Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031117 (peripheral neuropathy)
 10,577 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presence of a monoclonal protein in the serum of a patient with peripheral neuropathy raises the suspicion of systemic amyloidosis, POEMS syndrome, macroglobulinemia, multiple myeloma, or lymphoma. If these conditions are excluded, the patient is classified as having a monoclonal gammopathy of undetermined significance (MGUS) with an associated neuropathy. Approximately one half of patients with peripheral neuropathy and IgM monoclonal gammopathy have IgM antibodies that bind to a myelin-associated glycoprotein (MAG). In addition to MAG, other antigens such as glycolipids or gangliosides represent other target antigens. Monoclonal gammopathies and motor neuron diseases have also been reported. Sensorimotor peripheral neuropathy occurs in about 15% of patients with primary amyloidosis but is uncommon in multiple myeloma.
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PMID:Monoclonal proteins in neuropathy. 132 50

A 40 year old Chinese woman presented with anasarca and later developed features of POEMS syndrome. These are peripheral neuropathy (P), organomegaly (O), endocrine dysfunction (E), monoclonal gammopathy (M), and skin changes (S) which are usually associated with plasma cell dyscrasia. In our patient, monoclonal gammopathy was not detected on immunofixation electrophoresis and was revealed only after analysis of kappa/lambda light chain ratio of the raised serum IgA immunoglobulin. Needle liver biopsy of her grossly enlarged liver showed marked accumulation of glycogen and presence of giant mitochondria in the hepatocytes, a feature not previously reported.
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PMID:Poems syndrome. 174 70

This report deals with a case of double gammopathy (IgM-kappa, IgG-lambda) with Crow-Fukase syndrome, which developed into primary macroglobulinemia four years after the diagnosis. In May 1980, a 74-year-old woman was admitted to the hospital because of a rapid progression of peripheral neuropathy. The patient was diagnosed as having Crow-Fukase syndrome from the following data: albumin-cytologic dissociation of cerebrospinal fluid, peripheral edema, diffuse hyperpigmentation of the skin, diabetic glucose intolerance, serum double gammopathy (IgM-kappa, IgG-lambda) and hepatomegaly. The administration of prednisolone yielded the improvement of neuropathy. In December 1984, serum IgM level was increased from 104 mg/dl to 3,025 mg/dl. Plasma cells in the bone marrow increased in the percentage from 5.6% to 18.4%, and then Bence Jones protein (kappa type) was excreted in the urine. No antibody activity to myelin antigens was detected in the serum. The patient died of cerebral infarction in 1985. At postmortem examination, lymphomatous involvement was found in the jejunum. At the immunohistological examination of the tumor specimens, the morphology and the distribution of IgM- and IgG-positive cells corresponded to that of kappa- and lambda-positive cells, respectively. A small number of cells containing both kappa and lambda light chains were also demonstrated. It seems likely that IgM (kappa)- and IgG (lambda)-positive cells were derived from the common precursor cells.
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PMID:[Progression from Crow-Fukase syndrome with double gammopathy (IgM-kappa, IgG-lambda) to primary macroglobulinemia]. 194 31

Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castleman's disease, an unusual lympho-proliferative disorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders.
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PMID:POEMS syndrome, osteosclerotic myeloma and Castleman's disease: a case report. 195 37

We reviewed charts of patients seen at Mayo Clinic Rochester and Mayo Clinic Jacksonville to determine the importance and frequency of optic disk swelling in patients with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). A computer search identified 148 patients who were classified as having a monoclonal gammopathy and peripheral neuropathy. A review of the 148 charts disclosed that 11 patients had POEMS syndrome. Eight of these 11 patients had optic disk swelling identified by an ophthalmologist. In all eight patients, optic disk swelling was either the initial sign or was present the first time we saw the patient. Optic disk swelling may be an early sign in POEMS syndrome.
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PMID:Optic disk swelling with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). 215 13

Four cases of plasma cell type Castleman's disease (CD) are described. Two patients had localized forms (one mediastinal and the other mesenteric) and presented systemic manifestations associated with hypergammaglobulinemia and severe anemia. In both cases, the lesions were revealed by computerized tomography scans and cures were obtained by the complete surgical removal of the masses, which led to the rapid disappearance of the systemic manifestations. The other 2 patients had the multicentric form of CD and presented more extensive clinical and biological symptoms. One of these developed severe peripheral neuropathy and endocrine anomalies during the late phase of his disease, which led us to discuss the relationship between multicentric CD and the POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change) syndrome first described in Japan. Three of our patients presented with hypochromic microcytic anemia too severe to be explained by an inflammatory syndrome alone, and was likely due to several mechanisms. The etiology of CD remains unknown. The histological characteristics of angiofollicular lymph node hyperplasia are among the most important criteria for the diagnosis of localized and multicentric forms of CD, which can easily be made on a lymph node biopsy. However, it must be noted that this lesion can also be observed (but only rarely) in HIV (human immunodeficiency virus) - infected patients. The localized form is always considered to be benign, but, to date, there is no formal argument definitively supporting the malignancy of the multicentric one, in spite of its clinical similarity to a lymphoproliferative syndrome.
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PMID:[Castleman's disease (giant lymph node hyperplasia): clinical, biological and developing polymorphism. Apropos of 4 cases]. 216 41

A 52-yr-old woman developed exudative ascites 2 yr after the onset of peripheral neuropathy. Extensive evaluation revealed that the patient had no underlying liver disease, malignancy, infection, or cardiac or renal disease. The ascites initially responded to high-dose corticosteroid therapy. The patient had many clinical features of the recently described POEMS syndrome, including a persistent IgA lambda-paraprotein. Initially, her ascites responded to treatment with steroids. This is characteristic of the syndrome and should be considered in patients with POEMS syndrome and refractory ascites.
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PMID:Refractory ascites due to POEMS syndrome. 253 29

POEMS or Crow-Fukase syndrome is a multisystemic, clinically malignant disorder of obscure etiology. Peripheral neuropathy and plasma cell dyscrasia are central features. The authors now report 7 Chinese patients with this syndrome in which PCD or paraproteinemia were absent in 6, and 2 had a lymph node histology resembling that of hyaline-vascular Castleman's disease. Immunological abnormalities consisted of either increased or decreased numbers of B- and T-cells in 2 cases, and an elevated OKT4/OKT8 ratio with paradoxical dissociation of the lymphocyte transformations to various concentrations and types of mitogens in 1 case. This suggests that the underlying abnormalities of POEMS syndrome are heterogeneous and that it may be an immunologically related syndrome of varying etiology.
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PMID:The POEMS syndrome among Chinese: association with Castleman's disease and some immunological abnormalities. 255 70

We report the case of a 46-year-old woman presenting with a disorder characterized by the association of multicentric Castleman's disease, organomegaly, a solitary IgA lambda myeloma with lytic bone lesions but no serum monoclonal peak, peripheral neuropathy and skin changes. This unusual association resulted in an incomplete form of that peculiar multisystem syndrome described under a variety of names: Crow-Fukase syndrome, Shimpo's syndrome, Takatsuki's syndrome, PEP syndrome or POEMS syndrome. The possible relationship between localized and multicentric Castleman's disease with myeloma or extramedullary plasmacytoma on one hand and on the other hand with polyneuropathy and the other symptoms of the POEMS syndrome is discussed.
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PMID:Multicentric angiofollicular lymph node hyperplasia associated with a solitary osteolytic costal IgG lambda myeloma. POEMS syndrome in a South American (Paraguayan) patient. 260 20

A patient with osteosclerotic myeloma and POEMS syndrome, unresponsive to pulse prednisone and melphalan therapy, was admitted to the hospital for a trial of plasma exchange therapy. The presentation included IgG lambda monoclonal gammopathy, peripheral neuropathy, hepatosplenomegaly, hyperpigmentation and thickening of the skin, edema, and tense ascites. Laboratory tests confirmed hypothyroidism, hypogonadism, and adrenal insufficiency. Six exchange procedures failed to affect the clinical course, and the patient died. Greater-than-one-plasma-volume exchanges (patient's measured plasma volume, 2,703 cc) were performed. When IgG and cholesterol removal were compared to the predicted removal, based on the volume of plasma removed, significantly less reduction in concentration than predicted was measured. IgG concentrations increased postapheresis and, at 2 weeks, three-fourths of the removed IgG had reaccumulated. A reduced efficiency of removal of both IgG and cholesterol can be explained by postulating increased vascular permeability with free exchange of soluble substances from one compartment to another. If an abnormal product is produced by the disease and is responsible for the clinical syndrome, a more intensive schedule of plasma exchange therapy may be needed to achieve a sustained depletion of the responsible soluble substance. Alternatively, neither increased vascular permeability or the clinical manifestations are responsive to removal of a soluble substance or are caused by a soluble substance produced by the malignancy.
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PMID:Therapeutic trial of plasma exchange in osteosclerotic myeloma associated with the POEMS syndrome. 299 55


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