPs) (Dr AM Halliday) showedabsent responses on the right and delay on the left with alatency 161 ms (normal upper limit of latency 115 ms) andan amplitude of 2 pV. A Kveim test was positive and Man-toux 1:1000 was negative.The diagnosis of sarcoidosis was supported by the hilar

adenopathy, lymphopenia, raised 24 hour urinary calciumexcretion, high SACE levels, and the positive Kveim test.The CSF was compatible. Treatment was started with sys-temic steriods (80 mg prednisolone daily) and within twoweeks the left visual acuity had improved to 6/9 with fullcolour vision and resolution of the inferior field defect.There was no improvement in the vision of the right eye.

Case 3A 26-year-old Caucasian woman presented in February1983 with blurred vision in the left eye progressing over twoweeks associated with ocular discomfort on eye movements.She attended the local eye hospital where visual acuity wasrecorded as 6/60 in the left eye and 6/6 in the right. Fundus

Fig 7 Case 4. Visual acuity in the right eye was 6/5 with fullvisualfield. Visual acuity in the left eye was 6/60 with a densecentral scotoma and a constricted peripheralfield.

Fig 8 Case 4. Late phasefluorescein angiogram ofleft eyeshowing some disc leakage but no macular oedema.

examination was normal. A diagnosis of acute optic neuro-pathy was made and she was treated with prednisolone 20mg daily and within two weeks the left visual acuity hadimproved to 6/12. The steroids were gradually discontinuedbut three weeks after the last dose the left visual acuity againdropped to 6/60 and the left optic disc was noted to be pale.Two months later she developed pain in both eyes, againexacerbated by ocular movement and visual acuity was now6/18 on the right and 6/60 on the left. She was treated withprednisolone 40 mg daily and the visual acuity on the rightimproved to 6/5 but there was no improvement in the lefteye. However, the daily dose of prednisolone had to bemaintained above 20 mg to prevent deterioration of vision inthe right eye. She also complained of episodes of transienttingling in the hands and feet but reported no other neu-rological symptoms. There was no history of previous jointor skin disease.

General examination revealed a Cushingoid appearancewith striae on the legs and abdomen. Visual acuity was 6/6 in

Fig 9 Case 6. The right visualfield was irregularlyconstricted due to numerous arcuate defects: the blind spotwas enlarged.

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the right and 6/60 in the left eye with a dense temporal andinfero-nasal field defect (fig 6). The right visual field was fullto a small object. There was a marked left relative afferentpupillary defect. Colour vision was normal in the right eyebut absent in the left. Slit lamp examination was normal.The right optic disc showed temporal pallor and the leftoptic disc was atrophic. Neurological examination was oth-erwise normal.

Investigation showed a neutrophil leucocytosis of16,800/mm3 and normal electrolytes and liver function tests.CSF contained 2 WBC/mm3 and protein 34 mg/100 ml witholigoclonal bands. SACE was 18 nmol/min/ml (normalrange 16-53). Chest radiographs showed bilateral hilarlymphadenopathy and a Kveim test showed non caseatinggranulomata. Pattern reversal VEPs (Dr AM Halliday)showed a latency of 122 ms (normal upper limit of latency115 ms) and amplitude 4-2 uV on the right eye stimulationand no consistent response from the left eye. Somatosensoryand auditory evoked potentials were normal. Skull radio-grahs showed enlargement of the left optic foramen andorbital CT scan no abnormality of the optic nerves.

Case 4A 32-year-old West Indian man who had lived in the UnitedKingdom since 1960, presented in December 1979 with afour week history of blurring of central vision in the left eyewhich had been associated with pain in the eye for threeweeks. There were no other neurological symptoms. Whileawaiting admission he became breathless with a cough andretrosternal soreness and was admitted elsewhere where aclinical diagnosis of pulmonary sarcoidosis was made and aKveim test performed. His chest symptoms improved spon-taneously and he was discharged without treatment. How-ever, during the 6 weeks of this illness the vision in the lefteye had continued to deteriorate and he lost a stone and ahalf in weight.On admission examination showed a thin man without

lymphadenopathy or rash. The chest was clear. Visual acuityin the right eye was 6/6 N5 and in the left 6/60, < N48. Therewas no colour appreciation on the left, and colour vision wasnormal on the right. There was a left relative afferent pupil-lary defect. The visual field was full on the right but on theleft showed a small dense central scotoma with constrictedperipheral field (fig 7). The right fundus was normal but theleft disc showed supero-nasal swelling. There was no evi-dence of retinal vasculitis or uveitis. Fluorescein angio-graphy showed late leakage at the left optic disc withoutvenous leakage or macular oedema (fig 8).

Investigations showed a normal blood count and anESR of 66 mm in I hour. There were detectable immunecomplexes by polyethylene glycol precipitation at a con-centration of 8-8 mg IgG/dl (3.5 + 1 3 mglgG/dl). Chestradiographs confirmed the presence of bilateral hilarlymphadenopathy with fine nodular shadowing in the midzones. The SACE was elevated to about 100 nmol/min/ml.The previously inoculated Kveim test showed non caseatinggranulomata. The CSF showed oligoclonal bands but noother abnormality.The diagnosis of sarcoidosis was made on the basis of the

radiological appearances in the chest, the raised SACE andthe positive Kveim test. He was treated with a high dose ofprednisolone 60 mg daily with rapid improvement in his


ocular pain and visual loss. After one week's treatmentvisual acuity was 6/5 N5 bilaterally with normal fields. Sub-sequently he has been maintained on prednisolone 10 mgdaily. However, whenever the dose has been reduced, he hasexperienced ocular pain with drop of visual acuity to 6/6 anddesaturation of colour, accompanied by breathlessness. Attwo year follow-up the left optic disc is mildly atrophic.

Case 5A 31-year-old Caucasian man awoke one morning inDecember 1981 with loss of vision in the right eye associatedwith frontal headache and pain on eye movement. A pro-visional diagnosis of acute optic neuropathy was made else-where and he was treated with a short course of systemicsteroids with relief of the headache and some improvementin his vision. A week later he developed a polyarthropathywith tenosynovitis of his right hand.

Five years previously he was diagnosed as having acutesarcoidosis when he presented with erythema nodosum,polyarthropathy and bilateral hilar lymphadenopathy. Hewas not treated with steroids at this time and his symptomsresolved spontaneously. His mother had multiple sclerosisbut there was no other relevant family or past medical his-tory.

In January 1982 he was admitted for further investigationfollowing his visual loss. Physical examination revealed anobese normotensive white male with no lymphadenopathyor skin rashes. His chest was clear and his cardiovascularsystem was normal. He had restricted movements of thesmall joints of his hands and of his shoulders. There was noabnormality on neurological examination. Visual acuity wasreduced on the right to 6/12 N8 with 50% desaturation of ared object. The visual field to the Goldmann 14 isopter wasirregularly constricted consistent with numerous arcuatedefects and the blind spot was enlarged (fig 9). There was a

Fig 10 Case 5. The right optic disc is swollen with retinalexudates andperipapillary leakage (arrow).



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Clinicalfeatures of5 patients with isolated optic nerve disease and sarcoidosis

Patient Sex Age Clinical diagnosis CSF Chest Optic nerve SACENo (years) radiograph (CTscan) nmol/min/ml

WBC X 106/i Protein Oligoclonalgm% bands

1 F 36 Progressive optic neuropathy 19 lymphs 69 Absent Normal Thick (R) -

optic nerve2 F 50 Progressive optic neuropathy 21 lymphs 74 Absent Normal Thick nerves 74

with large R.optic foramina

3 F 30 Acute optic neuropathy 2 lymphs 34 Present BHL2 Large R. optic 18foramina

4 M 32 Acute optic neuropathy 11 lymphs 30 Present BHL Normal > 1005 M 31 Acute optic neuropathy 19 lymphs 69 Absent Normal Normal 55

(BHLin past)

SACE = Serum angiotensin converting enzyme.BHL = Bilateral hilar lymphadenopathy.Normal values 15-53 nmol/min/ml.

right relative afferent pupillary defect. Ocular movementswere normal. The anterior chambers were quiet and therewas no vitreous activity. The right optic disc was swollenwith haemorrhages and exudates between the disc and mac-ula (fig 10). Examination of the left eye was normal. Fluo-rescein angiography showed dilated capillaries in the righteye with mild leakage from these in the late phase. There wasno leakage at the macula or from the peripheral vessels. Theleft eye was normal.

Investigations in January 1982 showed haemoglobin 14-8g/dl white count 3-6 x 10/1(54% neutrophils, 34% lympho-cytes) ESR 8 mm/hour. Urea and electrolytes, liver functiontests, serum and urinary calcium were normal. VDRL,TPHA were negative. Cerebrospinal fluid examinationshowed an opening pressure of 200 mm CSF and no cells.The protein level was 50 mg/100 ml and no oligoclonalbands were detected. HLA typing was Al, A9, B8 and B17.Radiographs of his chest, skull, hand and spine and anorbital CT scan were normal. Pattern reversal VEPs (DrFenwick) showed a latency of 84 ms (normal upper limit oflatency 106 ms) and amplitude 7-7 pV on the left and latency108 ms and amplitude 3-3 pV on the right. He was treatedwith another course of systemic steroids but there was nofurther improvement in vision, and the steroids were tailedoff after eight weeks. A Mantoux test was positive at 1:1000dilution and Kveim test showed non caseating granulomata,both performed one month after steroid treatment wasfinished.

Discussion

We have described five patients with isolated opticnerve disease in association with sarcoidosis. Threepatients presented with acute or subacute painfulvisual loss and the other two with chronic painlessvisual failure. The diagnosis of sarcoidosis was basedon standard clinical criteria.5 In the first case ofprogressive visual failure the diagnosis was proved byoptic nerve biopsy. Of the three patients presentingwith acute or subacute optic neuropathy, one (case 5)had been diagnosed previously as having sarcoidosis

and case 4 presented with acute visual loss in associ-ation with acute pulmonary symptoms and very highlevels of SACE. In case 3 bilateral hilar lymph-adenopathy was found at the time of visual loss.Cases 4 and 5 had acute manifestations of sarcoidosis.Our patients showed a spectrum of visual abnormal-ities ranging from acute reversible visual loss tochronic progressive visual failure. Both mani-festations could be seen in different eyes of an individ-ual patient.

Progressive visual failureIn the two patients with chronic failure (cases 1 and 2)the visual loss occurred gradually over a period fromfour weeks to eight months and was not associatedwith pain in the affected eye. Both eyes weresequentially affected in each case, progressing to noperception of light in the more severely affected eye.Case 2 developed an inferior altitudinal field defect,but the subsequent rapid response to steroids sug-gested that the pathogenesis was not primarily vascu-lar.The appearances of the right optic disc of case 1 are

worthy of comment. Initially the disc was atrophicwith two vascular prominences in the centre; fouryears later the disc had become markedly infiltratedwith dilated and tortuous vessels. Infiltrated,"cauliflower" optic discs are well documented in sar-coidosis.68 These optic nerve granulomata com-monly occur in association with ocularinflammation.O10 11 However, if uveitis and otherstigmata of sarcoidosis are absent the diagnosis maybe confused with that of optic nerve tumours. 12-16We have been unable to find a previous report of sar-coid granulomata developing within an atrophicnerve as in case 1. Case 2 had a thick left optic nerveand may have also had granulomata within it.

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Acute or subacute visual lossThe three patients (cases 3, 4, and 5) who presented inthis way were initially diagnosed as suffering fromacute optic neuritis. However, by the time the patientshad been referred for further ophthalmological opin-ion they had developed unusual features for this diag-nosis. Acute optic neuritis of the type associated withmultiple sclerosis, often referred to loosely as "demy-elinating optic neuritis", is characterized by rapidvisual loss, progressing over a week or so, oftenaccompanied by ocular pain increased by eye move-ment. Examination usually reveals a negative centralscotoma, a relative afferent pupillary defect and anormal or mildly swollen optic disc. Spontaneousrecovery occurs in 90% of patients often beginningwithin about two weeks of the visual deficit reachingits peak and continuing for several weeks.17 Systemicor local (retro-ocular) corticosteroids may shorten theduration of the visual loss but do not alter the ulti-mate visual prognosis.18 19

Certain features in our patients were atypical for adiagnosis of demyelinating optic neuritis:-I.Although all three patients suffered pain in theaffected eye particularly on eye movement, and one(case 5) experienced rapid visual loss, the other twonoticed progression of the visual deficit over two tosix weeks. 2. Only one patient (case 4) developed thevisual field changes of central scotoma with periph-eral constriction resembling those of demyelinatingoptic neuritis. The remaining two patients had moreextensive field loss, case 3 showing central scotomatabreaking out to involve the periphery and case 5 anirregularly constricted field consistent with multiplearcuate scotomata. These observations contrast withthe results of study of 165 patients with demyelinatingoptic neuritis in which 76% had central field defectswith peripheral constriction.20 The optic discs of ourpatients varied from atrophy to mild disc swellingconfirmed by fluorescein angiography. Case 5 had adeep subretinal haemorrhage on the nasal margin ofthe right optic disc. Peripapillary haemorrhages indemyelinating optic neuritis are not infrequentthough subretinal haemorrhages are extremely rare inour experience.Investigation Results of visual evoked responses andexamination of the cerebrospinal fluid (table) did notserve to distinguish demyelinating optic neuritis fromoptic neuropathy complicating sarcoidosis. Oli-goclonal bands may be found in both2' and a delayedvisual evoked potential is not specific for optic neu-ritis.22 An enlarged right optic foramen was found incase 3 but the orbital CT scan did not demonstrate athick optic nerve in any of these four patients.Treatment Two patients (cases 3 and 4) showed agood response to corticosteroids. In case 3 the visionimproved in both eyes when steroids were given


within two weeks of onset of symptoms. However,when treatment was given after the visual deficit in theleft eye had been present for more than eight weeksthis beneficial effect no longer occurred. In case 4 thevision improved from 6/60 to 6/9 within 24 hours ofstarting treatment.The vision of the right eye of case 3 and the left eye

of case 4 remained steroid sensitive and reduction ofsteroids below a certain maintenance dose (20 mgprednisolone daily in case 3, 10 mg prednisolone dailyin case 4) resulted in drops in visual acuity and furthervisual field loss. A persistent dependence of visualfunction on steroids is rare in demyelinating opticneuritis.'7 The third patient (case 5) showed an initialimprovement during the first week on steroids butthereafter the visual deficit remained static.

There is only one other well documented report ofacute optic neuropathy resembling demyelinatingoptic neuritis occurring in the presence of sarcoid-osis.23 Pieron et al described a 23-year-old WestIndian woman with hilar lymphadenopathy whopresented with rapid visual loss in the right eyeaccompanied by central scotoma and raised gammaglobulin in the cerebrospinal fluid. The visual acuityand visual field returned to normal after twelve daysof treatment with prednisolone and synacthen.

Salvesen documented the first case of an acute opticneuritis-like syndrome in a patient with sarcoidosisand chronic lupus pernio" but the clinical details arescanty and follow up seven months later showed thatvision had improved only from hand movements to6/36. Similarly, Beardsley et al25 reported three casesof "retrobulbar neuritis" in their series of elevenpatients. The diagnosis of retrobulbar neuritis wasmade because of the normal optic disc and theabsence of neurological findings. In each, progressivepainless loss of vision occurred over a period rangingfrom one week to several months.25 In the latter casespontaneous recovery of vision occurred but the onsetwas unlike demyelinating optic neuritis. The othertwo patients improved on steroids.The mechanisms of visual loss in these patients

deserves comment. Progressive visual failure wasshown in one case to be associated with involvementof the optic nerve by granulomata and in two otherswith enlargement of the nerve at CT and/or enlarge-ment of the optic foramen. It seems likely that com-pression of the optic nerve fibres by sarcoid tissuecaused the visual loss in these progressive cases. Themechanism of visual loss in the other cases must beuncertain in the absence of histological data. In thepatients who became steroid dependent, it is possiblethat variations in size of granulomata were deter-mined by changes in dose. It is impossible to excludethe occurrence of a demyelinating optic neuritis of thetype associated with multiple sclerosis having devel-



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oped independently of the presence of sarcoidosis inthese patients. None of the patients has yet developedother clinical features of multiple sclerosis, but theperiod of follow-up is too short to conclude that theywill not do so.26The occurrence of irreversible visual loss in several

of our patients and the development of steroiddependency in others suggest that these drugs may beneeded for patients developing optic neuropathy in asetting of sarcoidosis. Suppressive doses should beused for a short initial period and the dose thenreduced fairly rapidly. Long-term steroids should beavoided if possible; if they are required the doseshould be kept to the minimum necessary to controlthe symptoms, and when possible an alternate dayregime should be used.

We thank Dr CJ Earl and Mr John Wright for allow-ing us to report patients under their care, Dr DonaldMitchell and Dr Nigel Bateman for performing theKveim tests, Miss Josephine Lace for secretarial assis-tance and Mr Richard Dewhirst for preparation ofthe illustrations.

References

'Spalton DJ, Sanders MD. Fundus changes in histologi-cally confirmed sarcoidosis. Br J Ophthalmol 1981 ;65:348-58.

2Matthews WB. Neurosarcoidosis. In: Vinken PJ, BruynGW, eds. Handbook ofClinical Neurology, Amsterdam:North Holland 1979;38:521-42.

3Ingestad R, Stigmar G. Sarcoidosis with ocular andhypothalamic-pituitary manifestations. Acta Ophthal-mol fCopenh) 1971;49: 1-10.

4Obenauf CD, Shaw EH, Sydnor CF, Klintworth GK. Sar-coidosis and its ophthalmic manifestations. Am J Oph-thalmol 1978;86:648-55.

'James DG. Ocular Sarcoidosis. In: F Clifford Rose, ed.The Eye in General Medicine. London: Chapman andHall, 1983:348-66.

6Noble KG. Ocular Sarcoidosis occurring as a unilateraloptic disc vascular lesion. Am J Ophthalmol 1979;87:490-3.

7Goldberg S, Newell FW. Sarcoidosis with retinalinvolvement. Report of 2 cases. Arch Ophthalmol1944;32:93-6.

8Laties AM, Scheie HG. Evolution of multiple small

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tumors in sarcoid granuloma of the optic disc. Am JOphthalmol 1972;74:60-6.

9Gass JDM, Olson CM. Sarcoidosis with optic nerve andretinal involvement. Arch Ophthalmol 1976;84:945-50.

°Kelley JS, Green WR. Sarcoidosis involving the opticnerve head. Arch Ophthalmol 1973;89:486-8.

Krohel GB, Charles H, Smith RS. Granulomatous opticneuropathy. Arch Ophthalmol 1981 ;99:1053-5.

12Statton R, Blodi FC, Hanigan J. Sarcoidosis of the opticnerve. Arch Ophthalmol 1964;71:834-6.

Anderson WG, Parker JJ, Sondheimer FK. Optic for-amen enlargement caused by sarcoid granuloma. Radi-ology 1966;86:319-22.

14Frisen L, Lindgreen BJ, Macgregor JL, Stattin S. Sarcoid-like disorder of the intracranial optic nerve. J NeurolNeurosurg Psychiatry 1977;40:702-7.

s Gudeman SK, Selhorst JB, Susac JO, Waybright EA. Sar-coid Optic Neuropathy. Neurology (NY) 1982;32:597-603.

16Lustgarten JS, Mindel JS, Yablonski ME, Friedman AH.An unusual presentation of isolated optic nerve sar-coidosis. J Clin Neurophthalmol 1983;3: 13-8.

17 McDonald WI. Acute Optic Neuritis. Br J Hospital Med1977;18:42-8.

18Bowden AN, Bowden PMA, Friedmann Al, Perkins GD,Rose FC. A trial of Corticotrophin gelatin injection inacute optic neuritis. J Neurol Neurosurg Psychiatry1974;37:869-73.

9Gould ES, Bird AC, Leaver PK, McDonald WI. Treat-ment of optic neuritis by retrobulbar injection of tri-amcinolone. Br Med J 1977;1:1495-7.

20Perkin GO, Rose FC. In: Rose FC, ed. Optic Neuritis.Oxford: Oxford University Press, 1979:50-67.

21Kinnman J, Link M. Intrathecal production of oligo-clonal IgM and IgG in CNS sarcoidosis. Acta NeurolScand 1984;69:97-106.

22 Halliday AM, McDonald WI. In: Stalberg E, Young RR,eds. London: Butterworths, 1981:228-58.

23 Pierson R, Coulaud JM, Debure A, Mafart Y. Lesobre B.Neurite optique et sarcoidose. Sem Hop Paris1979;55:137-9.

24Salvesen HA. The sarcoid of Boeck, a disease ofimportance to internal medicine. Report of four cases.Acda Med Scand 1935;86:127-51.

2"Beardsley TL, Brown SVL, Sydner CF, Grimson BS,Klintworth GK. Eleven cases of sarcoidosis of the opticnerve. Am J Ophthalmol 1984;97:62-77.

26Compston DAS, Batchelor JR, Earl CJ, McDonald WI.Factors influencing the risk of multiple sclerosis devel-oping in patients with optic neuritis. Brain 1978;101:495-511.



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