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Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paraproteinaemia and neuropathy are each relatively frequent and may be associated by chance. However, a number of significant relationships have to be ruled out in the differential diagnosis. Malignant gammopathy should be excluded: multiple myeloma can lead to compression of the spinal cord or cauda equina; primary amyloidosis is occasionally involved; the rare but intriguing POEMS syndrome, consisting of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes, usually accompanies osteosclerotic myeloma. It can be associated with angio-follicular lymph node hyperplasia and needs to be recognized because radioablative therapy is curative. The 'benign' monoclonal gammopathies of undetermined significance, known as MGUS, are much more frequent. There is an IgM MGUS group with predominantly distal sensorimotor demyelinating polyneuropathy and another rather heterogeneous group with IgG or IgA MGUS and a tendency to a favourable response to plasmapheresis. The role of the monoclonal IgG and IgA antibodies is unclear. This chapter has focused on the pathogenetic mechanisms of neuropathies associated with IgM MGUS. In the majority of cases, monoclonal autoantibodies specific for particular carbohydrate epitopes bind to myelin and are now recognized as the primary cause of the disease manifestations, including widening of the myelin lamellae. While the autoantibodies have been shown to bind complement, the presence of inhibitors is invoked to explain the absence of acute inflammatory changes. The epitopes recognized with the highest affinity by the auto-antibodies are present on the myelin-associated glycoprotein (MAG) and could interfere with cell adhesion and cellular signally processes. In addition, binding to antigenically similar glycoproteins, such as PO, PMP-22 and some acidic glycolipids, may be a contributory factor. It is generally accepted that the anti-MAG autoantibodies are inducing a progressive demyelinating polyneuropathy by modifying axon-Schwann cell interactions.
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PMID:Paraproteinaemic neuropathies. 873 9

POEMS syndrome is a systemic disorder with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The association of POEMS syndrome with lympho-proliferative disorder is very commun. The pathogenesis remains poorly understood but implication of cytokines (interleukins 1 and 6) is suspected. We report a case of a classic POEMS syndrome (with polyneuropathy, hepatomegaly, diabetes melitus, hyperpigmentation, monoclonal IgG lambda, anasarca and solitary plasmocytoma), associated with high serum levels of interleukin 6.
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PMID:[POEMS syndrome: role and value of interleukin-6]. 878 86

A 65-year old man presented with complaints of sclerosis of skin and numbness in the extremities. During last 10 year, he had developed monoclonal gammopathy, Raynaud's phenomenon, ischemic heart disease, sigmoid colon cancer, hyperkalemia, polyneuropathy and scleroderma-like skin changes. Laboratory examinations revealed a monoclonal protein (IgA-lambda) and an elevated serum level of IL-6. Subsequently a diagnosis of POEMS syndrome was made based on the clinical features and laboratory findings which were characteristic of this syndrome. Further examinations showed the presence of glomerulonephritis and brain tumor. These various complications are of great interest in understanding the pathogenesis of POEMS syndrome.
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PMID:[A case of POEMS syndrome with various complications]. 881 May 50

Metabolic bone disease is a major cause of morbidity and mortality in patients suffering from multiple myeloma. This usually results from an imbalance between the osteoclast and osteoblast activity of bone resorption and formation due to the secretion of an osteoclast-activating factor by the myeloma cells. This generally takes the form of lytic lesions, hypercalcaemia and osteoporosis but, in a minority of patients, osteosclerosis is a striking feature. In a proportion of patients with gammopathy and the osteosclerotic form of the disease, there appears to be an association with other symptom complexes including Polyneuropathy, organomegaly, endocrine changes, and skin abnormalities. This article summarizes the clinical, radiological and laboratory features of this syndrome, which is known by the acronym 'POEMS'. It is important to recognize this condition, as it carries a better prognosis than the more common lytic form of the disease and because vincristine, and agents frequently used to treat this condition, may severely exacerbate the neuropathy and should be avoided. This syndrome is also important because it may give some insight into the pathophysiology of the plasma-cell dyscrasias.
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PMID:Osteosclerotic myeloma and 'POEMS' syndrome. 881 38

A 65-year-old woman with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) developed severe upper right quadrant colic with jaundice, massive haematemesis and melaena following an apparently uncomplicated percutaneous liver biopsy performed for suspected liver involvement. Symptom onset occurred about 18 hours after the procedure. Haemobilia was diagnosed on endoscopic retrograde cholangiopancreatography. An arteriovenous fistula in the third hepatic segment was demonstrated by transfemoral arteriography and successfully embolized using Gelfoam pledgets mixed with Ivalon fragments. A review of percutaneous liver biopsy related haemobilia is presented.
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PMID:Massive haemobilia after percutaneous liver biopsy in a patient with POEMS syndrome successfully treated by arterial embolization. 882 76

Pulmonary hypertension (PH) is an uncommon but lethal complication of some connective tissue diseases. We describe two cases of PH associated with plasma cell dyscrasia, with polyneuropathy (P), organomegaly (O), endocrinopathy (E), M protein (P) and skin changes (S) described as POEMS syndrome. Two patients, one female and one male, were hospitalized for dyspnoea. Both had a history of POEMS syndrome a few years previously. PH was diagnosed from cardiac catherization, and complete investigations did not reveal other underlying disease. In Case No. 1, mean pulmonary artery pressure was 40 mmHg and pulmonary vascular resistance was 775 dyne x s x cm-5 x m2, with a significant reversibility to vasodilators. Nifedipine and fluindrone treatment was initiated. The patient was still alive 18 months later. In Case No. 2, mean pulmonary artery pressure was 28 mmHg and pulmonary vascular resistance was 243 dyne x s x cm-5x m2, with a slight reversibility to vasodilators. The patient died 6 months later. Our observations suggest that pulmonary hypertension (PH) may be associated with the POEMS syndrome, and that patients suffering from the POEMS syndrome and dyspnoea should be tested for PH.
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PMID:Pulmonary hypertension associated with POEMS syndrome. 886 8

The POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin Changes) is often unrecognized by ophthalmologists. We report the case of a 51-year-old female suffering from a POEMS syndrome with a bilateral optic disc edema.
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PMID:[Papilledema and POEMS syndrome. Apropos of a case]. 888 10

The Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes (POMEMS) syndrome is a rare multisystem disorder of obscure pathogenesis, associated with osteosclerotic myeloma. Unlike multiple myeloma without neuropathy, circulating levels of proinflammatory cytokines (IL-1 beta, TNF-alpha IL-6) are increased in patients with POEMS syndrome. Sites of IL-1 beta production include lymph node and bone marrow tissues. These data support the view that pleiotropic effects of proinflammatory cytokines released secondary to a strong activation of the monocyte/macrophage system, take part in the multisystemic expression of the disease.
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PMID:[Pro-inflammatory cytokines: a pathogenic key of POEMS syndrome]. 888 41

We describe two cases of POEMS syndrome, both with polyneuropathy, monoclonal gammopathy of the IgG lambda type, thickening of the skin with Raynaud phenomenon, multiple osteosclerotic lesions and hypothyroidism. One of them, also had papilledema, elevated cerebrospinal fluid protein, intracranial hypertension and phrenic nerve palsy; the other one had ascites and hepatosplenomegaly. Phrenic nerve palsy associated to this syndrome has not been described previously.
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PMID:[The POEMS syndrome, apropos of 2 cases and review of the literature]. 896 62

Approximately 10% of patients with peripheral neuropathy of otherwise unknown etiology have an associated monoclonal gammopathy. Both the neuropathies and the monoclonal gammopathies in these patients are heterogeneous, but several distinct clinical syndromes that may respond to specific therapies can be recognized. It is important to recognize these syndromes because monoclonal gammopathies also occur in 1% of the normal adult population, and in some cases, monoclonal gammopathies are coincidental and unrelated to the neuropathy. In patients with IgM monoclonal gammopathies, IgM M proteins frequently have autoantibody activity and are implicated in the pathogenesis of the neuropathy. IgM M proteins that bind to myelin-associated glycoprotein (MAG) have been shown to cause demyelinating peripheral neuropathy; anti-GM1 antibody activity is associated with predominantly motor neuropathy, and anti-sulfatide or chondroitin sulfate antibodies are associated with sensory neuropathy. The IgM monoclonal gammopathies may be malignant or nonmalignant, and polyclonal antibodies with the same specificities are associated with similar clinical presentations in the absence of monoclonal gammopathy. IgG or IgA monoclonal gammopathies are associated with neuropathy in patients with osteosclerotic myeloma or the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy myeloma, and skin changes). Amyloidosis or cryoglobulinemic neuropathies can occur with either IgM or IgG and IgA monoclonal gammopathies. Therapeutic intervention depends on the specific clinical syndrome but is generally directed at removing the autoantibodies, reducing the number of monoclonal B cells, and interfering with the effector mechanisms.
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PMID:Pathogenesis and therapy of neuropathies associated with monoclonal gammopathies. 896 15


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