British Journal ofOphthalmology, 1991, 75, 368-371

CASE REPORTS

Pattern of arterial involvement of the head, neck,and eyes in giant cell arteritis: three case reports

Z Butt, J F Cullen, E Mutlukan

AbstractThe findings oftwo post-mortem examinationsand oneCT scan ofpatients with biopsy provedgiant cell arteritis (GCA) are presented. Thepresence or absence of intracranial involve-ment in GCA is discussed.

Giant cell arteritis (GCA) is rarely fatal, andreferences to the condition in post-mortemmaterial are uncommon.'-16 However, this maybe related to non-recognition of a fatal outcomein patients with GCA and because post-mortemexaminations of elderly patients dying fromvascular disorders are not routinely carried out.GCA may be concealed among the cases diag-nosed as ischaemic catastrophes due to arterio-sclerosis. "

Patients suffering from GCA have a range ofsymptoms including headache, jaw claudication,fever, anorexia, and loss of vision. However,atypically they may present with symptoms ofcerebral ischaemia, the aetiology ofwhich may beconfused with that of other conditions such asarteriosclerosis. We suggest that such atypicalsymptoms may be secondary to embolism andthat the diagnosis of GCA should not be over-looked. A raised erythrocyte sedimentation ratetogether with positive findings on temporalartery biopsy should help in confirming or refut-ing the diagnosis. GCA is treatable, and treat-ment leads to symptomatic improvement.

Case reports

Princess Alexandra EyePavilion, RoyalInfirmary, EdinburghEH3 9HAZ ButtJ F CullenE MutlukanCorrespondence to:Dr Z Butt, FRCS.Accepted for publication14 February 1991

CASE 1

An 80-year-old man presented in 1973 with a

three-month history of temporal headaches,influenza-like symptoms, and anorexia. Acuteloss of vision in the right eye had occurred twodays previously and acute loss ofvision in the lefteye followed on the day of presentation. Visualacuity was no perception of light in the right eyeand perception of light in the left eye. His pupilsdid not react to light, and there was bilateraloedema ofthe discs. The Westergren erythrocytesedimentation rate was 90 mm/h. Anteriorischaemic optic neuropathy secondary to GCAwas diagnosed, and high-dose corticosteroidtreatment was initiated. His mental conditiondeteriorated over the next few days, and he wasthought to have sustained a cerebrovascularaccident. He became comatose and died secon-dary to hypostatic pneumonia. His temporal

arteries at necropsy showed characteristicchanges ofGCA (see below).

Post-mortenfindings. Macroscopically the mainarteries at the base ofthe brain were virtually freefrom atheroma, but a plug of rather firm clot waspresent in the stump of the right internal carotid.The circle of Willis was normally constituted.Several haemorrhagic infarcts were noted in thecerebrum (frontal, parietal, temporal, andoccipital lobes) and cerebellum. Microscopicexamination confirmed that these werevery recent, practically terminal, infarcts.Occasionally small meningeal arteries overlyingthe cortical infarcts were noted to contain recentthrombus, but in none of the sections was thereevidence of giant cell arteritis.

Sections of the temporal, ophthalmic, verte-bral, internal carotid (in neck), external carotid,left common carotid, and coronary arteriesshowed changes typical of giant cell arteritis.Histological abnormalities noted included:(1) Diffuse fibrous thickening of the intimaassociated with mucinous changes and narrowingor occlusion of the lumen. (2) The presence oforganising mural thrombus (both internal caro-tid arteries). (3) Fragmentation and dis-appearance of the internal elastic lamina.(4) Chronic inflammatory changes which weretransmural but principally involved the media,especially in relation to damaged internal elasticlamina.The inflammatory cell infiltrate was predomi-

nantly composed of lymphocytes and epithelioidcells along with a few plasma cells and variablenumbers of Langhans giant cells. Foci offibrinoid degeneration were seen in the deeperlayers of the fibrosed intima and in the media insome sections. In many of the affected vesselssevere atheroma complicated the histologicalpicture. The terminal segment of the rightinternal carotid artery showed no evidence ofGCA, containing only red blood clot (notthrombus).Examination of the globes showed: (1) Oede-

matous optic discs but normal and patent centralretinal vessels. (2) Infarction in the retro-laminar region of the optic nerve (Figs 1 and 2).(3) Chronic inflammatory changes ofthe adjacentposterior ciliary arteries. Many of the vesselswere occluded.

CASE 2An 86-year-old woman presented in June 1988with an eight-day history of loss of vision in her

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Pattern ofarterial involvement ofthe head, neck, and eyes in giant cell arteritis: three case reports

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X'w,Figure I Histologicalsection ofright optic nerve.(H-E and eosin stain,x20.)

right eye. This was associated with decreasedappetite, weakness, jaw claudication, generalmalaise, and shoulder stiffness.She had had a right cataract extraction with

intraocular lens implant in August 1987. She wasdependent on her right eye for vision because herleft eye had a dense cataract. Visual acuity was6/60 in the right eye and perception oflight in theleft. The right pupil was unreactive to light, butpupil adhesions to the anterior vitreous werepresent. The left pupil reacted sluggishly tolight. Her optic discs were pale but not swollen.She had a total bilateral ophthalmoplegia. Thepatient did not co-operate for visual field testing.A provisional diagnosis of posterior ischaemicoptic neuropathy was made at this time.The Westergren ESR was 110 mm/h, and high

dose corticosteroid treatment was started. Thediagnosis of GCA was confirmed by temporalartery biopsy. X-rays of the skull, opticforamina, and pituitary fossa were normal.Unfortunately there was further visual reductionto no perception of light in the right eye andperception of light in the left eye.A CT scan suggested symmetrical cortical

infarctions round the calcarine fissure, features

consistent with cortical blindness (Fig 3). How-ever, this was not confirmed by post-mortemexamination performed three months later (seebelow).

In September 1988 she had a massive gastro-intestinal haemorrhage, probably a complicationof high-dose steroid treatment, and died.

Post-mortemfindings. Microscopically, sectionsof the cerebral cortex and basal ganglia showed afew tiny old infarcts. The calcarine cortices werenormal. Neither the arteries on the surface of thebrain and subarachnoid space nor within thebrain substance showed any evidence of arteritis.The carotid bifurcations were grossly athero-matous but not particularly stenosed. One or twosmall arterial twigs in the surrounding connec-tive tissue were thick walled, had probably beenthrombosed, and showed sparse lymphocyticinfiltration of their walls, most marked in theadventitia. No giant cells were identified inrelation to the internal elastic lamina. Similarchanges were present in the arterial twigs accom-panying the optic nerves, including the ophthal-mic artery but not the central retinal artery. Asolitary microscopic infarct was present in theright optic nerve in the retrolaminar region. Thus

Figure 2 Histologicalsection of left optic nerve.(H-E and myelin stain,xSS.)

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Butt, Cullen, Mutlukan

Figure 3 Case 2. CT scan.

these chronically inflamed scarred small arterieswere considered to represent a burnt out versionofGCA.

CASE 3A 65-year-old woman presented in 1964 with athree-week history of severe frontal headacheassociated with temporal tenderness. There wereno ocular symptoms and her vision was normal.The Westergren ESR was 83 mm/h, and biopsyof her right temporal artery showed classicalchanges of GCA. Corticosteroid treatment wasstarted and led to symptomatic improvement.Seven years later, while still on carefully

monitored treatment, she presented with anepisode of visual loss in the right eye, acuityfalling from previously recorded 6/6, N5, to6/60, N18. She had a right afferent pupillarydefect and a swollen pale disc. The ESR was 45mm/h. Details up to this time were reported byCullen." A repeat right temporal artery biopsyshowed no evidence of active arteritis. A diag-nosis of right anterior ischaemic optic neuro-pathy was made. Her dosage of steroids wasincreased, but unfortunately there was no visualrecovery.

It has been suggested that despite negativetemporal artery biopsies'8 the disease in this caseremained active in the posterior ciliary arteries,and this was reflected by moderately raised ESRreadings.

Figure 4 Case 3. CT scan.

In July 1987 she was seen again with a 10-dayhistory of loss of vision in her left eye which hadoccurred on awakening. The ESR was 75 mm/h.On examination her vision was perception oflight in the right eye and hand movements in theleft eye. The left pupil was reactive to light andthe disc was normal. A CT scan showed markedwidespread cortical atrophy and focal areas oflow density related to the tip of the occipital poleon both sides, left more than right, which wereconsistent with bilateral occipital infarction (Fig4). She died a year later. A necropsy was notperformed.

DiscussionIn our three patients changes typical of GCAwere present in the temporal arteries, proved bytemporal artery biopsy in cases 2 and 3 and bypositive histological changes in the post-mortemexamination ofthe superficial temporal and otherarteries in case 1. Necropsy carried out on twopatients with proved cerebrovascular diseaserelated to GCA failed to identify intracranialGCA. The cerebral infarcts found were theresults of embolism from extracranial vessels,which were damaged by arteritis and atheromaand contained thrombus, rather than from lesionsofthe intracerebral arteries themselves. This wasconfirmed by the multiple, haemorrhagic natureof the lesions.

In case 3 therewas evidence ofcerebral involve-ment on CT scan. However, it is not possible tosay whether this was due to lesions of theintracranial or extracranial vessels. This casetherefore neither confirms nor contradicts ourinitial hypothesis that cerebrovascular disease inGCA may not be due to intracranial arteritis.However, whatever the pathogenesis of thispatient's condition, it must allow for the possi-bility of simultaneous infarction of both occipitallobes. The simplest explanation would be em-bolism (due to thrombus from arteritic vertebralarteries) to the bifurcation of the basilar artery,with fragments of embolus being shed more orless symmetrically between the two posteriorcerebral arteries.7121415

Intracranial vessel involvement is suggested(although infrequently) in a number ofpublishedarticles.'"9 However, the basis for such a conclu-sion is not always certain. There is clinical, post-mortem, and angiographic support for intra-cranial involvement, but post-mortem sLudiesare the most reliable source of information, asangiographic and clinical evidence may be opento the criticism that coincidental arteriosclerosisor some other disease process could be the causeof cerebral involvement. Post-mortem evidencehas shown involvement of the intracranial part ofthe internal carotid, vertebral, and basilararteries and arteries of the base of the brain.1 2 5 7-9Angiographic abnormalities have been notedconsisting ofeither irregular areas of stenosis anddilatations'o or a saw-toothed appearance' inintracranial arteries. Hinck et al'0 found no post-mortem evidence of GCA related to theangiographic abnormalities, whereas Hirschet aP confirmed their angiographic findings bypathological examination, which showedfeatures consistent with GCA in the anterior,

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Pattern ofarterial involvement of the head, neck, and eyes in giant cell arteritis: three case reports

middle cerebral, and entire part of the basilarartery. The authors suggest that angiographicabnormalities may in fact be non-specific andrelated to embolic phenomena, thrombi, oratheroma, and may not always be conclusive ofintracranial pathology due to GCA." Similarcerebral angiographic findings have beenreported in other types of arteritis such as

polyarteritis nodosa, rheumatoid arthritis, andsystemic lupus erythematosus.6

In two reports,34 in contrast to those alreadymentioned, there does appear to be post-mortemevidence for intracranial involvement of smallercerebral vessels. However, in one ofthese reportsthe findings relate to minor changes in smallvessels (with dubious symptomatic conse-

quences),3 and in the other instance the clinicalfeatures are not entirely characteristic of GCAand another diagnosis cannot be excluded.4 Suchchanges may in fact be the result of otherconditions such as polyarteritis nodosa,Buerger's disease (endarteritis obliterans), tuber-culosis, and Takayashu's syndrome (pulselessdisease).7 In these four conditions giant cells withinflammatory changes have been noted in patho-logical specimens, but clinical symptoms andsigns help to differentiate them from GCA.However, signs and symptoms occasionally over-lap owing to similar sites of involvement.7A considerable literature tends to support our

findings."1618 Extracranial vertebral artery andinternal carotid artery involvement has beennoted, giving rise to cerebral manifestationseither through thromboembolic episodes, directextension of thrombus from the site of arteritisinto the artery of supply, or by reduced vertebralartery perfusion aggravated by concomitant arte-ritic or non-arteritic involvement of the internalcarotids or insufficiency of the circle of Willis.The vertebral arteritic changes reported by

Crompton3 and Wilkinson and Russell'2 werenoted to have a sharply defined upper borderabout 5mm above the point ofdural perforation.The terminal segment of the internal carotidartery within the dura was never involved.'2"'4This was thought to reflect the close correlationbetween susceptibility ofGCA and the amount ofelastic tissue in the media and adventitia of theindividual arteries of the head and neck, as GCAis thought to be an autoimmune reaction involv-ing arterial elastic tissue. Major transformationin the arterial muscle coats occurs as they enterthe skull, with a gross diminution in the amountof elastic tissue. Intracranial arteries may bespared owing to the presence of little or no elastictissue in their coats. 2 This would also explain our

pathological findings in the optic nerves, whichshowed infarction in the retrolaminar region dueto GCA affecting the posterior ciliary arteries.In both of our post-mortem examinations thecentral retinal vessels were patent.Cullen" and MacFaul2" have reported in an-

other case a similar picture of involvement of theposterior ciliary arteries and of the origin of thecentral retinal artery outside the globe andoutside the optic nerve. The central retinal arterywithin the optic nerves as well as the retinalvessels within the globe and the choroidal vesselswere all patent. This is important in view of thefact that the wall of the central retinal arterywithin the optic nerve contains very little elastictissue (in contrast to considerably more in thewalls of the ophthalmic and posterior ciliaryarteries,'2 and the internal elastic lamina ceases asthe central retinal artery emerges from the disc.

We are greatly indebted to Dr A F J Maloney and Dr A Gordon ofthe Pathology Department. We also thank Mrs M Marshall for hermany hours of secretarial assistance and Mr S Gairns, medicalphotographer, for his assistance with the prints.

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