Age and Ageing Advance Access originally published online on October 25, 2007
Age and Ageing 2007 36(6):695-697; doi:10.1093/ageing/afm136
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Case Reports |
Insidious posterior circulation stroke with rapid deterioration due to vertebral giant cell arteritis
1 SpR, Care of Elderly, University Hospitals of North Staffordshire, Stoke on Trent, West Midlands, UK
2 Consultant Geriatrician, New Cross Hospital, Wolverhampton, West Midlands, UK
Address correspondence to: Anil Kumar. Tel: 01782-715444; Fax: 01782-553587. Email: anilkumaruk{at}hotmail.com
Abstract
We report a patient with a posterior circulation stroke that presented insidiously, progressed rapidly and had an unusual aetiology of vertebral giant cell arteritis that was not recognised in a timely fashion. General awareness of this cause might alert clinicians to consider the diagnosis in similar cases, as prompt intervention may improve outcomes.
Keywords: giant cell arteritis, bilateral vertebral artery occlusion, stroke in the posterior circulation, elderly
A 74-year-old Caucasian gentleman was admitted with a 5-day history of malaise, and a 1-day history of slurring of speech. There was no history of any infection, limb weakness, nausea, vomiting or headache. He consumed approximately 70 units of alcohol per week, did not smoke, and lived alone. He had a history of hypertension, which was well controlled on doxazosin 4 mg/day and atenolol 50 mg/day. On admission, he had a staccato speech, horizontal nystagmus, intention tremor and a broadbased gait. A diagnosis of a cerebellar stroke was made. He was commenced on aspirin and transferred to the stroke unit. His routine blood tests, ECG and a chest x-ray were unremarkable. His CRP was elevated at 119 mg/l. A CT head scan was pending. Within hours he became very unwell with vomiting and shortness of breath. His oxygen saturation fell to 60%. He had a tachypnoea and audible crepitations in the chest and had developed an expressive dysphasia. A clinical diagnosis of aspiration pneumonia was made which was treated with high-flow oxygen and intravenous antibiotics. A portable chest x-ray showed patchy changes to the bases. As his condition continued to deteriorate, he was intubated in the ward and transferred to the ITU. A CT head scan showed that he had a right occipital infarct with cerebellar involvement (Figure 1). On the fourth day, while intubated, he had wide fluctuations in his blood pressure and pulse rate. An urgent repeat CT head scan showed a marked deterioration compared to his earlier scan. There was extensive low attenuation in both cerebellar hemispheres with complete infarction of the right cerebellar hemisphere. The area of infarction extended to involve most of the brainstem and left temporal lobe with gross hydrocephalus (Figure 2). The neurosurgical team who were contacted advised that surgical intervention would be futile for his hydrocephalus as his stroke had progressed to involve the brainstem, which has a bleak prognosis [1]. He was started on high-dose dexamethasone to decrease his cerebellar oedema.
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He continued to deteriorate and 2 days later the ventilator was turned off as there were no brainstem signs of life, and he died peacefully. The post mortem examination confirmed the radiological findings of bilateral cerebellar infarcts and gross hydrocephalus. It also showed patchy thrombotic occlusion of both vertebral arteries with histological features of florid giant cell arteritis (GCA) (intimal proliferation of a cellular infiltrate of lymphocytes and multinucleate giant cells). It was felt that the thrombotic material had developed on the background of the vasculitic process in the vertebral arteries.
GCA is a rare cause for stroke in the posterior circulation [2]. Sandercock et al. studied 244 cases of new cerebral stroke and found only two cases where the aetiology was arteritis [3]. Ruegg et al. reported three cases of GCA causing occlusion of bilateral vertebral arteries [4]. In this review article, they described five other cases of giant cell arteritis causing bilateral vertebral artery occlusion (BVAO). A review of the literature showed that in bilateral vertebral arteries occlusion due to giant cell arteritis, headache and raised ESR (above 45 mm) were present in 100% of cases; fever was present in only 50% and the overall mortality was 75%. In comparison, in BVAO due to atheromatous disease (studied in 110 cases), headache was present in 22%, none had fever, and the overall mortality was 19%. No data about ESR was found in the atheromatous group. Unfortunately, the ESR was not done in our patient but it may be normal in 22% of cases [5] which can be misleading. Concentrations of acute phase proteins, especially the CRP, may be raised but are not considered more helpful than a raised ESR [6]. Early diagnosis of giant cell arteritis is necessary for immediate initiation of intensive anti-inflammatory and immunosuppressive treatment, without which progressive deterioration and systemic involvement are likely to be fatal [7]. De Reuck and Vander [8] have reported that management of hydrocephalus following cerebellar infarction should be treated by surgical decompression which can be life saving.
We subsequently spoke to the patient's relatives who confirmed that the patient had a slow onset of slurring of speech for the last 5 weeks, and for the last 6 months he had been complaining of neck and shoulder pain. He refused to see doctors. These features would be consistent with the development of polymyalgia rheumatica, which subsequently progressed to cause an arteritis. With hindsight, if the patient's history was clearer at the time of the presentation we might have been alerted to the raised CRP as a possible cause of arteritis at his presentation. However, introduction of high-dose intravenous steroids may not have helped in this patient due to a rapid deterioration in his clinical condition from the secondary obstructive hydrocephalus.
Another point of interest is his initial deterioration, which was with vomiting and laboured breathing. A diagnosis of aspiration pneumonia was made which was treated with intravenous antibiotics and high-flow oxygen. His chest x-ray did not show any frank consolidation, his sputum sample did not grow any organisms, and the post mortem examination only revealed congested lungs but no consolidation. Hence, it can be inferred that his rapid clinical deterioration with initial hypoxia could be due to brainstem compression and not aspiration pneumonia.
This patient presented with insidious posterior circulation symptoms. Because of his heavy alcohol intake and slow onset of cerebellar symptoms, an alcohol-related problem was also suspected. His rapid deterioration was due to bilateral cerebellar infarcts causing hydrocephalus. This was not recognised clinically due to the difficulty of neurological assessment in patients on a ventilator. At the time of admission he did not venture the symptoms of shoulder and neck pain, which could have alerted the doctors of suspected vasculitis, and appropriate medication could have been started.
Following his ventilation, we were alerted that his stroke may have extended only by detection of wide fluctuations in his blood pressure and pulse rate. Even though a repeat urgent CT head scan revealed hydrocephalus with cerebellar infarct, surgical intervention could not be done to relieve his symptoms as the brainstem was already involved. He was initiated on high-dose dexamethasone, but it made no difference to his clinical condition. Recognition of arteritis a little earlier with early neurosurgical intervention [8] may have altered the course of his condition.
Approximately 4% of patients with GCA experience transient ischaemic attack (TIA), or stroke, at some point during their illness, although how many specifically result from GCA is unclear. A greater proportion of TIAs and infarctions occur in the vertebro-basilar territory than the carotid territory in patients with GCA in the general population [9]. Vertebro-basilar events may sometimes present as acute confusional states, or coma, as opposed to discrete focal syndromes [9]. Familiarity with not only typical but also the atypical presentations of GCA should facilitate improved diagnosis and management. Every patient presenting with TIA or stroke symptoms should have an ESR in addition to routine blood tests.
No conflicts of interest
- In patients over 50 years of age presenting with features of vertebro-basilar ischemia and neck pain, and/or elevated ESR giant cell arteritis should always be considered [4].
- A rapid deterioration in any patient with posterior fossa symptoms should raise the possibility of obstructive hydrocephalus.
- Even in patients with a clear-cut cause of death the post mortem examination should be encouraged as sometimes unexpected diagnoses can be found. It can be a learning point for everyone involved.
- We report this case with the hope that it will alert clinicians to atypical presentations of GCA which is an eminently treatable and reversible condition.
References
- Stroke Guidelines. Royal College of Physicians. (2006) Accessed on 28th March. London. http://www.rcplondon.ac.uk/pubs/books/stroke/stroke_guidelines_2ed.pdf.
- Mclean CA, Gonzales MF, Dowling JP. Systemic giant cell arteritis and cerebellar infarction. Stroke (1993) 24:899–902.
[Abstract/Free Full Text] - Sandercock PA, Warlow CP, Jones LN, et al. Predisposing factors for cerebral infarction: the Oxfordshire community stroke project. BMJ (1989) 298:75–80.
[Abstract/Free Full Text] - Ruegg S, Engelter S, Jeanneret C, et al. Bilateral vertebral artery occlusion resulting from giant cell arteritis: report of 3 cases and review of the literature. Medicine (Baltimore) (2003) 82:1–12.[CrossRef][Medline]
- Ellis ME, Ralston S. The ESR in the diagnosis and management of the polymyalgia rheumatica/giant cell arteritis syndrome. Ann Rheum Dis (1983) 42:168–70.
[Abstract/Free Full Text] - Swannell AJ. Fortnightly review: Polymyalgia rheumatica and temporal arteritis: diagnosis and management. BMJ (1997) 314:1329.
[Free Full Text] - Reinhard M, Schmidt D, Schumacher M, et al. Involvement of the vertebral arteries in giant cell arteritis mimicking vertebral dissection. J Neurol (2003) 250:1006–9.[CrossRef][Web of Science][Medline]
- De Reuck J, Vander Eecken H. Cerebellar infarction and internal hydrocephalus. Acta Neurol Belg (1978) 8:129–40.
- Ramachandran TS, Caselli RJ. Temporal/Giant Cell Arteritis. www.emedicine.com/neuro/topic 592.htm (Last accessed 8 October 2007).
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