Lessons of the month 4: Giant cell arteritis with normal inflammatory markers and isolated oculomotor nerve palsy

Abstract

Giant cell arteritis (GCA) is an important condition to suspect and treat early, as failure to do so can result in anterior ischaemic optic neuropathy and subsequent permanent visual loss. A 71-year-old woman presented to her local emergency department with a 1-week history of constant, moderatesevere global headache associated with intermittent periorbital pain. Two weeks later she developed sudden horizontal diplopia. Examination demonstrated right oculomotor nerve palsy. Her erythrocyte sedimentation rate (ESR) was 9 mm/hr. Repeat blood tests 1 month later showed an ESR of 67 mm/hr. Temporal artery biopsy was positive. A review from a cohort of 764 patients with suspected GCA who underwent biopsy found the sensitivity of an elevated ESR and C-reactive protein was 84% and 86%, respectively, but the specifi city was only 30%. Therefore, infl ammatory markers should only act as a guide, and caution should be taken in their interpretation especially with respect to the time of sampling in the disease evolution. Isolated oculomotor nerve palsy in association with GCA is rare. The fi rst case series was described by Miller Fisher in 1959 who observed two patients presenting with diplopia, ptosis and ocular palsies. In anyone over the age of 50 who develops a new, refractory headache and cranial neuropathy, GCA should be the fi rst consideration.

Analysis of Development Mechanism of Giant Cell Arteritis in Nude Mouse Model through Color Duplex Sonography and Computerized Tomography Nanocontrast Agent

“…The thickness of the aorta was significantly higher than that of the control group, the wall of the temporal artery was thickened, the lumen was significantly narrowed, there was much mononuclear cell infiltration in the temporal artery, and the phenomenon of a fullthickness crack appeared. These characteristics indicated that the nude mice in the experimental group showed symptoms of inflammatory damage and increased inflammatory cells, which was consistent with the current results of GCA biopsy [12], which fully illustrated the success of the construction of the GCA animal model in this study. The clinical diagnosis of superficial temporal artery GCA mainly depends on arterial angiography and biopsy [13].…”

Normal ESR, CRP and Platelet Count in Giant Cell Arteritis and Polymyalgia Rheumatica: A Diagnostic Conundrum

“…It is estimated that about 4% of GCA patients may have normal CRP and ESR, but those with normal inflammatory markers may actually have more prominent PMR symptoms and fewer systemic symptoms [8]. Although our patient consistently had a normal CRP, ESR and platelet count throughout the course of the disease, the temporal artery biopsy confirmed the diagnosis of GCA, which supports a body of evidence [9][10][11][12] suggesting that normal inflammatory markers should not exclude a diagnosis of GCA and PMR [13] . Clinical evaluation for any disease is very important.…”

An interesting case of temporal arteritis that manifested as ptosis and diplopia

“…Temporal artery biopsy is considered the gold standard; however, it has a sensitivity of only 15–40% [ 4 ]. About 25% of biopsies do not show granulomas [ 3 ] as demonstrated in our patient above.…”

“…Though there have been some reports in the literature of Horner’s syndrome in GCA [ 2 , 7 ], pupil-sparing isolated third nerve palsies were only noted twice [ 8 , 9 ] The pathogenesis behind ptosis or Horner’s syndrome remains obscure, likely due to the rarity of this manifestation; however, evidence points to muscle ischemia as the cause behind the visual compromise. In an autopsy done of a patient with ophthalmoplegia secondary to GCA, the extraocular muscles were noted to be necrosed [ 2 , 3 ], suggesting that GCA contributes to internal and external carotid artery ischemia and, subsequently, muscle ischemia and necrosis. There are no data on the effect of Horner’s syndrome or isolated third nerve palsies on the prognosis of visual loss in GCA.…”

“…Although most healthcare providers are aware of the common red flag symptoms of GCA such as blurriness of vision, headache and jaw claudication, lesser known manifestations such as isolated third nerve palsies (ptosis), diplopia and Horner’s syndrome can also occur. In fact, there are only two reported cases in the literature of isolated third nerve palsies [ 2 , 3 ], highlighting the significance of this unusual symptom in what is already a rare condition.…”