Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Among 54 patients with mixed cryoglobulinemia a peripheral neuropathy was demonstrated in 23 patients (42.6%). Five patients had a secondary form while the majority (18) had essential cryoglobulinemia. The immunoglobulin components were polyclonal in 5 cases, only present in trace amounts in 5 and of type II in 13 patients. Fifteen patients had symptoms of polyneuropathy, but in 18 cases the symptomatology had symmetrical features and in two cases asymmetrical characteristics. In 8 patients a mono-polyneuropathy was observed. The most significant electrophysiological finding was the decreased amplitude of the sensory action potential of the sural nerve, which we observed in 95% of the instances, whereas a decreased sensory conduction velocity was seen in 8/23 cases only. A biopsy of the sural nerve, performed in eleven cases, always showed abnormal epi- and endoneurial vessels. Epineurial vessel vasculitis was found in 4 cases and fibrous thickening of the vessel wall in two cases. Endothelial swelling of the endoneurial vessels was observed in 7 patients and luminal obliteration in 3 samples. Axonal degeneration demonstrated in 7 of the 11 processed sample tissues was the main pathological finding. Signs of de/remyelination were associated in 3 cases, and were the dominant lesions in a single specimen. The simultaneous biopsy of the peroneus brevis muscle showed signs of denervation in all samples, vasculitis in 7 cases and deposits of filaments plus annular bodies of the vessel wall in 2 samples. All these data support the hypothesis that ischaemic damage may be the final common pathway of the majority of peripheral neuropathies in mixed cryoglobulinemia patients.
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PMID:[Peripheral neuropathy with mixed cryoglobulinemia]. 196 89

The case of a 63 year old woman with mesenteric recurrence of a colonic carcinoma and infiltration of the duodenum is reported. To bypass duodenal stenosis a duodenojejunostomy was performed. Three months later the patient developed severe atypical polyarthritis which led to hospitalization. The arthritis affected large and small joints in an asymmetrical pattern. Fever and Raynaud's phenomenon of both hands accompanied the arthritis. Elevated sedimentation rate, acute phase proteins, cryoglobulinemia and immune complexes were remarkable laboratory findings. Rheumatoid factor was absent. In the subsequent course the polyarthritis was refractory to steroids and nonsteroidal anti-inflammatory drugs. Only treatment with broad-spectrum antibiotics ameliorated the arthritis. Postenteric reactive arthritis, septic arthritis and metastatic arthritis could be excluded. Although the patient had a family history of rheumatoid arthritis and a HLA-type DR4 the diagnosis of rheumatoid arthritis was not very likely since distal interphalangeal joints were affected, rheumatoid factor was absent and antibiotic therapy was successful. The case serves to discuss carcinoma-polyarthritis and bypass-arthritis as the main differential diagnosis.
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PMID:[Therapy refractory atypical polyarthritis and cryoglobulinemia in a patient with colon carcinoma and palliative intestinal bypass. Differential diagnosis: carcinoma-polyarthritis or bypass arthritis]. 748 38

Cryoglobulinemia represents an emerging cause of peripheral neuropathy, especially in Southern Europe, in view of its relationship with hepatitis C virus infection. In a series of 100 consecutive referral patients with uncharacterized peripheral neuropathies, we systematically investigated cryoglobulinemia to assess its diagnostic yield. The most frequent diagnosis was hereditary neuropathy (33%), 29% were acquired neuropathies of different types, and no cause could be identified in 27%. Cryoglobulinemic neuropathy was diagnosed in 11 patients (7 women and 4 men), aged 54-77 (mean = 63.5 years), most presenting with sensory polyneuropathy, often asymmetrical. Cryoglobulin was also detected in 2 additional patients in whom a final diagnosis of non-Hodgkin lymphoma was made. Purpura was absent in 4 patients (and in 2 with lymphoma), or restricted to discrete manifestations in the remaining patients, which did not provide a clue to the diagnosis. Thus, search for cryoglobulin proves useful in a substantial number of undiagnosed peripheral neuropathies (11% to 13% in our series), even in the absence of typical skin lesions, and it is recommended as a first-line investigation in patients with unexplained neuropathy presenting in middle to older age.
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PMID:Cryoglobulinemia is a frequent cause of peripheral neuropathy in undiagnosed referral patients. 1193 53

In HCV-related mixed cryoglobulinemia (MC) a peripheral neuropathy (PN) may occur. To evaluate the prevalence and the characteristics of PN, 133 consecutive patients with HCV-MC (117 type II, 16 type III) were studied. Neurologic evaluation was performed according to the guidelines of Italian Group for the Study of Cryoglobulinemias, using a neurological disability score and a neurological symptom score. In 52/133 patients an electrophysiologic study (ENG) of ulnar, peroneal and sural nerves was performed. For 27/52 patients ENG data registered at different times (interval 12-96 months) were available. In 11 patients a sural nerve biopsy was obtained. An overt PN, mostly as sensory asymmetrical or symmetrical nerve impairment, was found in 107/133 patients (80.4%). ENG abnormalities-reduction or absence of sensory and sometimes of motor action potential, normal or slightly impaired nerve conduction velocity, consistent with axonal damage- were detected in 48/52 patients (92.3%). In 26 out of the 27 patients observed at different times an evolution of PN was found. Nerve biopsies showed a prevalent axonal damage, swollen endothelial cells in epi- and perineurial vessels and scarce mononuclear perivascular infiltrates. No leukocytoclastic vasculitis was observed. Immunoglobulins and complement in sub-perineurial vessel wall were detected. CONCLUSIONS: In HCV-MC a PN is frequent. It is mostly a sensory and progressively worsening axonopathy. Different mechanisms may be involved in the pathogenesis of this disorder and a direct role of HCV cannot be excluded.
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PMID:[Peripheral nervous system involvement in HCV-related mixed cryoglobulinemia] 1246 75

A 75-year-old man, previously diagnosed as having chronic hepatitis C virus (HCV) infection, suddenly developed left foot drop, followed by progressive motor weakness and sensory disturbance in all of the extremities. Because of an elevated level of the rheumatoid factor (RF), he had been treated with antirheumatic drugs three years before the onset of his neurological symptoms. Within two months, he became unable to walk any more, and was transferred to our hospital. Neurologic examination showed asymmetrical severe muscular weakness and atrophy of all the limbs, and a sensory deficit under the level of the wrists and knees. Livedo reticularis was also noted in bilateral legs. Nerve conduction study revealed severe sensorimotor axonal neuropathy, and muscle biopsy specimens showed necrotizing vasculitis of small arteries in the perimysium. Serological tests indicated type II cryoglobulinemia (monoclonal IgAkappa + polyclonal IgG). A diagnosis of vasculitic neuropathy associated with HCV-related mixed cryoglobulinemia was made. A high-dose intravenous immunoglobulin therapy (IVIg) and a high-dose steroid therapy were not effective, and he died of alveolar hemorrhage probably due to pulmonary vasculitis. Postmortem pathological examination revealed severe vasculitis, accompanied by fibrinoid degeneration and the infiltration of predominant mononuclear cells into the small and medium-sized vascular walls of multiple organs such as the liver, kidney, pancreas and intestine as well as the peripheral nerves and skeletal muscles. A severe loss of myelinated fibers were also observed in the multiple peripheral nerves examined. We emphasize that patients with HCV infection and mixed cryoglobulinemia may develop severe systemic vasculitis resembling polyarteritis nodosa leading to often life-threatening polyvisceral failure, particularly in patients showing progressive mononeuropathy multiplex.
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PMID:[An autopsy case of systemic vasculitis associated with hepatitis C virus-related mixed cryoglobulinemia presenting severe peripheral neuropathy]. 1556 85

Paraneoplastic syndrome is defined as tumor-associated symptoms and signs not related to the physical effects of primary or metastatic tumors. The mechanisms of this syndrome include the production of bioactive soluble factors by tumor cells and autoimmune diseases elicited by the immune responses against tumors. Production of bioactive soluble factors causes endocrinologic symptoms. The paraneoplastic autoimmune process may affect the nervous system, cutaneous tissue, musculoskeletal system, hematopoietic cells or kidneys. Paraneoplastic rheumatic diseases show symptoms similar to inflammatory myopathy, polyarthritis, vasculitis, cryoglobulinemia and polymyalgia rheumatica. Rapid onset, unusual age, asymmetrical involvement of joints or refractoriness to standard immunosuppressive therapy suggests the presence of paraneoplastic autoimmune diseases. Autoimmune hematopoietic disorders include pure red cell aplasia, autoimmune hemolytic anemia and thrombocytopenia. Unexplained anemia or thrombocytopenia may indicate the presence of lymphoid neoplasms. Membranous nephropathy is a well-known glomerular disease associated with malignancy, and membranoproliferative glomerulonephritis, minimal change nephrotic syndrome, and antineutrophil cytoplasmic antibody(ANCA)-associated crescentic glomerulonephritis may be seen in cancer patients. Age and sex-appropriate cancer screening should be performed in patients with nephrotic syndrome due to membranous nephropathy.
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PMID:[Paraneoplastic autoimmune disorders]. 2056 97