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 Crow-Fukase syndrome or POEMS syndrome (Polyneuropathy, Organomegaly, Edema, Monoclonal protein, Skin changes) is an uncommon plasma cell dyscrasia leading to a multisystemic disorder. An optic disc swelling is often related to this syndrome. We report the case of a 51-year-old man with a papilledema. We presume that vasculitis or plasma cell infiltration is the cause of the papilledema.
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PMID:[POEMS syndrome and papilledema]. 130 37

We report here the case of a 20-year-old man with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M proteins, skin changes). This rare syndrome followed a 3-year history of a syndrome that mimics a chronic inflammatory demyelinating polyneuropathy (CIDP). Treatment with cyclophosphamide induced regression of the syndrome and improved peripheral nerve conduction.
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PMID:POEMS syndrome: follow-up study of a case. 132 2

POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein (M-protein), and skin changes. The authors describe a patient with POEMS syndrome who had osteosclerotic myeloma confirmed by open bone biopsy. Magnetic resonance imaging (MRI) showed discrete lesions of low signal intensity in both T1 and T2-weighted images. This patient is now being successfully treated with melphalan and prednisone with much improvement in skin thickening and sensory change in the lower extremities.
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PMID:POEMS syndrome--a case report. 141 69

The POEMS syndrome is an infrequently reported multisystem disorder which presents usually as an obscure polyneuropathy, with almost all cases reported in Japan. A 64 yr old caucasian man presented with a 12 mth history of a severe sensorimotor neuropathy in association with dermato-endocrine features. Detection of a monoclonal IgG kappa paraprotein and mixed osteosclerotic/lytic bone lesions consistent with a plasma cell dyscrasia led to diagnosis of the POEMS syndrome. Unique ultrastructural features were present on sural nerve biopsy in addition to the unusual association with monoclonal kappa-light chain. This case illustrates that the POEMS syndrome may also occur in caucasian subjects.
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PMID:Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia. 143 99

A new case of optic disk swelling associated with POEMS syndrome is described. This is a rare disease, but it should be suspected in cases of bilateral optic disk swelling when defining systemic components are present: polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal component (M) and skin changes (S). Usually visual acuity is maintained. However, loss of vision secondary to optic disk swelling and associated macular edema could be found.
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PMID:[Papilledema and POEMS syndrome]. 147 93

A 58-year-old man developed polyneuropathy, organomegaly, gynecomastia, skin pigmentation, and multiple myeloma (IgG-lambda type). Although transient clinical improvement was obtained with prednisolone and cyclophosphamide, his general condition deteriorated progressively, and he died 3 years after onset. Lymph nodes showed angiofollicular hyperplasia, and the sural nerve revealed segmental demyelination. At autopsy, the glomeruli were enlarged and showed mild mesangial cell proliferation with segmental mesangiolysis and proliferation of endothelial cells forming abnormal vessels. The massive volume of ascites contained a high level of interleukin-6 (IL-6). IL-6, a multifunctional cytokine, may be the pathogenic factor which induces the various clinical symptoms and pathological features of Crow-Fukase (POEMS) syndrome.
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PMID:An autopsy case of Crow-Fukase (POEMS) syndrome with a high level of IL-6 in the ascites. Special reference to glomerular lesions. 147 60

A rare association of multisystemic manifestations with plasma cell dyscrasia has been termed POEMS syndrome, which includes polyneuropathy, organomegaly, endocrinopathy. M-protein, and skin changes. From literature reports, organomegaly consists of hepatosplenomegaly and/or lymphadenopathy. We here present a case of POEMS syndrome that had most of the typical features of POEMS, except that organomegaly only involved the heart. The patient's multiple clinical manifestations, including cardiomyopathy, improved with chemotherapy, which is characteristic of POEMS syndrome. However, cardiomegaly or cardiomyopathy have not previously been described with POEMS. The present case suggests that cardiomegaly and cardiomyopathy may be added to the organomegaly in POEMS.
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PMID:POEMS syndrome presenting with cardiomegaly and cardiomyopathy. 153 13

A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. Biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.
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PMID:Polyneuropathy with osteosclerotic myeloma--POEMS syndrome. A case report. 154 70

POEMS syndrome [plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, skin changes] although mimicking the connective tissue diseases in several ways has not been reported in a patient who also meets criteria for the diagnosis of systemic lupus erythematosus (SLE). We describe a patient with POEMS syndrome and SLE and review the similarities and differences between POEMS syndrome and other connective tissue disorders.
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PMID:POEMS syndrome in systemic lupus erythematosus. 161 12

The authors present the results of an autopsy of a 67-year-old Japanese man with POEMS/Takatsuki/Crow-Fukase syndrome (P/T/CFS) diagnosed in 1972. Each component of the syndrome was gradually recognized after the resection of lumbar vertebral solitary plasmacytoma in 1967. The patient died in 1989 of generalized infection and renal failure. Autopsy revealed in the vertebral canal between the fifth and seventh thoracic vertebrae dorsal pachymeningeal fibrosis, with prominent amyloid deposition that oppressed the spinal cord. This condition was consistent with the final neurologic manifestation of the patient, bilateral motor and sensory disturbance below the sixth thoracic level. Myelopathy remained clinically unnoticed because neurologic disturbance had begun as peripheral polyneuropathy. Normocellular marrow with heterogeneously scattered lambda light chain-positive plasma cells and degeneration of the myelinated fibers of sciatic nerve also were observed. This is the first report of focal spinal amyloidosis associated with P/T/CFS.
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PMID:Report of a patient with POEMS/Takatsuki/Crow-Fukase syndrome associated with focal spinal pachymeningeal amyloidosis. 164 21


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