Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The polyneuropathy, organomegaly, edema, monoclonal protein, and skin changes (POEMS) syndrome is a multisystem disorder of unknown etiology. Neurologic manifestations include polyneuropathy, optic disk edema, and intracranial hypertension. We studied a patient with POEMS syndrome who had an infiltrative orbitopathy.
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PMID:Infiltrative orbitopathy, optic disk edema, and POEMS. 653 11

A 48-year-old woman had a variation of the syndrome of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (the so-called POEMS syndrome). The patient's neurological findings were entirely normal, but she had splenomegaly, hyperprolactinemia with galactorrhea and oligomenorrhea, a thyroid nodule with evidence of mild thyroiditis on aspiration biopsy specimen, and IgG-kappa monoclonal gammopathy, and hyperpigmentation and thickening of the skin. A short course of plasmapheresis (twelve 4-L exchanges in one month) did not alter any of the clinical abnormalities, but did result in a 70% decrease in the monoclonal IgG level (from 2.2 to 0.7 g/dl).
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PMID:POEMS syndrome: studies in a patient with an IgG-kappa M protein but no polyneuropathy. 680 33

Three patients with an unusual multisystemic syndrome characterized by polyneuropathy, organomegaly *especially hepatosplenomegaly), endocrine dysfunction, M-protein, and skin abnormalities (POEMS syndrome) are discussed. Characteristic radiographic features include single or multiple osteosclerotic lesions and peculiar variety of bony proliferation. The relationship of the disorder to multiple myeloma and plasmacytoma and the pathogenesis of the skeletal abnormalities remain obscure.
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PMID:Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the POEMS syndrome. Distinctive radiographic abnormalities. 724 23

We report the case of a 33-year-old Japanese male who presented with thrombocytosis, lower limb edema, severe polyneuropathy with elevated cerebrospinal fluid (CSF) protein level and serum IgA lambda monoclonal component, fulfilling the manifestations of Crow-Fukase syndrome. A high level of soluble interleukin-6 receptor in the CSF was also found, which fluctuated in parallel with the clinical course. Initial treatment with double-filtration plasmapheresis (DFPP) reduced the serum IgA paraprotein level with improvement of the sensory component of the polyneuropathy and decrease of soluble interleukin-6 receptor in the CSF. The remaining clinical features waned off after steroid treatment. The possible role of interleukin-6 in the pathogenesis of the Crow-Fukase syndrome and the utility of DFPP treatment are discussed.
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PMID:A case of Crow-Fukase syndrome with elevated soluble interleukin-6 receptor in cerebrospinal fluid. Response to double-filtration plasmapheresis and corticosteroids. 748 19

A 57-year-old woman with Crow-Fukase syndrome presented thiamine deficiency and pulmonary hypertension of unknown etiology. After oral administration of prednisolone and thiamine, echocardiogram showed marked improvement of the pulmonary hypertension. To our knowledge, this is the first case of this syndrome associated with thiamine deficiency and precapillary pulmonary hypertension, which may play a role in the pathogenesis of polyneuropathy and heart failure of this syndrome.
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PMID:Thiamine deficiency and pulmonary hypertension in Crow-Fukase syndrome. 749 83

Immunofluorescent staining (immunofluorescence bone marrow aspirate) and immunoperoxidase staining (immunoperoxidase bone marrow biopsy) were compared in 26 patients with plasma cell dyscrasia and less than 10% marrow plasma cells. Their clinical diagnoses included monoclonal gammopathy of undetermined significance (13 patients), treated multiple myeloma (four patients), multiple myeloma with less than 10% marrow plasma cells (two patients), primary systemic amyloidosis (two patients), monoclonal gammopathy of undetermined significance with neuropathy (two patients), angiofollicular lymph node hyperplasia (two patients, all with the POEMS [polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes] syndrome), and primary (amyloidosis) amyloid neuropathy (one patient). The percentage of plasma cells was greater than 5% in 23% of patients and less than or equal to 5% in 77% of patients. With immunofluorescence bone marrow aspirate and immunoperoxidase bone marrow biopsy, light-chain restriction was demonstrated in 84% of all cases and accurately determined in 96% of all cases as shown by serum and urine paraprotein analysis or tissue amyloid typing. Monoclonal populations of plasma cells can be readily identified with immunofluorescence bone marrow aspirate and immunoperoxidase bone marrow biopsy in most patients with paraproteins and marrow plasmacytoses not diagnostic of multiple myeloma.
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PMID:Bone marrow aspirate immunofluorescent and bone marrow biopsy immunoperoxidase staining of plasma cells in histologically occult plasma cell proliferative marrow disorders. 752 Feb 28

Castleman's disease is a rare clinicopathological entity characterized by multicentric angiofollicular lymph node hyperplasia. Peripheral neuropathy has infrequently been described in patients with Castleman's disease. A patient is described who presented initially with severe painful sensorimotor polyneuropathy of his lower limbs diagnosed as a plasma cell variant of Castleman's disease associated with features of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin change). The patient was treated with plasmapheresis, immunosuppressive agents, and intensive rehabilitation. His functional status improved from being wheelchair bound to ambulating independently with crutches. Clinicians should be alert to the relationship of Castleman's disease and mixed polyneuropathy because physical improvement is possible with treatment. Also, the evaluation of patients presenting with peripheral neuropathy of unknown etiology and lymphadenopathy should include lymph node biopsy to rule out Castleman's disease.
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PMID:Severe polyneuropathy: initial manifestation of Castleman's disease associated with POEMS syndrome. 760 93

We describe a patient with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) who was found to have renal involvement with particular renal pathological findings. So far, 17 other cases, most of them from Japan, of POEMS syndrome with renal involvement, have been published. Clinical features are variable: acute renal failure with anasarca or moderate chronic renal insufficiency with mild proteinuria. This latter presentation often passes unnoticed. There is no severe hypertension, no microangiopathic hemolytic anemia. Renal biopsy shows prominent glomerular changes which are unusual and distinct from membranoproliferative glomerulonephritis (MPGN) and from glomerular thrombotic microangiopathy (TMA). Mesangial proliferation and thickening of the capillary wall with double contour on light microscopy suggest an MPGN. By immunofluorescent microscopy, no immunoglobulins or complement deposits were found. The occurrence of mesangiolytic lesions has led to the term of "mesangiolytic glomerulonephritis". The presence, on electron microscopy, of lucent subendothelial spaces could indicate TMA. But there are neither thrombi nor arteriolar changes. We are inclined to consider that the microangiopathic lesions are due to chronic injury of glomerular endothelial cells, exacerbated at outbreaks of the disease. Increased production of IL 6 could support the efficacy of corticosteroid therapy, particularly in acute clinical situations.
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PMID:Renal involvement in POEMS syndrome. 763 52

We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
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PMID:The POEMS syndrome: report of six cases. 769 93

The association of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes forms a characteristic multisystem syndrome, Crow-Fukase syndrome, or acronym POEMS syndrome. The pathogenesis of the syndrome is still unknown. An M component found in more than three quarters of the patients appears not be the direct cause, because no evidence of deposition or interaction was observed in skin, peripheral nerve or endocrine organs with few exceptions. Solitary or multiple bone lesions, or plasmacytoma, are found in more than half of patients with Crow-Fukase syndrome. The nature of abnormal plasma cells of Crow-Fukase syndrome seems to be distinct from that of multiple myeloma. Substances elaborated by plasmacytoma cells are recently postulated as a pathogenesis of the disorder because of the finding that the Crow-Fukase syndrome may regress after resection or irradiation of solitary plasmacytoma, although a definite conclusion awaits further investigations.
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PMID:[Crow-Fukase syndrome]. 769 17


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