1,721,018 research outputs found

    Recurrent myelitis in common variable immunodeficiency successfully managed with high-dose subcutaneous immunoglobulin.

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    Acute myelitis is an aetiologically heterogeneous inflammatory disorder of the spinal cord. We report on a 71-year-old woman with a recurrent cervical and thoracic myelitis who presented with a new relapse of the disease. Neuromyelitis optica was ruled out such as other possible causes of acute and/or recurrent myelopathy. Serum immunoglobulin levels and specific antibody responses were consistent with the diagnosis of common variable immunodeficiency (CVID). She was treated with high-dose methylprednisolone and intravenous immunoglobulin. As a remission-maintaining drug, we decided to treat her with subcutaneous immunoglobulin (CSL Behring) at 0.2 g/kg/week at doses higher than usually employed in replacement therapy in CVID. At 3-year follow-up, the response to treatment was good. No relapses occurred. Our case suggests the effectiveness and safety of subcutaneous immunoglobulin in maintaining remission and in sparing prednisone in a woman with recurrent myelitis associated with CVID

    Intravenous Immunoglobulin and Mycophenolate Mofetil for Long-Standing Sensory Neuronopathy in Sjögren's Syndrome

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    Sensory neuronopathy is described in association with the Sjögren’s syndrome (SS). We studied a 55-year-old woman with a 4-year history of progressive asymmetric numbness, distal tingling and burning sensation in upper and lower limbs. In a few months she developed ataxia with increased hypo-anaesthesia. Electrodiagnostic tests revealed undetectable distal and proximal sensory nerve action potential in upper and lower limbs. Cervical spine magnetic resonance showed a signal hyperintensity of posterior columns. Previous treatment with high-dose glucocorticoids and azathioprine was ineffective. A combined treatment with intravenous immunoglobulin and mycophenolate mofetil was followed by a progressive and persistent improvement. This case documented the efficacy and the safety of co-administration of intravenous immunoglobulin and mycophenolate mofetil in sensory neuronopathy associated with SS refractory to conventional immunosuppressive therapy
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