1/6. Cryoglobulinemic neuropathy related to hepatitis c virus infection. Clinical, laboratory and neurophysiological study.Peripheral neuropathy is frequently reported in mixed cryoglobulinemia. As a close relationship has been established between mixed cryoglobulinemia and hepatitis c virus (HCV), the clinical, neurophysiological, and serologic findings of five patients affected by neuropathy, cryoglobulinemia and HCV infection were investigated. HCV infection was ascertained by the presence in the serum of anti-HCV antibodies detected by enzyme-linked immunosorbent assay(ELISA), and of HCV rna assessed by polymerase chain reaction (PCR). Initial symptoms included paresthesias and painful dysesthesias in the legs. Later on, the patients developed a mainly asymmetric axonal polyneuropathy or a multifocal neuropathy associated with signs of systemic vasculitis. In this case series we report the short-term prognosis, as well as the response to interferon (IFN) alpha and conventional treatment. The presence of HCV rna supports the hypothesis that a persistent HCV infection might be involved in the pathogenesis of mixed cryoglobulinemia and cryoglobulinemia-associated disorders.- - - - - - - - - - ranking = 1keywords = cryoglobulinemia (Clic here for more details about this article) |
2/6. Motor-axonal polyneuropathy associated with hepatitis c virus.The association between hepatitis c virus (HCV) infection, the presence of mixed cryoglobulinemia and peripheral neuropathy is well-documented (Apartis et al., 1996). HCV is the chief cause of essential mixed cryoglobulinemia (type II cryoglobulinemia) with cryoglobulins present in up to half of patients with HCV infection (Akriviadis et al., 1997). More recently it has been stated that peripheral polyneuropathy may be associated with HCV chronic infection without mixed cryoglobulinemia (Lidove et al., 2001). patients usually present with a clinical and electrophysiology--predominantly sensory axonopathies (Apartis et al., 1996; Heckmann et al., 1999) or less frequently with fulminating vasculitis and mononeuropathy multiplex syndrome (David et al., 1996)--especially when associated with cryoglobulinemia. We report, for the first time, the association between pure motor-axonal polyneuropathy and HCV infection without cryoglobulinemia.- - - - - - - - - - ranking = 1.2keywords = cryoglobulinemia (Clic here for more details about this article) |
3/6. hepatitis c virus infection, cryoglobulinemia, and peripheral neuropathy: a case report.hepatitis c virus (HCV) is essentially hepatotropic but its manifestations can extend beyond the liver. It can be associated with autoimmune diseases, such as mixed cryoglobulinemia, membranoproliferative glomerulonephritis, autoimmune thyroiditis, and lymphoproliferative disorders. The mechanisms that trigger these manifestations are not completely understood. We describe a 48-year-old man with chronic HCV infection (circulating HCV rna and moderate hepatitis as indicated by liver biopsy), cryoglobulinemia, and sensory and motor peripheral neuropathy. The diagnosis of multineuropathy was confirmed by clinical examination and electromyographic tests. A nerve biopsy revealed an inflammatory infiltrate in the perineurial space and signs of demyelination and axonal degeneration. The patient had no improvement of neurological symptoms with the use of analgesics and neuro-modulators. He was then treated with interferon-alpha (3 million units subcutaneously, 3 times per week) and ribavirin (500 mg orally, twice a day) for 48 weeks. Six months after the end of therapy, the patient had sustained viral response (negative HCV rna) and remission of neurological symptoms, but cryoglobulins remained positive. A review of the literature on the pathogenesis and treatment of neurological manifestations associated with HCV infection is presented. This report underscores the need for a thorough evaluation of HCV-infected patients because of the possibility of extrahepatic manifestations. Antiviral treatment with interferon and ribavirin can be effective and should be considered in patients with neurological complications associated with HCV infection.- - - - - - - - - - ranking = 1.2keywords = cryoglobulinemia (Clic here for more details about this article) |
4/6. Cryoglobulinemic neuropathy: ultrastructural study of the nerve capillaries.The authors report here the case of a female patient who developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have a myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material from this patient, electron microscopy showed pathological changes in the nervous tissue together with axonal degeneration and demyelination. But the most striking finding was the presence in the endoneurial space, of numerous masses made up of closely packed tubular structures. These masses occurred also in the walls and even in the lumen of all the vasa nervorum. Their morphologic features and dimensions were identical to cryoprecipitate which the authors extracted from the serum and which they examined with electron microscope.- - - - - - - - - - ranking = 0.2keywords = cryoglobulinemia (Clic here for more details about this article) |
5/6. Cryoglobulinemic neuropathy: a pathological study.A 53-year-old woman developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material, electron microscopy showed both axonal degeneration and demyelination. The most striking finding was the presence, in the endoneurial space, of numerous masses of closely packed tubular structures. These masses also were found in the walls of all the vasa nervorum and within the lumen of some vessels. The morphological features and dimensions of the deposits within nerve were identical to those of cryoprecipitates extracted from serum and examined with the electron microscope. An example of myeloma neuropathy with cryoglobulin deposits within the endoneurial space has not been reported previously.- - - - - - - - - - ranking = 0.2keywords = cryoglobulinemia (Clic here for more details about this article) |
6/6. A role for chronic hepatitis c virus infection in a patient with cutaneous vasculitis, cryoglobulinemia, and chronic liver disease. Effective therapy with interferon-alpha.A six-year history of repeated attacks of fatigue, fever, arthralgias, skin changes, Raynaud's phenomenon, and neuropathy is reported in a patient with chronic liver disease. The following diagnoses were made: (1) leukocytoclastic vasculitis; (2) acute urticaria; (3) cryoglobulinemia type II with Raynaud's phenomenon and low serum level of C4; (4) peripheral polyneuropathy; (5) sicca syndrome; and (6) chronic hepatitis c virus infection. Despite therapy with corticosteroids symptoms increased gradually over years. In the first PCR of the nested PCR analysis, HCV-rna was exclusively detected in the cryoglobulin fraction but not in the serum supernatant, suggesting that antibodies bind HCV particles, forming circulating immune complexes. As diagnoses 1-5 are well-known organ manifestations of cryoglobulinemia, we speculated whether treatment of hepatitis c with IFN-alpha (3 million IU IFN-alpha 2b three times a week) would inhibit HCV replication, decrease the cryocrit level and thereby ameliorate organ manifestations such as neuropathy and vasculitis. During treatment with IFN-alpha only a very weak or no signal could be detected for HCV-rna in the cryoglobulin fraction as well as in the serum supernatant. This held true also for the serum supernatant in the second PCR. In parallel, cryoglobulin level, immunoglobulins, and liver enzymes decreased substantially to normal or near normal levels. Clinical symptoms-leukocytoclastic vasculitis and neuropathy-disappeared. We conclude that chronic HCV infection is involved in the pathogenesis of cryoglobulinemia and that IFN-alpha might be an effective treatment in these patients.- - - - - - - - - - ranking = 1.4keywords = cryoglobulinemia (Clic here for more details about this article) |