Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 67-year-old man developed jaw claudication followed by loss of vision in the left eye caused by anterior ischemic optic neuropathy (AION). An erythrocyte sedimentation rate was normal, but C-reactive protein was slightly elevated. Although the patient had no evidence of a right optic neuropathy, magnetic resonance imaging (MRI) revealed bilateral optic nerve sheath enhancement. A temporal artery biopsy was consistent with active giant cell arteritis (GCA). Our case demonstrates that bilateral optic nerve sheath enhancement on MRI can be seen in the setting of unilateral AION. This unique combination of clinical and imaging findings has not been reported previously and extends the clinical spectrum of presentation of GCA.
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PMID:Giant Cell Arteritis Presenting as Unilateral Anterior Ischemic Optic Neuropathy Associated With Bilateral Optic Nerve Sheath Enhancement on Magnetic Resonance Imaging. 2785 29

Giant cell arteritis with ocular involvement is an ocular emergency. Arteritic anterior ischaemic optic neuropathy (AAION) is the most common ophthalmological manifestation associated with this disease. Visual loss is usually permanent with rare cases showing visual recovery. Visual improvement, if it occurs, is generally limited, and the visual field defects are persistent and severe. The main goal of AAION treatment is the preservation of vision in the fellow eye. In patients with neurophthalmological manifestations, high-dose corticosteroids should be initiated immediately and aggressively, and maintained thereafter. We present a case of AAION and severe vision loss where significant visual recovery was seen after treatment.
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PMID:Giant cell arteritis: a closer look at its ophthalmological manifestations. 2641 75

Posterior ischaemic optic neuropathy is a rare cause of visual loss believed to be due to infarction in the territory of the pial branches of the ophthalmic artery. The disorder most commonly occurs in the context of prolonged surgery or giant cell arteritis, and the absence of clinical signs in the eye means that the diagnosis is one of exclusion. Here, we present two cases studies of patients who developed posterior ischaemic optic neuropathy confirmed by the observation of secondary changes on diffusion-weighted imaging sequences. In the first case visual loss followed robotic pelvic surgery, and in the second case it was associated with multiorgan dysfunction secondary to severe pancreatitis. Our cases demonstrate that in the right clinical context, diffusion-weighted imaging can provide a positive diagnosis of acute posterior ischaemic optic nerve injury in the acute phase.
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PMID:Using Diffusion-Weighted Magnetic Resonance Imaging to Confirm a Diagnosis of Posterior Ischaemic Optic Neuropathy: Two Case Reports and Literature Review. 2792 49

Bilateral simultaneous nonarteritic anterior ischaemic optic neuropathy (NAION) is extremely rare. A 57-year-old woman presented with bilateral optic disc oedema and peripapillary splinter haemorrhages. Initial visual acuities were hand movements in the right eye and light perception in the left eye. The patient had a mildly elevated diastolic blood pressure and glucose intolerance. Erythrocyte sedimentation rate and C-reactive protein levels were within normal limits. Temporal artery biopsy was negative for temporal arteritis. Marked visual improvement occurred in both eyes (0.8 in the right eye, 0.6 in the left eye) after systemic steroid therapy in the 16th month of follow-up.
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PMID:Bilateral Simultaneous Nonarteritic Anterior Ischaemic Optic Neuropathy: Case Report. 2816 91

A 75-year-old hypertensive female with stable idiopathic intermediate uveitis presented with bilateral sequential optic neuropathy with optic disc swelling. The optic neuropathy in the first affected eye (right) was thought to be due to non-arteritic anterior ischaemic optic neuropathy (NAION). Asymptomatic left optic disc swelling was found at routine review 2 months later, and a diagnosis of giant cell arteritis (GCA) was sought. Temporal artery duplex ultrasound showed the "halo sign," but a subsequent temporal artery biopsy showed light-chain (AL) amyloidosis with no signs of giant cell arteritis. In this case, bilateral sequential ischaemic optic neuropathy mimicking non-arteritic anterior ischaemic optic neuropathy was the presenting sign of systemic amyloidosis involving the temporal arteries.
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PMID:Bilateral Non-arteritic Anterior Ischaemic Optic Neuropathy as the Presentation of Systemic Amyloidosis. 2934 73

A retired businessman presented to the infectious diseases department with a history of ongoing fevers and myalgia and raised inflammatory markers. This continued despite adequate antibiotic treatment of an epididymo-orchitis. Extensive investigations, including bone marrow and liver biopsies and a positron emission tomography, did not reveal a cause but showed reactive change in the bone marrow. Later, he developed a vasculitic rash and vision loss due to non-arteritic anterior ischaemic optic neuropathy. High-dose steroids were immediately initiated. A temporal artery biopsy was performed, which confirmed a healing large vessel vasculitis, possibly giant cell arteritis. He has responded very well to therapy. We must better appreciate the limitations of positron emission tomography in investigating a fever of unknown origin. The case also encourages awareness of autoimmune disorders as the leading category of causative diseases for this in older age groups.
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PMID:Positron emission tomography and reframing vasculitis as a spectrum of disease when investigating a patient with a fever of unknown origin. 3023 1

Ultrasound examination has become an important tool in the diagnosis of giant cell arteritis, revealing the suggestive hypoechoic arterial wall thickening (halo sign). Vertebral artery involvement is not unusual in giant cell arteritis, but bilateral vertebral artery occlusion secondary to this affection is a rare condition and only a few cases have been reported so far. We report a case of an 84-year-old male patient with anterior ischaemic optic neuropathy secondary to giant cell arteritis, with bilateral occlusion of the vertebral arteries manifested with nonspecific neurological signs, detected during the ultrasound scan of the cervical arteries. The rarity of this case lies in the reasonably good outcome at the three-month and six-month follow-up and the absence of temporal artery involvement, despite the severe vertebral artery affection with bilateral occlusion.
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PMID:Bilateral vertebral artery occlusion without stroke secondary to giant cell arteritis. 3157 40

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, moderate-severe 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 specificity was only 30%. Therefore, inflammatory 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 first 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 first consideration.
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PMID:Lessons of the month 4: Giant cell arteritis with normal inflammatory markers and isolated oculomotor nerve palsy. 3218 66

Light chain (AL) amyloidosis may present with the features of vasculitis, including giant cell arteritis (GCA). Similarities between GCA and AL-amyloidosis can potentially cause confusion in diagnosis, in which case, temporal artery biopsy (TAB) should be performed to make a definitive diagnosis. Herein we report a case of a bilateral anterior ischaemic optic neuropathy (AION), showing evidence of AL-amyloidosis on the temporal artery biopsy. A 75-year-old male with AL-amyloidosis secondary to monoclonal gammopathy of undetermined significance (MGUS) presented to our hospital for subacute painless progressive visual impairment. Based on his elevated inflammatory markers and his age, he was suspected to have giant cell arteritis. However, a temporal artery biopsy excluded GCA, and the Congo red staining was positive for amyloid deposition. This present case reveals that AL-amyloidosis may present with visual impairment, high inflammatory markers, and involvement of temporal arteries, concerning for GCA. TAB with Congo red staining is found to be crucial for making the correct diagnosis.
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PMID:Ocular manifestation of giant cell arteritis vs AL-amyloidosis: similar presentations but different approaches. 3278 54


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