Sudden onset of horizontal diplopia in an elderly patient can be due to giant cell arteritis. Although the patient reported was a vasculopath, a concurrent headache, mild reduction in visual acuity, scalp tenderness and raised inflammatory markers lead to a diagnosis of giant cell arteritis (GCA) and commencement of steroids. After an inconclusive temporal artery biopsy and resolution of visual acuity, the steroids were stopped, but within this time her hypertensive and diabetic treatment had been markedly disturbed. The rationale for treatment is discussed

D'Ath, P.

Wilson, C. M.

City Research Online

Wilson, C. M. & D'Ath, P. (2017). A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell Arteritis. BAOJ Ophthalmology, 2(1), 007. City Research OnlineOriginal citation: Wilson, C. M. & D'Ath, P. (2017). A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell Arteritis. BAOJ Ophthalmology, 2(1), 007. Permanent City Research Online URL: http://openaccess.city.ac.uk/16395/ Copyright & reuseCity University London has developed City Research Online so that its users may access the research outputs of City University London's staff. Copyright © and Moral Rights for this paper are retained by the individual author(s) and/ or other copyright holders.  All material in City Research Online is checked for eligibility for copyright before being made available in the live archive. URLs from City Research Online may be freely distributed and linked to from other web pages. Versions of researchThe version in City Research Online may differ from the final published version. Users are advised to check the Permanent City Research Online URL above for the status of the paper.EnquiriesIf you have any enquiries about any aspect of City Research Online, or if you wish to make contact with the author(s) of this paper, please email the team at publications@city.ac.uk.A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell ArteriisClare M Wilson and Penny J D’Ath, BAOJ Ophthalmol 2017 2: 12: 007BAOJ Ophthalmol, an open access journal                                                                                                                                                               Volume 2; Issue 1; 007Clare M Wilson1* and Penny J D’Ath21Chelsea and Westminster Hospital NHS Foundaion Trust, 369 Fulham Road, London, SW10 9NH, United Kingdom2Division of Optometry & Visual Sciences, City, University of London, Northampton Square, London, EC1V 0HB, United Kingdom BAOJ Ophthalmology*Corresponding author: Clare M Wilson, Chelsea and Westminster Hospital NHS Foundaion Trust, 369 Fulham Road, London, SW10 9NH, United Kingdom, E-mail: clarewil25@yahoo.comSub Date: 16 January, 2017, Acc Date: 18 January, 2017, Pub Date: 26 January, 2017.Citaion: Clare M Wilson and Penny J D’Ath (2017) A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell Arteriis. BAOJ Ophthalmol 2: 007.Copyright: © 2017 Clare M Wilson and Penny J D’Ath. This is an open-access aricle distributed under the terms of the Creaive Commons Atribuion License, which permits unrestricted use, distribuion, and reproducion in any medium, provided the original author and source are credited.Case ReportSummarySudden onset of horizontal diplopia in an elderly patient can be due to giant cell arteritis. Although the patient reported was a vasculopath, a concurrent headache, mild reduction in visual acuity, scalp tenderness and raised inlammatory markers lead to a diagnosis of giant cell arteritis (GCA) and commencement of steroids. Ater an inconclusive temporal artery biopsy and resolution of visual acuity, the steroids were stopped, but within this time her hypertensive and diabetic treatment had been markedly disturbed. he rationale for treatment is discussed.Case ReportA 70 year old lady presented to her routine diabetic retinopathy clinic appointment complaining of recent onset diplopia and mild headache. As well as having poorly controlled diabetes, she also sufered a myriad of other related illnesses including hypertension, renal failure and peripheral vascular disease which had resulted in a let below knee amputation. She had endured a prolonged hospital stay of over eight months duration the previous year due to infected leg ulcers.On direct questioning, her mild headache was globally distributed, although she reported some tenderness on combing her hair in the let temporal region. he diplopia was horizontal and had started that morning. She had no recent weight loss, anorexia, malaise, fever or joint pains. She had no jaw claudication or myalgia.On examination, her best corrected visual acuity was 6/6 in the right eye and 6/12 in the let eye. he let eye vision had decreased slightly from 6/9+1 at the previous visit four months earlier. here was no relative aferent pupillary defect and Ishihara colour plate testing revealed full colour vision bilaterally. Ocular motility testing revealed a let esotropia and decreased let lateral gaze. Anterior segments were healthy and intraocular pressures were within the normal range. Dilated examination revealed bilateral non proliferative diabetic retinopathy, no clinically signiicant macular oedema and healthy pink optic discs. here was mild temporal artery tenderness on the let hand side. Neither temporal artery was pulsatile. Humphrey automated visual ield testing was unremarkable. A diagnosis of sixth nerve palsy possibly secondary to GCA was made.On presentation, her blood pressure and blood glucose were well controlled, urinalysis was unremarkable, ESR was 54 and CRP was 18. Platelets were within normal range. She was commenced on oral prednisolone, adcal and lansoprazole and arrangements were made for a temporal artery biopsy to be performed. She was already taking aspirin. She was given a Fresnel prism to alleviate the diplopia.Temporal artery biopsy (TAB) was challenging and histopathology revealed a venular structure had been excised. he diagnosis was therefore still not clear two weeks following presentation. Her visual acuity had improved to 6/9 in the let eye within this time. he high dose oral steroids were disrupting her blood pressure and blood sugar control signiicantly and a decision to gradually reduce the prednisolone dose was made ater discussions with the rheumatology team. Her hypertension and diabetes reverted to improved control and the headaches subsided. he sixth nerve palsy was almost completely resolved six weeks following the onset of the condition. A retrospective diagnosis of microvascular sixth nerve palsy was assigned to this vasculopathic patient.Discussionhe American College of Rheumatology 1990 Criteria classiies GCA as follows [1]:1. Age of onset ≥50 years of age2. New headache3. Temporal artery tender/reduced pulsation4. Elevated ESR, deined as 50mm/h (Westergren method)BAOJ Ophthalmol, an open access journal                                                                                                                                                               Volume 2; Issue 1; 007Page 2 of 3Citaion: Clare M Wilson and Penny J D’Ath (2017) A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell Arteriis. BAOJ Ophthalmol 2: 007.5. Abnormal arterial biopsy – showing necrotising vasculitis with predominant mononuclear cell iniltration or granulomatous inlammationhis patient was 70 years old, had a new onset headache, temporal artery tenderness and absent temporal artery pulsation. Her ESR and CRP were raised. he upper limit for normal ESR has also been described to increase with age and has been deined as age divided by 2 for men and age plus 10 divided by 2 for women. As this lady was 70 years old, her upper limit according to this method was 40mm/h. ESR is traditionally the most important laboratory test for GCA. However, there are many reports of patients with a normal ESR yet positive temporal artery biopsy. here are also many other reasons for this lady who had been recently hospitalised with leg ulcers, to have a raised ESR. CRP is an acute phase protein released by liver hepatocytes in response to inlammation. It has been found that CRP is more speciic for GCA, and that a combination of raised ESR and raised CRP is the most speciic [2]. GCA predominantly afects Caucasians over 50 years, with women afected at least twice as oten as men. Monozygotic twin concordance and familial clustering suggests an inherited component. he HLA-DR4 haplotype has been associated with GCA, as have the intercellular adhesion molecule-1 (ICAM-1) genes [3]. TAB is the gold standard for diagnosing GCA, yet has limited sensitivity, and can be challenging particularly when the artery is non-pulsatile due to extensive disease. It is suggested that a second biopsy on the opposite side should be performed following an unsuccessful irst biopsy, although second attempts only give a low positive yield, for example 9% of cases in one study [4]. Ultrasonography aids artery detection prior to biopsy, and it has been suggested that duplex ultrasonography should be the irst-line investigation for diagnosing GCA, with biopsy being reserved for patients with a negative scan [5]. Although TAB has a low complication rate of 0.5%, there is a potential risk of facial nerve damage or stroke [6-8]. Halo sign of oedema is a hypoechoic halo in the artery wall, and is probably caused by oedema. Oral prednisolone is irst-line acute therapy for GCA, but the initial starting dose varies widely. Fraser et al recommend a 3-day induction dose of IV methylprednisolone at 15mg/kg/d followed by oral prednisolone maintenance therapy at 1mg/kg/d initially for GCA with visual loss [9]. his is echoed by the Royal College of Physicians [10]. Despite treatment with IV corticosteroids, visual acuity will reduce in 27% of patients with GCA (mostly within the irst six days), however the treatment is efective at reducing the likelihood of fellow eye involvement. Without treatment, the risk of fellow eye involvement is 54% to 95% [10].he Royal College of Physicians guideline entitled “Diagnosis and management of GCA” includes features to aid early recognition of GCA [10]:1. Abrupt onset headache2. Scalp tenderness3. Jaw and tongue claudication4. Visual symptoms5. Constitutional symptoms6. Polymyalgia symptoms7. Limb claudicationOf these features, the reported patient had only scalp tenderness; her presenting features being horizontal diplopia. Extraocular motility disorders in GCA are thought to be due to extraocular muscle ischaemia caused by thrombotic occlusion of the respective muscular arteries [2]. GCA is a systemic vasculitis, preferentially involving medium and large sized arteries, particularly those of the extracranial branches of the carotid artery. It can cause arteritic anterior ischaemic optic neuropathy (AAION). Other causes of AAION are rare and include polyateritis nodosa, systemic lupus erythematosis and herpes zoster. he histopathological hallmark of GCA is granulomatous inlammation surrounding the internal elastic lamina in medium and large vessels. he arterial media is most commonly involved, but all three layers may be afected. Macrophages, lymphocytes and ibroblasts make up the inlammatory iniltrate. Multinucleated giant cells may or may not be present, and are pathognomic of disease, fragmenting the internal elastic lamina. Visual loss has been associated with presence of giant cells [11]. “Skip lesions” may occur due to varied temporal and spatial disease. Inlammation leads to local vascular damage and intimal hyperplasia which may result in stenosis or occlusion. here is also thought to be an angiogenic role in GCA. he multinucleated giant cells produce platelet derived growth factor (PDGF) and vascular endothelial growth factor (VEGF). Normal arteries have avascular media and intima with a capillary network (vasa vasorum) in the adventitia only. In GCA, new capillaries appear in the media and intima and are more numerous in arteries with luminal stenosis secondary to advanced intimal hyperplasia [12]. It could be argued that this patient had only minimal visual loss and was at low risk of GCA. Visual acuity can be normal in GCA. In a study of 123 eyes with visual acuity loss due to GCA, 21% had a visual acuity of 6/12 or better. but 62% had a visual acuity of 6/60 or worse [13]. Conversely, 21% of patients with GCA have occult GCA – that is visual loss and a positive temporal artery biopsy without any of the systemic features of GCA.A luorescein angiogram may have aided diagnosis. he main lesion in GCA causing visual loss is thrombosis and occlusion of the posterior cerebral arteries giving choroidal illing defects. However, this must be done in the early stages as collateral circulation resolves the illing defects in time. Evidence concerning possible increased mortality rate in patients with GCA over the normal population is limited and contradictory. It seems that death due to cardiovascular disease is increased in patients with GCA [14].Conclusionshe treatment of the reported lady with steroids based on new BAOJ Ophthalmol, an open access journal                                                                                                                                                               Volume 2; Issue 1; 007Page 3 of 3Citaion: Clare M Wilson and Penny J D’Ath (2017) A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell Arteriis. BAOJ Ophthalmol 2: 007.onset diplopia with a headache with marginally raised ESR and CRP is controversial. However, the devastating consequences of untreated GCA may warrant a lower threshold for intervention in these cases. Important points to note:1. GCA is a challenging diagnosis2. ESR is a non speciic indicator of inlammation – increases in malignancy, infection, CT disorders, trauma, anaemia, hypercholesterolaemia3. CRP and platelet count can help with diagnosing GCA4. USS for guided biopsy, and potentially for optical biopsy5. he possible side efects of steroids should not be underestimated.References1. Hunder G G, Bloch D A, Michel B A, Stevens M B, Arend W P, et al. (1990) The American College of Rheumatology 1990 criteria for the classiicaion of giant cell arteriis. Arthriis Rheum 33(8): 1122-1128.2. Hayreh SS (2009) Ischaemic opic neuropathy. Prog Rein Eye Res 28(1): 34-62.3. Chew S S, Kerr N M, Danesh-Meyer H V (2009) Giant cell arteriis. J Clin Neurosci 16(10): 1263-1268.4. Hayreh S S, Podhajsky P A, Zimmerman B (2010) Nonarteriic anterior ischemic opic neuropathy: ime of onset of visual loss. Am J Ophthal-mol 124(5): 641-647.5. Ball E L, Walsh S R, Tang T Y, Gohil R, Clarke J M (2010) Role of ultra-sonography in the diagnosis of temporal arteriis. Br J Surg 97(12): 1765-1771.6. Ikard RW (1988) Clinical eicacy of temporal artery biopsy in Nash-ville, Tennessee. South Med J 81(10): 1222-1224.7. Bhai M T, Goldstein M H (2001) Facial nerve injury following superi-cial temporal artery biopsy. Dermatol Surg 27(1): 15-17.8. Haist S A (1985) Stroke ater temporal artery biopsy. Mayo Clin Proc 60(8): 538.9. Fraser J A, Weyand C M, Newman N J, Biousse V (2008) The treatment of giant cell arteriis Rev Neurol Dis 5(3): 140-152.10. Dasgupta B (2010) (On behalf of the GCA Guideline Development Group). Concise guidance: diagnosis and management of giant cell arteriis. Clin Med (Lond) 10(4): 381-386.11. Chatelain D, Duhaut P, Schmidt R Loire R, Bosshard S, et al (2009) Pathological features of temporal arteries in paients with giant cell arteriis presening with permanent visual loss. Ann Rheum Dis 68(1): 84-88.12. Mitchell E B, Cestari D M (2009) Giant cell arteriis and angiogenesis: a review. Semin Ophthalmol 24(3): 190-193.13. Hayreh S S, Podhajsky P A, Zimmerman B (1998) Occult giant cell arte-riis: ocular manifestaions. Am J Ophthlamol 125(4): 521-526.14. Philip R, Luqmani R (2008) Mortality in systemic vasculiis: a systemic review. Clin Exp Rheumatol 26(5 Suppl 51): S94-104.

A Case of Sixth Cranial Nerve Palsy and Suspected Giant Cell Arteritis

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