1/78. poems syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.poems syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, poems syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that poems syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/78. Successful treatment of IgM paraproteinaemic neuropathy with fludarabine.OBJECTIVES: To evaluate the response of four patients with IgM paraproteinaemic neuropathy to a novel therapy-pulsed intravenous fludarabine. BACKGROUND: The peripheral neuropathy associated with IgM paraproteinaemia usually runs a chronic, slowly progressive course which may eventually cause severe disability. Treatment with conventional immunosuppressive regimens has been unsatisfactory. Fludarabine is a novel purine analogue which has recently been shown to be effective in low grade lymphoid malignancies. methods: Four patients were treated with IgM paraproteinaemic neuropathy with intravenous pulses of fludarabine. Two of the four patients had antibodies to MAG and characteristic widely spaced myelin on nerve biopsy and a third had characteristic widely spaced myelin only. The fourth had an endoneurial lymphocytic infiltrate on nerve biopsy and a diagnosis of Waldenstrom's macroglobulinaemia. RESULTS: In all cases subjective and objective clinical improvement occurred associated with a significant fall in the IgM paraprotein concentration in three cases. Neurophysiological parameters improved in the three patients examined. The treatment was well tolerated. All patients developed mild, reversible lymphopenia and 50% mild generalised myelosuppression, but there were no febrile episodes. CONCLUSION: Fludarabine should be considered as a possible treatment for patients with IgM MGUS paraproteinaemic neuropathy.- - - - - - - - - - ranking = 8.8390147621875keywords = nerve (Clic here for more details about this article) |
3/78. November 1998--70 year old woman with SLE, paraproteinemia and polyneuropathy.A 70 year old woman developed a sensory-motor polyneuropathy 12 years after almost continuous treatment with hydrochloroquine for SLE. sural nerve biopsy disclosed abundant lipid inclusions in schwann cells and vessels against a background of axonal degeneration. After cessation of therapy the patient improved moderately. This case underscores the usefulness of nerve biopsy in the study of management of patients with peripheral neuropathy associated with systemic illnesses.- - - - - - - - - - ranking = 8.8390147621875keywords = nerve (Clic here for more details about this article) |
4/78. 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 = 17.678029524375keywords = nerve (Clic here for more details about this article) |
5/78. adult-onset nemaline myopathy and monoclonal gammopathy: a case report.A 47-year-old female developed proximal limb weakness after hysterectomy for uterine fibromatosis. muscle strength slowly improved, but relapse occurred at age 52. She presented with progressive gait difficulty, proximal limb weakness, and painful calves. family history was not contributory. cranial nerves, deep tendon reflexes, and sensation were normal. serum creatine kinase was normal. An IgG kappa monoclonal protein was found. Nerve conduction studies were normal, but EMG showed brief small polyphasic motor unit action potentials with early recruitment in proximal muscles. Muscle biopsy showed abundant rods, atrophic muscle fibres, and type 1 fibre predominance. The sarcolemma was immunoreactive for IgG kappa. plasmapheresis was unsuccessful, but methylprednisolone and azathioprine led to moderate improvement of muscle strength, associated with reduced monoclonal protein levels. This is the third case report, describing the association of monoclonal gammopathy and late-onset nemaline myopathy. Presence of a monoclonal protein at the sarcolemma and responsiveness to immunosuppressive treatment are suggestive of a dys-immune origin.- - - - - - - - - - ranking = 4.4195073810938keywords = nerve (Clic here for more details about this article) |
6/78. Focal myelin swellings and tomacula in anti-MAG IgM paraproteinaemic neuropathy: novel teased nerve fiber studies.Focal myelin swellings and tomacula in teased nerve fibers from a case of IgM anti-myelin-associated glycoprotein (MAG) paraproteinaemic neuropathy were examined using a novel technique. Five different morphologic abnormalities were identified--myelin sheath outfolding, myelin sheath infolding, enlargement of the adaxonal space, myelin degeneration, multiple increased concentric loops--and a combination of these structural abnormalities often occur in association with myelin degeneration. Similar structural changes were found in externally normal segments of teased fibers without evidence of myelin swelling or tomacula from the same case. These structural abnormalities are consistent with a disturbance of the normal adhesion functions of MAG in the maintenance of axon-myelin relationships.- - - - - - - - - - ranking = 22.097536905469keywords = nerve (Clic here for more details about this article) |
7/78. High rate of monoclonal gammopathy among immunocompetent subjects with primary cytomegalovirus infection.serum samples from immunocompetent adults with primary cytomegalovirus (CMV) or Epstein-Barr virus (EBV) infection and from solid-organ or bone marrow transplant recipients with primary CMV infection were examined for paraproteins. Several immunocompetent patients with CMV infection but none with EBV infection presented with an M component, which implies that the M component connects CMV infection to a risk of B cell malignancy.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/78. Monoclonal gammopathy and spurious hypophosphatemia.BACKGROUND: Spuriously low levels of plasma phosphate have been reported previously in patients with multiple myeloma and polyclonal gammopathy. We report 2 cases of spurious hypophosphatemia in patients with elevated concentrations of serum monoclonal immunoglobulins, 1 of whom had monoclonal gammopathy of undetermined significance and the other multiple myeloma. methods: Plasma phosphate concentrations were measured using nondeproteinized and deproteinized plasma samples from patients with monoclonal gammopathies. RESULTS: In 2 patients with monoclonal gammopathy, the levels of plasma inorganic phosphate were reported as <1.0 mg/dL when the phosphate concentration was determined using an analyzer that employs nondeproteinized plasma. When the samples were reanalyzed using a laboratory method that removes serum proteins, normal or elevated concentrations of phosphate were found. Plasma levels of phosphate in 4 other patients with monoclonal gammopathy were normal by both methods. CONCLUSIONS: These data confirm previous reports that spurious hypophosphatemia occurs in some patients with increased levels of serum monoclonal immunoglobulins when laboratory methods using nondeproteinized samples are employed. The occurrence of unusually low plasma phosphate concentrations in patients without symptoms or clinically apparent causes of hypophosphatemia should alert physicians to search for monoclonal gammopathy.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/78. Microvasculitic paraproteinaemic polyneuropathy and B-cell lymphoma.Microvasculitis may play a greater part in the pathogenesis of paraproteinaemic neuropathies than is generally recognised, producing tissue destruction by convergent immune and physical mechanisms. We present a patient with a clinical syndrome of mononeuritis multiplex and a circulating IgM lambda paraprotein, in whom bone marrow aspiration revealed a lymphoplasmacytoid lymphoma. Microvasculitic changes were present in the first nerve biopsy, and the second showed extensive destruction of neural architecture and deposition of IgM-related material. A 2-stage pathogenic cascade is postulated and explored with a review of the relevant literature.- - - - - - - - - - ranking = 4.4195073810938keywords = nerve (Clic here for more details about this article) |
10/78. Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia.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.- - - - - - - - - - ranking = 8.4195073810938keywords = nerve, organ (Clic here for more details about this article) |
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