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 55-year-old male suffered sudden onset of dysarthria and mild left hemiparesis due to a right intracerebral small hemorrhage. On admission, six subcutaneous elastic hard lumps were found on the scalp with painless and regular pulsation. The lumps were located along the course of the bilateral superficial temporal arteries (5 locations) and the occipital artery. The patient did not have symptoms of headache or blurred vision associated with temporal arteritis. The largest lump was removed for cosmetic reasons and definitive diagnosis. Histological examination demonstrated many infiltrating inflammatory cells along the entire vascular wall but without giant or fibrinoid necrosis. These multiple scalp aneurysms were probably caused by atypical temporal arteritis.
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PMID:Multiple scalp aneurysms caused by atypical temporal arteritis--case report. 974 45

Over a one month period, a 74-year-old man developed typical features of giant cell arteritis (GCA) including visual changes, headache, scalp tenderness, and an elevated erythrocyte sedimentation rate. In addition, he had reproducible painless dysarthria that was precipitated by chewing or prolonged talking and was relieved by resting the jaw. The dysarthria with chewing along with the other classical symptoms of GCA subsided with treatment. To our knowledge this is the first report of painless dysarthria associated with GCA.
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PMID:Dysarthria associated with giant cell arteritis. 1022 20

We report a 73-year-old patient with giant cell arteritis who presented with right arm paresis and dysarthria. Vasculitis of the vertebral arteries induced multiple ischemic cerebral lesions in the vertebrobasilar territory, ultimately leading to death despite intensive immunosuppressive therapy. This case illustrates that prompt relief from symptoms of giant cell arteritis upon steroid treatment does not correspond to a remission of the vasculitic process itself. Clinicians should therefore be aware of the possible occult involvement of the vertebral or other major arteries.
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PMID:[Multiple ischemic vertebrobasilar lesions in temporal arteritis]. 1590 93

Anti Phospholipid Syndrome (APS) is a relatively new conception of syndrome complex first noticed in 1983. It may be primary or secondary to other diseases like SLE, RA, Systemic sclerosis, behchet's syndrome, temporal arteritis, sjogren's syndrome psoriatic arthropathy etc. Clinical manifestations are consequences of vascular thrombosis and embolism like DVT, pulmonary embolism, stroke, TIA, complication of pregnancy with pregnancy loss. We report a 34 years married female housewife who presented with sudden onset of nausea, vomiting, vertigo, dysphagia, dysarthria and ataxia. She had a chronic leg ulcer. Neurological findings were consistent with lateral medullary syndrome due to stroke though she was normotensive, nondiabetic with normal lipid profile. She had history of two abortions in last three years. Investigations were done accordingly and she fulfilled the diagnostic criteria of APS. No secondary cause was detected after thorough clinical examination and laboratory investigations. She was treated symptomatically along with oral anticoagulation. She improved slowly but steadily.
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PMID:Anti phospholipid syndrome. 1918 54

Giant cell arteritis (GCA) is the most common form of systemic vasculitis in adults. Patients usually present with headache and visual symptoms, and have an elevated erythrocyte sedimentation rate. It has been reported that 3-4% of patients with GCA develop ischemic events secondary to vertebral artery stenosis or occlusion. The mainstay of therapy of GCA is high dose steroid and/or methotrexate. A case is described of a patient who initially presented with intermittent double vision, mild headache and unremarkable MRI and MR angiography of the head and neck. The patient was diagnosed and treated for ocular myasthenia. The patient was readmitted 2 months later with imbalance and worsening headache, and workup suggested bilateral cerebellar infarction, complete occlusion of the left vertebral artery and a high grade stenosis of the right vertebral artery. Erythrocyte sedimentation rate and C reactive protein were elevated. Temporal artery biopsy demonstrated changes consistent with GCA. During the course of the treatment with corticosteroids and immunosuppressant, the patient developed dysarthria, left facial droop and left hemiplegia, and was found to have complete occlusion of both vertebral arteries. The patient was emergently taken for revascularization of the occluded segment using angioplasty and stent placement. The patient had significant improvement of neurological symptoms within 3 days after the procedure and continued to improve during hospitalization. Endovascular treatment of vasculitis affecting the intracranial vessels is not yet established. Our experience with successful treatment of complete occlusion of the vertebral artery secondary to GCA using endovascular intracranial angioplasty and stent placement is reported.
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PMID:Angioplasty and stent placement for complete occlusion of the vertebral artery secondary to giant cell arteritis. 2199 Apr 65

Hypereosinophilic syndrome is a rare disorder of the hematopoietic system. The disease is characterized by continuously high number of eosinophils (>1.5 x 10(9)/L) for more than six months. Other possible causes of hypereosinophilia, such as allergic and parasitic diseases, malignant disease, Churg-Strauss disease and infection should be eliminated. The most common manifestations of hypereosinophilic syndrome are pulmonary, skin, gastrointestinal, cardiac difficulties and neurologic lesions. Numerous neurologic lesions have been described, in particular of the central and peripheral nervous systems. Review of the literature revealed the following to have been recorded so far: mononeuritis multiplex, sensory polyneuropathy, radiculopathy, myalgia, myositis and perimyositis, neuropathy, ataxia, paraplegia, ophthalmologic abnormalities, optic neuritis, hemiplegia-hemiparesis, spasmodic quadriplegia, seizures, meningitis, cerebral infarction, organic psychosyndrome, other mental changes, stroke, temporal arteritis, leptomeningeal dissemination, memory deficits and dysarthria.
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PMID:Neurologic manifestations of hypereosinophilic syndrome--review of the literature. 2292 4

An 84-year-old male with a 3-month history of headache and elevated C-reactive protein levels was admitted for biopsy of the superficial temporal artery, which led to the diagnosis of giant cell arteritis (GCA). Two days after prednisolone therapy was initiated, the patient began to experience transient vertigo attacks. Two days later, dysarthria, left-sided hemiparesis, right abducens palsy, and horizontal nystagmus developed. Magnetic resonance (MR) imaging disclosed fresh infarctions in the vertebrobasilar territory. Since the patient became drowsy because of brainstem compression and hydrocephalus due to cerebellar swelling, emergency suboccipital decompression surgery and ventricular drainage were performed. Subsequently, the patient's consciousness levels improved. MR angiography revealed right vertebral artery (VA) occlusion and left VA stenosis due to arteritis. Ischemic stroke is a serious though relatively rare complication of GCA. Similar cases have been reported, in which ischemic stroke developed despite or possibly due to steroid therapy. To our knowledge, this is the first description of vertebrobasilar infarction associated with GCA in the Japanese population. The merits and potential demerits of steroid therapy are briefly discussed.
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PMID:Vertebrobasilar infarction related to giant cell (temporal) arteritis: case report. 2439 Jan 82

Autosomal dominantly inherited oculopharyngeal muscular dystrophy (OPMD) is caused by a trinucleotide repeat expansion in exon 1 of the polyadenylate binding protein nuclear 1 (PABPN1) gene on chromosome 14q. A large family with OPMD was recently identified in Pretoria, South Africa (SA). Molecular studies revealed a (GCG)11(GCA)3GCG or (GCN)15 mutant allele. The (GCN)15 mutation detected in this family has been described previously in families from Uruguay and Mexico as a founder effect. To our knowledge, this is the first report of an SA Afrikaner family with molecularly confirmed OPMD. The proband, a 64-year-old woman, presented to the neurology outpatient department at Steve Biko Academic Hospital, Pretoria. A sibship of 18 individuals was identified, of whom eight had OPMD. Four patients were interviewed and examined clinically, and electromyographic studies were performed. Molecular analysis of the PABPN1 gene was performed by polymerase chain reaction amplification and direct sequencing of exon 1 in three of the patients. Patients presented with ptosis, external ophthalmoplegia, dysphagia, dysarthria and mild proximal weakness. High foot arches and absent ankle reflexes raised the possibility of peripheral neuropathy, but electromyography showed only mildly low sensory amplitudes, and myopathic units in two patients.
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PMID:A South African family with oculopharyngeal muscular dystrophy: Clinical and molecular genetic characteristics. 2642 47

Unusual presentations of Giant Cell Arteritis (GCA) can sometimes delay the diagnosis and its prompt treatment. An 83-year-old male patient was admitted in the emergency department with a few hours evolution of tongue swelling, dysphagia and dysarthria. He also complained of a bitemporal headache with about 4 months of evolution and resistant to all treatment prescribed, including ergotamine, that he had started one week before. Upon examination, the patient presented a bilateral temporal pain and reduced mobility of the tongue which evolved to complete cyanosis. The blood tests revealed normocytic normochromic anaemia, an erythrocyte sedimentation rate of 62 mm/h, and C-reactive protein of 23,6 mg/dl. Cranial CT scan was normal and the cervical CT angiogram showed reduced vascularization of the left submandibular gland and of the base of the tongue. The cervical doppler ultrasound was compatible with arterial inflammation. Given the high suspicion of GCA, the patient was immediately put on a high dose of corticosteroid, resulting in a big improvement of the symptoms, which continued in the following weeks. In conclusion, the clinical suspicion of GCA is fundamental for an early diagnosis. The authors consider that ergotamine might have triggered tongue ischemia in this case.
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PMID:Tongue ischemia - an unusual presentation of Giant Cell Arteritis. 3157 44