Letters
279
Giant cell arteritis, oculomotor nerve palsy, and acute hearing loss L Loffredo, S Parrotto, F Violi Institute of Clinical Medicine I, University of Rome ‘La Sapienza’, Rome, Italy
Giant cell arteritis, also referred to as temporal arteritis or Horton’s disease, is a systemic vasculitis affecting large- and medium-sized arteries. Giant cell arteritis is characterized clinically by fever, anaemia, high erythrocyte sedimentation rate (ESR), and headaches (1, 2). In this letter, we report for the first time a case of histologically proven giant cell arteritis in which two rare clinical manifestations, unilateral third nerve palsy and sensorineural hearing loss, coexisted. An 80-year-old woman was admitted to hospital with a 2-week history of headaches, shooting occipital neck pain, diplopia, and hypoacusia. No past history of diabetes and hypertension was described. Physical examination revealed pupillary dilatation in the left eye, bilateral temporal artery prominence, and stiffness. Visual acuity and fundoscopy were normal. An audiogram revealed complete left-sided hearing loss and rightsided hypoacusia. At admission, tests showed no abnormalities except fibrinogen 456 mg/dL, ESR 44 mm/h (normal valueƒ15 mm/h), and C-reactive protein (CRP) 24 mg/L (normal valueƒ6 mg/L). On the second day ptosis and external strabism of the left Francesco Violi, Division IV Clinical Medicine, University ‘‘La Sapienza’’, Viale del Policlinico, 00161 Rome, Italy. E-mail:
[email protected] Received 26 March 2003 Accepted 27 February 2004
eye developed. Because a unilateral oculomotor nerve palsy associated with headache led us to suspect central neurovascular disease, the patient underwent a magnetic resonance angiography (MRA) of the head and neck revealing no abnormalities. On colour duplex ultrasono graphy no fluximetric alterations of the carotid, vertebral, ophthalmic, ciliar, or retinal arteries were seen. We hypothesized a giant cell arteritis and the patient was treated immediately with high-dose corticosteroids (50 mg prednisone daily). After 4 days of corticosteroid treatment the headaches disappeared and tests showed an ESR of 11 mm/h. After 5 days of corticosteroid therapy, a temporal artery biopsy was performed in order to make a definitive diagnosis; the resulting diagnosis was giant cell arteritis of the temporal artery. The patient was discharged after 8 days of hospitalisation with domiciliary therapy of 50 mg corticosteroids/day, pump proton inhibitors, bisphosphonates, calcium, and 1,25-OH-vitamin D for prevention of corticosteroid-induced osteoporosis. Over the following weeks, the patient developed diffuse alopecia without scab ulceration and there was an improvement in her oculomotor nerve palsy. After 1 month, the patient had normal visual acuity, while left pupillary dilatation persisted; an audiogram showed a resolving sensorineural hearing loss to the left
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ear, with a return of hearing in the 20 – 30 dB level, and normal hearing in the right ear; and the alopecia stopped. After 6 weeks the corticosteroid dose was reduced with the association of 5 mg methotrexate weekly as a corticosteroid-sparing agent. After 2 months, while the patient was well and receiving 25 mg corticosteroids daily and 5 mg methotrexate weekly, tests showed normal values for ESR, CRP, and glycaemia, but leucopenia and ipogammaglobulinaemia developed. Methotrexate was discontinued and 37.5 mg daily of corticosteroids resumed. After 3 months, tests were normal and the therapy was 16 mg methyl-prednisolone, bisphosphonates, calcium, and 1,25-OH-vitamin D. Isolated hearing loss and oculomotor nerve palsy are two rare clinical signs of giant cell arteritis. In a review of the literature we found only 20 cases of sensorineural hearing loss and more than 20 cases of third nerve palsy, though the latter included reports of pupillary involvement, with giant cell arteritis that was not histologically confirmed or associated with blindness (3 – 5). This report is the first describing a case of giant cell arteritis with both of these manifestations. The mechanism of hearing loss in giant cell arteritis is unclear; it is assumed to be secondary to inflammatory involvement of the vertebrobasilar system or the terminal cochleovestibular vessels, which subsequently leads to partial or total hearing loss (6, 7). Visual loss is the most dangerous and irreversible complication of giant cell arteritis; because an early diagnosis plays a key role in preventing blindness, it is important to recognize its various ocular manifestation (8). The oculomotor nerve palsy in our patient probably supports the Fisher hypothesis of an arteritis involving the posterior cerebral arteries, the vessels that supply blood to the third cranial nerve (9); a vasculitic process of these arteries may lead to ischaemia of vasa nervorum, provoking diplopia, palpebral ptosis, and external strabism. The differential diagnosis in patients with acute oculomotor nerve disorder is very wide and includes
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Letters
vascular aneurysm, ischaemic damage, degenerative neoplastic and metabolic processes, infective, and inflammatory disorders (10). This case illustrates that hearing loss and third nerve palsy may be two early manifestations of giant cell arteritis; these clinical signs may precede visual loss, the most dreaded and irreversible complication of this vasculitis. For this reason, patients presenting with these manifestations, with a high suspicion of giant cell arteritis, must be treated immediately with high-dose corticosteroids; although the initial treatment with 50 mg prednisone had the desired effect in our patient, the recommended dose is 1.0 – 1.5 mg/kg/day (11).
References 1. Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33:1122 – 8. 2. Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia reumatica and giant cell arteritis. N Engl J Med 2002;347: 261 – 71. 3. Saadoun D, Cacoub P, Costedoat-Chalumeau N, Sbai A, Piette JC. Horton’s disease and corticosteroid-responsive hearing loss. Ann Med Int 2000;151:600 – 2. 4. Bondeson J, Asman P. Giant cell arteritis presenting with oculomotor nerve palsy. Scand J Rheumatol 1997;26:327 – 8. 5. Davies GE, Shakir RA. Giant cell arteritis presenting as oculomotor nerve palsy with pupillary dilatation. Postgrad Med J 1994;70:298 – 9. 6. Berrettini S, Ravecca F, Bruschini L, Ursino F, SellariFranceschini S. Progressive sensorineural hearing loss: immunological causes. Acta Otorhinolaryngol Ital 1998;59: S33 – 42. 7. Hausch RC, Harrington T. Temporal arteritis and sensorineural hearing loss. Semin Arthritis Rheum 1998;28:206 – 9. 8. Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol 1998;125:509 – 20. 9. Fisher CM. Ocular palsy in temporal arteritis. Minn Med 1959;42:1258 – 68. 10. Biousse V, Newman NJ. Third nerve palsy. Semin Neurol 2000;20:55 – 74. 11. Gran JT. Some thoughts about the etiopathogenesis of temporal arteritis – a review. Scand J Rheumatol 2002;31:1 – 5.
