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Editor,—Giant cell arteritis (GCA) presents either as localised or systemic vasculitis and is found typically in individuals older than 50 years and is often associated with polymyalgia. GCA involves most frequently the temporal artery and may be bilateral. Other affected vessels include aorta, carotid, vertebral, ophthalmic and, rarely, coronary arteries. Headache, jaw claudication, and scalp tenderness are frequent complaints. Anterior arteritic ischaemic optic neuropathy is the best known, most common, and severe neuro-ophthalmic manifestation. We describe the case of a patient with third and sixth cranial nerve palsy, as well as suspected liver involvement as presenting signs in GCA.
A 69 year old female patient was referred for examination of ptosis in the right eye and diplopia. The past medical history was unremarkable. One month before admission the patient had suffered from severe headache, loss of appetite, and weight loss. Two weeks later the patient noticed a droopy right eyelid and started to experience double vision.
On examination visual acuity was full in both eyes and the optic discs were normal. Both pupils were equal and reactive. The visual fields were intact. There was ptosis, slight esotropia, and a deficiency in elevation, downgaze, and abduction of the right eye (Fig1).
Based on these findings the diagnosis of a partial external oculomotor and abducens palsy was made.
Laboratory testing was remarkable for an elevated erythrocyte sedimentation rate (ESR), 75 mm in the first hour, and C reactive protein (CRP) 42 mg/l. The red and white blood cell counts were normal as well as autoimmune antibody titres. Serological antibody titres for herpes virus, cytomegalovirus, Epstein–Barr virus,Borrelia burgdorferi, and hepatitis virus types A, B, and C were normal. Liver enzymes were elevated: AST (GOT) 72 IU/l, ALAT (LPT) 121 IU/l, alkaline phosphatase 195 IU/l, gamma GT 235 IU/l. Cerebrospinal fluid revealed normal opening pressure, cell count, glucose, and protein concentration. Except for dysfunction of the ocular motility in the right eye, the neurological examination was normal. Magnetic resonance imaging of the brain (MRI) and a chestx ray were unremarkable. Ultrasound and computed tomography of the abdomen were normal. Histological examination of a fine needle liver biopsy displayed small areas of necrotic changes and low grade lobular inflammation that fitted the morphological diagnosis of non-specific hepatitis (Fig 2A). Colour Doppler imaging (CDI), using a 5–10 MHz broad band linear array transducer, was performed in search of temporal arteritis and reported to be normal. Temporal artery biopsy was therefore performed and therapy with intravenous methylprednisolone (250 mg twice a day) was started immediately. Microscopic examination of the biopsy specimen revealed florid giant cell arteritis (Fig 2B).
Within 3 weeks the oculomotor dysfunction improved rapidly along with normalisation of the ESR, CRP, and all liver factors.
Involvement of cranial nerves, such as abducens and oculomotor nerve, leading to diplopia is a known complication of CGA. Diplopia, however, can occur as well without a distinct pattern of cranial nerve involvement.1-3 Primary affection of the eye muscles and/or cranial nerves has been discussed as pathophysiological mechanisms for ophthalmoplegia. Liver involvement has frequently been reported in patients with GCA and polymyalgica rheumatica and is usually reflected by raised serological liver enzymes.4-6In our case, a minute solitary infiltrate was found in the fine needle liver biopsy specimen; although non-specific, it is consistent with systemic manifestation of GCA, and the return to normal serological parameters of liver function upon steroid medication provides a further argument of this view.
The diagnosis of CGA is based on the history, the clinical picture, and the temporal artery biopsy. Schmidt et alsuggested that patients with typical clinical signs of GCA and a clear halo on CDI might be treated with steroids without performing a biopsy.7 In our patient with biopsy proved giant cell arteritis, however, CDI was normal. Although CDI has a high specificity, the sensitivity is still not satisfactory, indicating that for the diagnosis of temporal arteritis histology is still the gold standard. Based on our experience with this patient we would like to emphasise that new onset of ophthalmoplegia can be the first presenting sign of GCA, leading the patient to consult a doctor. We should therefore add GCA to the differential diagnosis in patients with oculomotor and sixth nerve palsies especially when systemic involvement, such as hepatitis, is present.
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