1/12. Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/12. The spectrum of chronic inflammatory demyelinating polyneuropathy.research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.- - - - - - - - - - ranking = 5.0190322249717keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/12. guillain-barre syndrome occurring in two women after ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus.We report two women who presented with a guillain-barre syndrome just after a ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus. One had characteristic clinical signs and the other had major motor involvement. At neurophysiologic investigations, one had typical demyelinating neuropathy whereas the second had mainly axonal degeneration. At ultrastructural examination of a peripheral nerve biopsy, features of macrophage-associated demyelination were present in both nerve specimens, thus confirming the diagnosis of acute inflammatory demyelinating polyneuropathy, i.e., guillain-barre syndrome. Prominent axonal involvement was also present in the motor nerves of the second patient. insulin therapy had to be permanently continued and these two cases are quite different from the transient diabetes sometimes observed in certain cases of guillain-barre syndrome. Both the latter and insulin-dependent diabetes mellitus probably have auto-immune mechanisms. It is likely that in our two patients both auto-immune diseases were triggered by a common event. Such cases of guillain-barre syndrome have to be distinguished from other acute diabetic neuropathies.- - - - - - - - - - ranking = 0.2407199691812keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
4/12. neurology. 4: multiple sclerosis.multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system (CNS) and a common cause of disability in young adults; it is most likely an autoimmune disease. Typically, MS initially follows a relapsing-remitting course, but most patients eventually develop secondary progressive MS, where there is progressive deterioration without relapses or remissions; in some patients, MS has a primary progressive course. The diagnosis of MS requires evidence of CNS lesions disseminated in time and place, as well as the exclusion of other likely causes of these lesions; the clinical history, neurological examination and investigations, such as magnetic resonance imaging of the brain and spinal cord, all have key roles in the diagnosis. education and counselling of the patient and family members are essential for good patient management. Moderate to severe attacks of MS are best treated with intravenous infusions of high-dose methylprednisolone. Interferon beta reduces the frequency of attacks and the progression of disability in relapsing-remitting MS. Symptomatic therapy is important in the management of spasticity, pain, urinary problems and the other symptoms or complications of MS.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
5/12. Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy.Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune disease characterized by recurrent demyelination and remyelination with resultant thickening of the peripheral nerves. We report a case in which sonography was instrumental in demonstrating diffuse peripheral nerve hypertrophy. On sonography, both brachial plexuses were found to be diffusely hypertrophic and hypoechoic. Similar findings were noted for the median, sciatic, and femoral nerves. The brachial plexus findings were confirmed by MRI.- - - - - - - - - - ranking = 5.8263759159507keywords = chronic inflammatory demyelinating, polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/12. Motor dominant neuropathy in sjogren's syndrome: report of two cases.Most of the peripheral neuropathies in sjogren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of guillain-barre syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating polyradiculoneuropathy. These patients received i.v. immunoglobulin therapy. To our knowledge, this is the first report indicating that i.v. immunoglobulin has beneficial effects on motor dominant neuropathy in SS.- - - - - - - - - - ranking = 0.55023826854642keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/12. Autoimmune phenomena in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia.Autoimmune paraneoplastic syndromes are commonly encountered in patients with myelodysplastic syndromes (MDS). A review of case reports and small series suggest as many as 10% of MDS patients may experience various autoimmune syndromes. Clinical manifestations of such phenomena may include an acute systemic vasculitic syndrome, skin vasculitis, fever, arthritis, pulmonary infiltrates, peripheral polyneuropathy, inflammatory bowel disease, glomerulonephritis, and even classical connective tissue disorders, such as relapsing polychondritis. On the other hand, asymptomatic immunologic abnormalities have also been reported in these patients. These autoimmune manifestations frequently respond to immunosuppressive agents including steroids and occasional hematologic responses to steroid therapy have also been reported. We report five patients with history of MDS who manifested different spectrums of autoimmune phenomena including: pyoderma gangrenosum (PG), vasculitis, Coombs negative hemolytic anemia, idiopathic thrombocytopenia, and chronic inflammatory demyelinating polyneuropathy (CIDP). We also review the incidence, nature, course and response to therapy of these manifestations and discuss potential pathogenic mechanisms.- - - - - - - - - - ranking = 0.0095161124858409keywords = demyelinating (Clic here for more details about this article) |
8/12. CNS demyelination in autoimmune diseases.autoimmune diseases represent a diverse group of disorders that have generally of unknown etiology and poorly understood pathogenesis. They may be organ-specific or systemic, giving rise to overlapping syndromes; more than one autoimmune disease may occur in the same patient. Numerous case reports have documented that multiple sclerosis (MS) may be present concurrently with other autoimmune diseases, most commonly rheumatoid arthritis, autoimmune thyroid disease, type I diabetes mellitus and pernicious anemia. case reports of disseminated encephalomyelitis (DEM) coincidental with other autoimmune diseases are rare. Many of systemic autoimmune diseases cause central nervous system (CNS) demyelination and are frequently then diagnosed as MS, whereas they often are instances of DEM, the result of vascular, granulomatous or postinfectious manifestations. We have reviewed 15 patients with autoimmune diseases and CNS demyelination in order to determine the nature of the demyelinating process.- - - - - - - - - - ranking = 0.0095161124858409keywords = demyelinating (Clic here for more details about this article) |
9/12. Acute and chronic demyelinating inflammatory polyradiculoneuropathy. association with autoimmune diseases and lymphocyte response to human neuritogenic protein.Of 66 patients (31 female and 35 male) with demyelinating inflammatory polyradiculoneuropathy (DIP), 12% (8/66) had a chronic relapsing and/or progressive course (CR-DIP) and 88% (58/66) had an acute monophasic illness (acute guillain-barre syndrome or GBS). Ten (15%) of the 66 had one or more associated putative autoimmune diseases; of these ten, five had CR-DIP and five had GBS. Cell-mediated immune responsiveness (CMI) of 30 cases with DIP was tested in vitro by lymphocyte transformation. peripheral nervous system neuritogenic protein (NP) and central nervous system encephalitogenic myelin basic protein were the challenge antigens. Eighteen (60%) of the 30 patients had CMI to NP of human peripheral nervous system origin when a stimulation index (SI) of 2 or more was evaluated as positive; eight 27% (1) had CMI to NP when a positive SI was 3 or more. Of the 44 control patients with other neuropathies, only two (4.6%) demonstrated CMI to NP (SI, greater than or equal to 2). The in vitro response of patients with DIP to myelin basic protein (7/30) was not significantly different from that of the control population (16/44). The high incidence of DIP associated with autoimmune diseases and the CMI to NP in this group suggest that DIP may be an autoimmune disease with NP as one possible major antigen.- - - - - - - - - - ranking = 1.6427526216845keywords = polyradiculoneuropathy, demyelinating (Clic here for more details about this article) |
10/12. IgM monoclonal gammopathy and neuropathy in two siblings.A sister and a brother with a progressive mixed axonal and demyelinating polyneuropathy were found to have a monoclonal IgM gammopathy of kappa and lambda type, respectively. sural nerve and cutaneous nerve specimens obtained by biopsy showed deposits of IgM on myelin sheets. Sera from both patients contained antibodies directed to bovine peripheral nerve myelin as determined by ELISA technique and to normal human peripheral nerve myelin as demonstrated by indirect immunofluorescence histochemistry. These siblings may have a genetic predisposition to the formation of autoantibodies with peripheral nerve myelin as the target for the immune attack.- - - - - - - - - - ranking = 0.0095161124858409keywords = demyelinating (Clic here for more details about this article) |
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