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where the disease is prevalent In many cases of POEMS there is lymphadenopathy attributable to the angiofollicular hyperplasia of Castleman disease Another characteristic feature of the osteosclerotic-related polyneuropathy is a greatly elevated CSF protein The presence of the disease can be suspected from the presence of an immunoglobulin spike in the blood, often polyclonal or biclonal rather than monoclonal, and possessing a lambda light chain component The diagnosis requires the demonstration of the osteosclerotic lesion by a radiographic survey of the long bones, pelvis, spine, and skull (a bone scan is often insensitive) and a bone marrow examination, which shows a moderate increase in the number of well-differentiated plasma cells In most of our patients there have been several discrete bone lesions concentrated in the ribs and spine; the skull and long bones may harbor such lesions as well, or the lesions may be in the form of a single nodule, often in the spinal column Treatment The neuropathy that complicates a solitary plasmacytoma may improve markedly following irradiation of the bone lesion Multiple lesions, including those in the POEMS syndrome, when treated with chemotherapy (melphalan and prednisone) or focused radiation, demonstrate some improvement or stabilization in the neuropathy Treatment with plasma exchange has yielded uncertain but somewhat positive results in our patients Waldenstrom Macroglobulinemia Macroglobulinemia was the term applied by Waldenstrom to a systemic condition occurring mainly in elderly persons and characterized by fatigue, weakness, and a bleeding diathesis Immunoelectrophoretic examination of the blood disclosed a marked and mostly monoclonal increase in the IgM plasma fraction About half of patients with Waldenstrom disease and polyneuropathy will have anti-MAG antibodies, similar to the proportion in patients with nonmalignant IgM paraproteins A signi cant proportion of patients with this hyperproteinemia have a hyperviscosity state manifest by diffuse slowing of retinal and cerebral circulation giving rise to episodic confusion, coma, impairment of vision, and sometimes strokes (Bing-Neel syndrome) The neuropathy, when present, evolves over months or longer and may be asymmetrical, particularly at the onset, but becomes symmetrical and distal The pattern in our patients has been of the latter type, very slowly progressive, and limited to the feet and legs with mild sensory ataxia and loss of knee and ankle jerks The CSF protein is usually elevated and the globulin fraction increased In a case recorded by Rowland and colleagues, the polyneuropathy was purely motor and simulated motor neuron disease; other cases of this type, albeit rare, have been brought to our attention in clinical exercises and medical writings Treatment is discussed below Cryoglobulinemia As mentioned previously in the section on vasculitic neuropathies, cryoglobulin, a serum protein that precipitates on cooling, is usually of the IgG or IgM type and most often polyclonal While cryoglobulinemia may occur without any apparent associated condition (essential cryoglobulinemia), it also accompanies a wide variety of disorders such as mulltiple myeloma, lymphoma, connective tissue disease, chronic infection, and, particularly, hepatitis C Peripheral neuropathy occurs in a small proportion both of the essential and symptomatic cases Occasionally the neuropathy evolves over a period of a few days and remits rapidly More often it takes the form of a distal symmetrical sensorimotor loss, which develops insidiously in association with the Raynaud phenomenon and purpuric eruptions of the skin Initially, the neuropathic symptoms consist only of pain and paresthesias that may be precipitated by exposure to cold (as often, there is no. birt barcode tool How to add barcodes using free Eclipse BIRT barcode generator ...
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