Pattern of Arterial Involvement Ofthe
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368 BritishJournal ofOphthalmology, 1991, 75, 368-371 CASE REPORTS Br J Ophthalmol: first published as 10.1136/bjo.75.6.368 on 1 June 1991. Downloaded from Pattern of arterial involvement of the head, neck, and eyes in giant cell arteritis: three case reports Z Butt, J F Cullen, E Mutlukan Abstract arteries at necropsy showed characteristic The findings oftwo post-mortem examinations changes ofGCA (see below). and one CT scan ofpatients with biopsy proved Post-mortenfindings. Macroscopically the main giant cell arteritis (GCA) are presented. The arteries at the base ofthe brain were virtually free presence or absence of intracranial involve- from atheroma, but a plug ofrather firm clot was ment in GCA is discussed. present in the stump ofthe right internal carotid. The circle of Willis was normally constituted. Several haemorrhagic infarcts were noted in the Giant cell arteritis (GCA) is rarely fatal, and cerebrum (frontal, parietal, temporal, and references to the condition in post-mortem occipital lobes) and cerebellum. Microscopic material are uncommon.'-16 However, this may examination confirmed that these were be related to non-recognition of a fatal outcome very recent, practically terminal, infarcts. in patients with GCA and because post-mortem Occasionally small meningeal arteries overlying examinations of elderly patients dying from the cortical infarcts were noted to contain recent vascular disorders are not routinely carried out. thrombus, but in none of the sections was there GCA may be concealed among the cases diag- evidence ofgiant cell arteritis. nosed as ischaemic catastrophes due to arterio- Sections of the temporal, ophthalmic, verte- sclerosis. " bral, internal carotid (in neck), external carotid, Patients suffering from GCA have a range of left common carotid, and coronary arteries symptoms including headache, jaw claudication, showed changes typical of giant cell arteritis. fever, anorexia, and loss of vision. However, Histological abnormalities noted included: atypically they may with present symptoms of (1) Diffuse fibrous thickening of the intima http://bjo.bmj.com/ cerebral ischaemia, the aetiology ofwhich may be associated with mucinous changes and narrowing confused with that of other conditions such as or occlusion of the lumen. (2) The presence of arteriosclerosis. We suggest that such atypical organising mural thrombus (both internal caro- symptoms may be secondary to embolism and tid arteries). (3) Fragmentation and dis- that the diagnosis of GCA should not be over- appearance of the internal elastic lamina. looked. A raised erythrocyte sedimentation rate (4) Chronic inflammatory changes which were together with positive findings on temporal transmural but principally involved the media, on September 30, 2021 by guest. Protected copyright. artery biopsy should help in confirming or refut- especially in relation to damaged internal elastic ing the diagnosis. GCA is treatable, and treat- lamina. ment leads to symptomatic improvement. The inflammatory cell infiltrate was predomi- nantly composed oflymphocytes and epithelioid cells along with a few plasma cells and variable Case reports numbers of Langhans giant cells. Foci of fibrinoid degeneration were seen in the deeper CASE 1 layers of the fibrosed intima and in the media in An 80-year-old man presented in 1973 with a some sections. In many of the affected vessels three-month history of temporal headaches, severe atheroma complicated the histological influenza-like symptoms, and anorexia. Acute picture. The terminal segment of the right loss of vision in the right eye had occurred two internal carotid artery showed no evidence of days previously and acute loss ofvision in the left GCA, containing only red blood clot (not eye followed on the day of presentation. Visual thrombus). acuity was no perception of light in the right eye Examination of the globes showed: (1) Oede- and perception oflight in the left eye. His pupils matous optic discs but normal and patent central did not react to light, and there was bilateral retinal vessels. (2) Infarction in the retro- Princess Alexandra Eye oedema ofthe discs. The Westergren erythrocyte laminar region of the optic nerve (Figs 1 and 2). Pavilion, Royal sedimentation rate was 90 mm/h. Infirmary, Edinburgh Anterior (3) Chronic inflammatory changes ofthe adjacent EH3 9HA ischaemic optic neuropathy secondary to GCA posterior ciliary arteries. Many of the vessels Z Butt was diagnosed, and high-dose corticosteroid were occluded. J F Cullen treatment was E Mutlukan initiated. His mental condition deteriorated over the next few days, and he was Correspondence to: Dr Z Butt, FRCS. thought to have sustained a cerebrovascular CASE 2 Accepted for publication accident. He became comatose and died secon- An 86-year-old woman presented in June 1988 14 February 1991 dary to hypostatic pneumonia. His temporal with an eight-day history of loss of vision in her Pattern ofarterial involvement ofthe head, neck, and eyes in giant cell arteritis: three case reports 369 Br J Ophthalmol: first published as 10.1136/bjo.75.6.368 on 1 June 1991. Downloaded from *.~~ ~ ~~A..........~ ~~~~~~~~~~~~~ ~~~~~~~~~~~~~~ . ..;..... ,, .. I - ,.. !.4 Figure I Histological section ofright optic nerve. (H-E and eosin stain, X'w, x20.) right eye. This was associated with decreased consistent with cortical blindness (Fig 3). How- appetite, weakness, jaw claudication, general ever, this was not confirmed by post-mortem malaise, and shoulder stiffness. examination performed three months later (see She had had a right cataract extraction with below). intraocular lens implant in August 1987. She was In September 1988 she had a massive gastro- dependent on her right eye for vision because her intestinal haemorrhage, probably a complication left eye had a dense cataract. Visual acuity was of high-dose steroid treatment, and died. 6/60 in the right eye and perception oflight in the Post-mortemfindings. Microscopically, sections left. The right pupil was unreactive to light, but of the cerebral cortex and basal ganglia showed a pupil adhesions to the anterior vitreous were few tiny old infarcts. The calcarine cortices were present. The left pupil reacted sluggishly to normal. Neither the arteries on the surface ofthe light. Her optic discs were pale but not swollen. brain and subarachnoid space nor within the She had a total bilateral ophthalmoplegia. The brain substance showed any evidence ofarteritis. patient did not co-operate for visual field testing. The carotid bifurcations were grossly athero- A provisional diagnosis of posterior ischaemic matous but not particularly stenosed. One or two optic neuropathy was made at this time. small arterial twigs in the surrounding connec- The Westergren ESR was 110 mm/h, and high tive tissue were thick walled, had probably been dose corticosteroid treatment was started. The thrombosed, and showed sparse lymphocytic diagnosis of GCA was confirmed by temporal infiltration of their walls, most marked in the artery biopsy. X-rays of the skull, optic adventitia. No giant cells were identified in foramina, and pituitary fossa were normal. relation to the internal elastic lamina. Similar Unfortunately there was further visual reduction changes were present in the arterial twigs accom- to no perception of light in the right eye and panying the optic nerves, including the ophthal- perception oflight in the left eye. mic artery but not the central retinal artery. A http://bjo.bmj.com/ A CT scan suggested symmetrical cortical solitary microscopic infarct was present in the infarctions round the calcarine fissure, features right optic nerve in the retrolaminar region. Thus on September 30, 2021 by guest. Protected copyright. Figure 2 Histological section ofleft optic nerve. (H-E and myelin stain, xSS.) 370 Butt, Cullen, Mutlukan In July 1987 she was seen again with a 10-day history of loss of vision in her left eye which had occurred on awakening. The ESR was 75 mm/h. Br J Ophthalmol: first published as 10.1136/bjo.75.6.368 on 1 June 1991. Downloaded from On examination her vision was perception of light in the right eye and hand movements in the left eye. The left pupil was reactive to light and the disc was normal. A CT scan showed marked widespread cortical atrophy and focal areas of low density related to the tip ofthe occipital pole on both sides, left more than right, which were consistent with bilateral occipital infarction (Fig 4). She died a year later. A necropsy was not performed. Discussion Figure 3 Case 2. CT scan. In our three patients changes typical of GCA were present in the temporal arteries, proved by these chronically inflamed scarred small arteries temporal artery biopsy in cases 2 and 3 and by were considered to represent a burnt out version positive histological changes in the post-mortem ofGCA. examination ofthe superficial temporal and other arteries in case 1. Necropsy carried out on two patients with proved cerebrovascular disease CASE 3 related to GCA failed to identify intracranial A 65-year-old woman presented in 1964 with a GCA. The cerebral infarcts found were the three-week history of severe frontal headache results of embolism from extracranial vessels, associated with temporal tenderness. There were which were damaged by arteritis and atheroma no ocular symptoms and her vision was normal. andcontained thrombus, ratherthanfromlesions The Westergren ESR was 83 mm/h, and biopsy ofthe intracerebral arteries themselves. This was of her right temporal artery showed classical confirmed by the multiple, haemorrhagic nature changes of GCA. Corticosteroid treatment was ofthe lesions. started and led to symptomatic improvement. In case 3 there was evidence ofcerebralinvolve- Seven years later, while still on carefully ment on CT scan. However, it is not possible to monitored treatment, she presented with an say whether this was due to lesions of the episode of visual loss in the right eye, acuity intracranial or extracranial vessels. This case falling from previously recorded 6/6, N5, to therefore neither confirms nor contradicts our 6/60, N18. She had a right afferent pupillary initial hypothesis that cerebrovascular disease in http://bjo.bmj.com/ defect and a swollen pale disc.