Journal ofNeurology, Neurosurgery, and Psychiatry 1991;54:826-829

Papilloedema in Behcet's disease: value ofMRI indiagnosis of dural sinus thrombosis

Kamal M El-Ramahi, M Zuheir Al-Kawi

AbstractBehcet's disease is a multisystem diseasecharacterised by the clinical triad of oralulcers, genital ulcers and uveitis.Nervous system involvement is frequentand occasionally precedes other mani-festations. Behcet's disease is notfrequently considered in the differentialdiagnosis -of papilloedema. We reportfour cases of Behcet's disease in whichpapilloedema occurred with or withoutdural sinus thrombosis. MRI is of greatvalue in the investigation of suchpatients as it can demonstrate venoussinus thrombosis non-invasively or sug-gest the diagnosis by showing theassociated parenchymal lesions secon-dary to small vessel pathology.

Behcet's disease is recognised as a multisys-tem disease. Besides the classic triad ofrecurrent oral ulcers, genital ulcers and uveitisa wide spectrum of signs have been reported.'Any part of the neuraxis can be involved witha predilection to the brainstem and dien-cephalon.2 Vascular manifestations includearterial occlusions, thrombosis, aneurysmalformation3 and venous thrombosis,4papilloedema without associated uveitis mayoccur as a presenting manifestation. It isusually attributed to dural sinus thrombosis,5but well documented cases of such occurren-ces are rare even in countries where the dis-ease is frequent. Our report emphasises thisfinding and demonstrates the value of MRI inthe diagnosis of dural sinus thrombosis.

Department ofMedicine, King FaisalSpecialist Hospitaland Research Centre,Riyadh, Saudi ArabiaKM El-RamahiM Z Al-KawiCorrespondence to:Dr El-Ramahi, Departmentof Medicine, King FaisalSpecialist Hospital andResearch Centre, PO Box3354, Riyadh 1121 1, SaudiArabia.Received 17 August 1990and in final revised form22 January 1991.Accepted 24 January 1991

Case Report 1A 22 year old Saudi female presented withdecreased vision, headaches, nausea, vomitingand abdominal pain. History of recurrent oraland genital ulcers, skin papules and pustules,and arthritis was obtained.Funduscopy revealed bilateral papilleodema

and retinal vasculitis. Visual acuity was downto light perception in the left eye and countingfingers at 3 metres in the right.

Neurological examination showed left armdrift and left sixth nerve paresis. Otherpositive findings were oral ulcers, papulo-pus-tular skin lesions and right knee swelling andtenderness.Prothrombin time (PT), partial thrombo-

plastin time (PTT), fibrinogen, reptilase timeand antithrombin III were normal. Eryth-rocyte sedimentation rate (ESR) was 45 mm/h, platelets 545 x 103/ml'. Euglobulin lysistime was abnormally prolonged; pre stress 8 h(N 2-6 h) and post stress 7 h (N 40-80 min-utes).CT scan of the brain with contrast showed a

filling defect in the superior sagittal sinus

(Delta sign) (fig 1). MRI of the head showedsuperior sagittal and straight sinus throm-boses (fig 2). Cerebral angiography by intra-arterial injection confirmed superior sagittalsinus thrombosis (fig 3). Lumbar punctureopening pressure was 360 mm H20 with nor-mal cells, glucose and protein.

She was treated with heparin and pred-nisone 60 mg a day and her visual acuityimproved slightly. The vitreous cellular infil-trate and retinal vasculitis disappeared.

Repeat MRI of the brain showed evidenceof recanalisation of the thrombosed sagittalsinus (fig 4). Addition of Azathioprine did notresult in any further improvement. She wasthen maintained on prednisone 10 mg daily.There was no recurrence of oral or genitalulcers, skin lesions or arthritis on this regime.Visual acuity remained unchanged 14 monthslater.

Case Report 2A 19 year old Saudi female presented in 1983with headache, rapidly deteriorating vision,vomiting and arthritis of her knees. She hadrecurrent oral and genital ulcers. Bilateralpapilloedema without uveitis was present.Visual acuity was 6/60. She was given pred-nisone 60 mg daily. Headache and oral ulcersrecurred whenever the dose of prednisone wasdecreased. Examination revealed left sixthnerve palsy and multiple skin boils on thebuttocks.

Laboratory data revealed ESR 108 mm/h,rheumatoid factor (RF), antinuclear antibody(ANA), C-reactive protein (CRP), rapid plas-min reagin (RPR), circulating immune com-plexes (CIC) and antithrombin III were nor-

Figure 1 Enhanced CT scan of the head. Patient 1showing afilling defect in the superior sagittal sinus(Delta sign) (arrowhead).

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Papilloedema in Behcet's disease: value ofMRI in diagnosis of dural sinus thrombosis

Figure 2 Gadoliniumenhanced MRI of the headTl weighted image (TR2000 TE20). (Patient 1)Showing irregularfillingdefects in the straight sinus(arrowheads) and thesuperior sagittal sinus.

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,a+

Figure 3 Cerebral angiography confirms non-filling of the posterior half of thsagittal sinus (arrowhead) and the straight sinus. (Patient 1).

Figure 4 Gadoliniumenhanced MRI of the headTl weighted image(TR2000 TE20).(Patient 1) showingfillingof the recanalised superiorsagittal sinus.

mal. Euglobulin lysis time was considerablyprolonged with no lysis in 24 hours.CT scan of the brain suggested transverse

~ sinus occlusion with marked venous_ collaterals and venous dilatation.

Carotid angiogram showed completeocclusion of the right transverse sinus and atthe mid left transverse sinus. Lumbar punc-ture opening pressure was 320 mm H20. CSFanalysis was normal. Lumbo-peritoneal shuntwas performed followed by treatment withprednisone and chlorambucil. Visionremained impaired. Repeat digital substrac-tion angiography (DSA) by intra-arterialinjection showed occlusions of both transversesinuses. Five years later she developed epis-cleritis of the right eye which recurred a yearlater.

Case Report 3A 23 year old Saudi male presented to anotherhospital in September 1988 with headaches,blurred vision and horizontal diplopia. Afternormal CT scan he was diagnosed as havingbenign intracranial hypertension. Symptomssubsided spontaneously within one month. InNovember 1988, he experienced sudden

A. weakness of his left arm which subsided spon-taneously after two weeks. In May 1989he developed intermittent fever with chills,

,A shortness of breath, right sided pleuritic painand palpitation. He was re-admitted to thelocal hospital with fever and bilateralpapilloedema. Echocardiogram showed a rightventricular mass, he was then referred to ourhospital.

History of recurrent oral and genital ulcerswas confirmed. Multiple fresh ulcers on thelips, tongue and cheeks and two scrotal scarswere present. There was no uveitis, papil-loedema nor were there any neurologicaldeficit detected on examination in our hospital.Pulse in the Right Radial artery was absent.

Pertinent laboratory data included, leuco-cytosis 22-6 x 103/ml3, platelets 328 x103/ml3 and raised ESR 116 mm/h. The fol-lowing laboratory tests gave negative or

esuperlor normal results, RF, ANA, CRP, PT, PTT,plasminogen, reptilase time and antithrombinIII. Euglobulin lysis time was prolongedto eight hour pre-stress (N 2-6 h) and sevenhour post-stress (N 40-80 minutes). Bilateralpulmonary emboli were detected on scinti-graphy, and a right ventricular thrombuson echocardigram and right ventriculogram.Pulmonary angiography revealed pulmonaryvasculitis.CT scan of the head at the referring hos-

pital was negative. MRI of the head showed a0 5 cm area of high signal intensity in the deepwhite matter in the right parietal region on T2weighted images. No dural sinus thrombosiswas present, digital substraction angiographywas not performed.He was treated with prednisone 60 mg/day,

azathioprine and warfarin. At a twelve monthfollow up, no recurrence of papilloedema norany visual or neurological manifestations werepresent. He continued to receive prednisone10 mg/day, azathioprine and warfarin.

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Table 1 Summary of clinicalfeatures and selected laboratory results

Sexl Oral Genital Skin Occular Neurological Euglobulin Dural sinusNo age ulcers ulcers lesions Arthritis manifestations manifestations ESR lysis time thrombosis

1 F/22 + + + + Bilateral papilloedema Left sixth 45 mm/h Prolonged +Retinal vasculitis nerve palsyVisual acuityLight perception

2 F/19 + + + + Bilateral papilloedema Left sixth 108 mm/h Prolonged +Episcleritis nerve palsyVisual acuityRight-lightperceptionLeft-No lightperception

3 M/23 + + - - Bilateral papilloedema Left arm weakness 116 mm/h Prolonged -

4 M/ 18 + + - - Bilateral papilloedema - 150 mm/h Prolonged +

+ = Present.- = Absent.ESR = Erythrocyte Sedimentation Rate.

Case Report 4An 18 year old Saudi male presented withfever of three months duration and history ofrecurrent oral and scrotal ulcers and head-aches. Upon admission, he developed hae-moptysis. Examination revealed two scrotalulcers and bilateral papilloedema withoutuveitis. He was acutely ill with marked res-

piratory distress. Haemoglobin was 7-7 gm/dl,platelets 821 x 103/ml3 and ESR 150 mm/h.Lupus anti-coagulant and antithrombin IIIwere negative while euglobulin lysis time was

prolonged to 21 hours (normal range 2-6hours) pre-stress and 22 hours post-stress(normal range 40-80 minutes). Pulmonaryscintigraphy strongly suggested of pulmonaryemboli. Echocardiogram showed right and leftventricular masses, and a biopsy of one

proved it to be a thrombus. Pulmonaryangiography was positive for vasculitis withaneurysms. CT scan of the brain was negative.MRI of the head was compatible withsuperior sagittal sinus thrombosis and throm-bosis of the right transverse sinus extendinginto the right sigmoid sinus. DSA was notcarried out.

After initial treatment with heparin, pred-nisone 60 mg/day and cyclophosphamide100 mg/day were added, he became asymp-tomatic.A repeat MRI of the head before discharge

showed recanalisation of the thrombosed sin-uses. Papilloedema resolved and he remainedwell during the next twenty months of followup. Cyclophosphamide was discontinued andprednisone maintained at 5 mg/day.

DiscussionFour cases with Behcet's disease are presented,papilloedema was present in all, evidence ofdural sinus thrombosis was documented inthree. Tables 1 and 2 summarise clinical andradiological manifestation. Patients 1 and 2fulfil the new criteria for diagnosis of Behcet'sdisease.6 Although cases 3 and 4 do not fulfilthese criteria, the presence of oral and genitalulcers and evidence of pulmonary vasculitiswith aneurysm strongly supports the diagnosis.

Papilloedema, headache and intracranialhypertension were seen in patients 1 and 2. Inboth, the CT scans revealed sagittal sinusthrombosis or transverse sinus occlusion andthis was further elicited by cerebral angiogra-phy (table 2). MRI in patient 1 showed a

thrombosed superior sagittal sinus, in additionto a thrombosed straight sinus. CT scans were

normal in patients 3 and 4 but MRI revealedsuperior sagittal, right transverse and rightsigmoid sinus thrombosis in patient 4 and an

intraparenchymal area of high signal intensityin the white matter in patient 3 suggesting thediagnosis and eliminating the need forangiographic studies. Recanalisation of sinusesthrombosed earlier was clearly demonstratedon MRI in patients 1 and 4.

Papilloedema, headache and vomiting as

manifestations ofraised intracranial pressure inBehcet's disease were first reported byMasheter in 1959.7 Papilloedema withoutheadache and vomiting was also described byWadia in 19578 and Berlin in 1960.9 Five cases

ofpapilloedema in Behcet's disease were repor-

ted by Kalbian in 1970. It was not accompanied

Table 2 Summary of the imagingfeatures and CSF pressure

CSF openingNo Headache pressure CT scan MRI Cerebral angiography

1 + 360 mm H,0 Superior Sagittal Superior Sagittal and straight Superior Sagittal sinussinus thrombosis sinus thrombosis thrombosis

2 + 320 mm H,O Transverse sinus ND Right transverse and mid-leftocclusion sinus occlusion

3 + ND Normal 0-5 cm high intensity signal in NDthe deep white matter inthe parietal region

4 + ND Normal Superior Sagittal, Right transverse NDand Right sigmoid sinus thrombosis

+ = Present.ND = Not done.

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Papilloedema in Behcet's disease: value ofMRI in diagnosis of dural sinus thrombosis

or preceded by uveitis in two patients.5Various imaging methods were utilised in the

diagnosis of dural sinus thrombosis. The goldstandard has been cerebral angiography show-ing a filling defect in the superior sagittal sinusand prolonged cerebral circulation."2' DSAprovided the advantage of improved imagesand decreased amount of contrast needed.MRI was reported to show abnormal signal

in thrombosed dural sinuses of variousaetiologies. Also MRI was used to demonstratebrain abnormalities in Behcet's disease,21-24 butthere are no reports documenting dural sinusthrombosis by MRI in Behcet's disease.CT scan is useful in showing changes com-

patible with dural thrombosis.'2 But it is lesssensitive than angiography and a negative CTdoes not rule out dural sinus thrombosis.' 016Decreased fibrinolytic activity was reported

in some patients with pseudotumour cerebriand suggested an unfavourable prognosisassociated with recurrence or severe complica-tions.25 Diminished fibrinolytic activity in theperipheral blood of patients with Behcet'sdisease has also been reported.26 This maypartly explain the predilection of patients withBehcet's disease to develop thrombotic eventsin general, and dural sinus thrombosis inparticular with subsequent raised intracranialpressure. Our data are consistent with thisobservation. Another potential cause forpseudotumour cerebri in these patientsincludes sudden withdrawal of corticosteroids,this was not the case in any of our patients.We recommend MRI in patients with Beh-

cet's disease presenting with papilloedema andsupport the theory that these may be due todural sinus thrombosis.

1 Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behcet disease(syndrome). Semin Arthritis Rheum 1979;8:223-60.

2 O'Duffy JD, Goldstein NP. Neurological involvement in 7

patients with Behcet's disease. Am J Med 1976;61:170-8.3 Hamza M. Large artery involvement in Behcet's disease.

Rheumatol 1987;14:554-9.4 Chajek T, Fainaru M. Behcet's disease. Report of 41 cases

and review of the literature. Medicine 1975;54:179-96.5 Kalbian VV, Challis MT. Behcet's disease. Report of

Twelve cases with three manifesting as papilledema. Am JMed 1970;49:823-9.

6 International Study Group for Behcet's Disease. Criteria fordiagnosis of Behcet's Disease. Lancet 1990;335:1078-80.

7 Masheter HC. Behcet's syndrome complicated by intracran-ial thrombophlebitis. Proc Roy Soc Med 1959;52:1039-40.

8 Wadia N, Williams E. Behcet's syndrome with neurologicalcomplications. Brain 1957;80:59-63.

9 Berlin C. Behcet's disease as a multiple system complex.Arch Derm 1960;82:73-79.

10 Pamir MN, Kansu T, Erbengi A, Zeileli T. Papilledema inBehcet's syndrome. Arch Neurol 1981;88:643-5.

11 Bousser MG. Cerebral vein thrombosis in Behcet's syn-drome (letter). Arch Neurol 1982;39:322.

12 Rosenberger A, Adler OB, Haim S. Radiological aspects ofBehcet's disease. Radiology 1982;144:261-4.

13 Bank I, Weart C. Dural sinus thrombosis in Behcet's disease.Arthritis Rheum 1984;27:816-18.

14 Bousser MG, Chiras J, Bories J, Castaigne P. Cerebralvenous thrombosis. A review of 38 cases. Stroke 1985;16:199-213.

15 Wechsler B, Bousser MG, Houng DU LT et al. Cerebralvenous sinus thrombosis in Behcet's disease (letter). MayoClin Proc 1985;60:891.

16 Brissaud P, Laroche L. De Gramont A, Krulik M. Digitalangiography for the diagnosis of dural sinus thrombosis inBehcet's disease. Arthritis Rheumatism 1985;28:359-60.

17 Harper CM, O'Neill BP, O'Duffy JD, Forbes GS. Intracran-ial hypertension in Behcet's disease: Demonstration ofsinus occlusion with use ofDigital Substraction Angiogra-phy. Mayo Clin Proc 1985;60:419-22.

18 Ben-Itzhak J, Keren S, Simon J. Intracranial thrombosis inBehcet's syndrome. Neuro Radiology 1985;27:450.

19 Barbas CSV, de Carvalho CPP, Delmonte VC, et al. Behcet'sdisease. A rare case of simultaneous pulmonary andcerebral involvement. Am J Med 1988;85:576-8.

20 Serdaroglu P, Yazici H, Ozdemir C, et al. Neurologicinvolvement in Behcet's syndrome. A prospective study.Arch Neurol 1989;46:265-9.

21 Willeit J, Schmutzhard E, Aichner F, et al. CT and MRimaging in Neuro Behcet's disease. J Comput AssistTomogr 1986;10:313-18.

22 Fukuyama H, Kameyama M, Nabatame H, et al. Magneticresonance images ofneuro-Behcet syndrome show precisebrain stem lesions. Report of a Case. Acta Neurol Scand1987;75:70-73.

23 Vidaller A, Carratala J, Moreno R, et al. Magnetic Reson-ance Imaging in Neuro-Behcet's Disease (letter). Br JRheumatology 1988;27(l):79-80.

24 Purvin V, Dunn DW, Edwards M. MRI and cerebral venousthrombosis. Comp Radiol 1987;11:75-79.

25 Al-Kawi MZ, Chavis P, Bashir R, et al. Fibrinolysisin pseudo tumor cerebri in brain edema, pathogenesis,imaging and therapy, D Long Editor, Advances in neur-ology, Vol 52. 1990:539.

26 Mishima H, Masuda K, Shjimada S, et al. Plasminogenactivators activity levels in patients with Behcet's Syn-drome. Arch Ophthalmology 1985;103:935-6.

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