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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 67 years old woman with giant cell arteritis and neuritis multiplex is reported. The diagnosis was based on the microscopic appearances of temporal artery biopsy specimens. The authors described the involvement of peripheral nerves in this disease and made differential diagnosis with polyarteritis nodosa. There was also hemorragic infarction of the brain without giant-cells in brain vessels. It is concluded that this diagnosis should be considered in any elderly patient with peripheral neuropathy.
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PMID:[Multiple neuropaty caused by giant cell arteritis. Clinico-pathological report of a case]. 68 60

Peripheral nerves are rarely involved in Horton's disease, and when peripheral neuropathy predominates in the clinical complex it is sometimes a source of diagnostic errors. We report two cases of giant cell arteritis revealed by peripheral neuropathy.
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PMID:[Peripheral nerve diseases disclosing Horton's disease (2 cases)]. 164 80

Very few cases of pulmonary vasculitis that cannot be classified into a single category of vasculitis have been described. We report the first case of a vasculitic process in which pulmonary involvement with asthma, eosinophilic interstitial infiltrates, and small nodules were seen in association with jaw claudication and temporal arteritis with giant cells found on biopsy. Other signs of systemic involvement were also present such as peripheral neuropathy, hematuria with erythrocytic casts and proteinuria, pericardial effusion, and a dilated cardiomyopathy. The histopathologic picture was complex and unique. The early age of onset, the multisystemic involvement, and the prompt response to cyclophosphamide pointed to a diagnosis of "polyangiitis overlap syndrome," with some aspects of Churg-Strauss syndrome and also temporal arteritis. Physicians should be aware of these polymorphous and life-threatening pulmonary vasculitic syndromes, which require aggressive immunosuppressor therapy.
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PMID:Concurrent Churg-Strauss syndrome and temporal arteritis in a young patient with pulmonary nodules. 272 57

Of 166 consecutive patients with histologically confirmed giant cell (temporal) arteritis (GCA) seen during a 3-year period, 23 (14%) had clinically diagnosed peripheral neuropathic syndromes temporally coincident with clinically active GCA. Electromyography and nerve conduction studies were performed in 16, confirming abnormalities in all. Of the 23 patients, 11 had a generalized peripheral neuropathy, nine had multiple mononeuropathies, and three had a mononeuropathy. The nerves affected as mononeuropathies were the median, ulnar, peroneal, tibial, and sural nerves, and the C-5 and L-5 nerve roots. Angiography, performed in two patients, demonstrated widespread arteritis involving the lower limbs and, after 3 months of oral corticosteroid treatment in one of these patients, an amputation specimen showed chronic arteritis.
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PMID:Peripheral neuropathic syndromes in giant cell (temporal) arteritis. 283 68

Neurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occurred in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.
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PMID:Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. 838 87

Peripheral neuropathy is described in a patient with biopsy proven giant cell arteritis. Sural nerve biopsy showed myelin and axonal degeneration. Such an uncommon manifestation was resolved with corticosteroid therapy.
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PMID:Peripheral neuropathy associated with temporal arteritis. 964 18

Primary systemic amyloidosis or AL-amyloidosis is an uncommon disease characterized by the accumulation in vital organs of a fibrillar protein consisting of monoclonal light chains. It is a plasma-cell dyscrasia related to multiple myeloma where clonal plasma cells in the bone marrow produce immunoglobulins that are amyloidogenic. A monoclonal component is present in the serum or urine of 90% of patients. The presentation of most patients with AL amyloidosis is usually related to congestive heart failure, nephrotic syndrome o peripheral neuropathy, but there are unusual features suggesting giant cell arteritis (GCA) and polymyalgia rheumatic (PMR). Although in the majority of AL cases the plasma cells clone is small, the assumption is that the outcome of the disease is uniformly fatal (median survival 12-15 months) and treatment is analogous to those used in malignant proliferative disease. We describe a patient with AL amyloidosis who presented with manifestations of GCA and PMR, and we review the main characteristics of primary amyloidosis.
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PMID:[Primary systemic amyloidosis presenting as polymyalgia rheumatica and giant cell arteritis]. 1149 41

Giant cell arteritis (GCA) is a granulomatous arteritis and it occurs older (more than 50 years) individuals. As GCA frequently involves temporal artery, this disease had been called as temporal arteritis. However, except for the temporal artery, GCA affects branches of the carotid arteries, as well as aorta and its major branches preferentially. Thus, this arteritis is collectively called as GCA from the characteristic histological findings. We systematically introduce the clinical pictures of GCA in the first half of this article. Because affected arteries of GCA are different in each patient, some patients do not present with classical clinical features, such as headache and tenderness of temporal artery. Recently, there are several variant forms of GCA have been recognized. Certain subtypes demonstrate organ dysfunction, such as visual loss or peripheral neuropathy with minimal or absent classical and systemic manifestations. This form of GCA is referred to as occult GCA. On the other hand, other form of GCA presents with a systemic inflammatory syndrome in the absence of focal ischemic symptoms. This is referred to as silent or masked GCA. In the latter half of this article, we introduce two patients presenting with such atypical presentations. One could be diagnosed as occult GCA, and the other was diagnosed as silent GCA with large vessel type. GCA is a heterogenous disease with more than a single clinical picture. This article can provide considerations of the wide spectrum of presentation of this characteristic systemic arteritis.
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PMID:Giant cell arteritis. 2439 Jan 6

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