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1/38. Vasculitic neuropathy in a patient with inactive treated lepromatous leprosy.

    A 46 year old Asian male with previously treated lepromatous leprosy developed a stepwise multifocal sensory disturbance 25 years later. neurophysiology demonstrated marked deterioration from previous studies. sural nerve biopsy disclosed a vasculitic process superimposed on inactive lepromatous leprosy. Immunocytochemical stains for mycobacterial antigen showed deposits within nerve and vessel walls. A delayed vasculitic neuropathy precipitated by persisting mycobacterial antigen is proposed.
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2/38. A possible novel isoform of peripheral myelin p0 protein: a target antigen recognized by an autoantibody in a patient with malignant lymphoma and peripheral neuropathy.

    We tried to characterize a 35-kD antigen recognized by the serum IgG of a patient with malignant lymphoma and peripheral neuropathy. By Western blotting, the serum IgG reacted with the 35-kD antigen in the human, bovine and mouse peripheral nerve (PN) but not with other neural and non-neural tissues. Immunohistochemical analysis showed immunoreactivity of the IgG in the compact myelin of PN. We constructed a human sciatic nerve cDNA library and screened it using IgG of the patient. Three independent clones were obtained. sequence alignment indicated that the inserts of these clones were homologous to the P0 cDNA, but that all three corresponded to the 3'-untranslational region of the P0 cDNA. To biochemically analyze the 35-kD antigen, myelin fractions of the human and bovine PN were prepared. The 35-kD antigen was purified from the crude myelin fraction by sodium dodecyl sulfate (SDS)-polyacrylamide gel electrophoresis. When the immunoreactivities of the 35-kD antigen for the IgG of the patient and a monoclonal anti-P0 antibody were compared with those of P0 protein for these antibodies, the 35-kD antigen reacted with both antibodies, but the P0 protein reacted with only the monoclonal anti-P0 antibody. These results suggest that the 35-kD antigen is an isoform of P0 protein. Although it is unlikely that the autoantibody may be the primary cause of neuropathy, because they were also detected in patients with lymphoma without overt neuropathy, they appear to be a modifying factor in the progression of neuropathy.
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3/38. Vasculitic neuropathy in hiv infection: a clinicopathological study.

    vasculitis causing peripheral neuropathy may be the first sign of hiv infection. We report four such cases in whom the onset of peripheral neuropathy led to the detection of hiv infection. Two patients presented with features of mononeuritis multiplex, while the other two had a lumbosacral polyradiculopathy. A prior history of blood transfusion was forthcoming in one of the patients. sural nerve biopsies in all the four cases and the muscle biopsy in two, histologically showed evidence of vasculitis. Immunohistochemically, the viral antigen was not demonstrable in any of the biopsies, but on electron microscope, virus-like particles were identifiable in the Schwann cell cytoplasm and the perivascular macrophages in one case. To the best of our knowledge, this is the only report that has documented the virus in the schwann cells as well as the perivascular macrophages lending credence to the fact that these viruses are neurotropic as well as lymphotropic. Immunoglobulin deposits were not demonstrable in any of the cases, suggesting that direct viral invasion may have a role in the pathogenesis of peripheral nerve vasculitis.
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4/38. Detection of cytomegalovirus antigens in phagocytosed serum complexes from a patient with rheumatoid arthritis, vasculitis, peripheral neuropathy, cutaneous ulceration, and digital gangrene.

    A patient with rheumatoid arthritis, vasculitis, peripheral neuropathy, cutaneous ulceration, and digital gangrene was studied. Circulating immune complexes were detected by C1q binding although serum complement levels were within the normal range. Immunofluorescent staining of buffy coat cells with specific antisera showed the presence of IgG and IgM in phagocytosed inclusions but complement c3 was not detected. A monoclonal antibody specific for cytomegalovirus detected antigens in phagocytosed inclusions on one occasion. These results may suggest that cytomegalovirus antigens are a hitherto unidentified component of serum complexes in patients with rheumatoid arthritis and may contribute to the pathogenesis of the vasculitic complications of rheumatoid arthritis by participating in immune complex formation.
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5/38. Malignant rhabdoid tumor arising from soft parts of the right thigh with unusual neurologic manifestation: report of a case.

    A case of malignant rhabdoid tumor (MRT) arising from the soft tissue of the right thigh in a 49-year-old Chinese female with peripheral neuropathy is reported. The tumor, exhibiting the salient features of MRT, was composed of sheets and nests of polygonal cells with prominent nucleoli and characteristic paranuclear inclusion-like hyaline globules under light microscopy which corresponded to aggregates of intermediate filaments under electron microscopy. The results of immunohistochemical studies of the tumor cells were also characteristic: cytokeratin ( ), vimentin ( ), epithelial membrane antigen (EMA) ( ), desmin (-), myoglobin (-), leukocyte common antigen (LCA) (-), kappa (-), lambda (-), IgG (-) and IgA (-). Serologic study revealed an M-component of IgA. The clinical evolution of the patient was highly aggressive and inevitably lethal. An adult malignant rhabdoid tumor is unusual, and its association with peripheral neuropathy and the coexistence of an M-component of IgA in this case appears to be unique. In this report, the differential diagnosis of histopathologic features, the association of peripheral neuropathy and the coexistence of an M-component of IgA are discussed.
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6/38. Acute neuropathies after peripheral blood stem cell and bone marrow transplantation.

    Neuromuscular complications are not uncommon after bone marrow and stem cell transplantation, especially in patients with allogeneic transplantations and graft-versus-host disease. The pathogenesis of these complications remains unclear, but the changes in immune modulation that occur after transplantation are likely to play a key role. We describe 4 patients who developed brachial plexopathy (3 cases) or multiple lumbosacral radiculopathies (1 case) between 5 days and 4 months after autologous peripheral blood stem cell (3 cases) or allogeneic bone marrow transplantation without evidence of graft-versus-host disease (1 case). Infectious, tumor-related, toxic, and metabolic causes were excluded in all cases. Recovery was limited in two cases and nearly complete in the other two patients. Brachial plexopathies and polyradiculopathies are potential complications of peripheral blood stem cell and bone marrow transplantation. It is possible that these disorders may be the result of autoimmune phenomena directed against specific nerve antigens.
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7/38. radial nerve palsy owing to localized hypertrophic neuropathy (intraneural perineurioma) in early childhood.

    Localized hypertrophic neuropathy, also termed intraneural perineurioma, is a rare disorder of unknown etiology that produces a slowly progressive painless focal lesion of a peripheral nerve. It is characterized histologically by concentric whorls ("onion bulbs") of epithelial membrane antigen-reactive, S-100 protein-negative perineurial cells surrounding nerve fibers. We report a radial nerve palsy in a child aged 2 years in whom the diagnosis of localized hypertrophic neuropathy was made by biopsy. Resection of the affected nerve segment and sural nerve grafting produced no useful recovery after 3 years, probably because of the long duration of denervation. When this mononeuropathy presents in early childhood, uncertainty over the time of onset can lead to difficulty in distinguishing this potentially treatable lesion from congenital and other causes of nerve palsy.
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8/38. cytomegalovirus-induced small-bowel hemorrhage in a patient with nonsystemic vasculitic neuropathy.

    A 73-year-old man who was being treated with corticosteroids for nonsystemic vasculitic neuropathy developed small-bowel hemorrhage after ileostomy for ileus. Immunohistochemical staining for cytomegalovirus (CMV) antigen in the ulcer in the resected ileum was positive; thus, cytomegalovirus infection of the small intestine caused his gastrointestinal manifestations. cytomegalovirus infection should be considered in the differential diagnosis of gastrointestinal diseases in patients with collagen vascular diseases receiving immunosuppressive agents.
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9/38. Cryptococcal infection presenting with lumbosacral polyradiculopathy: report of a case.

    Cryptococcal infection presenting primarily as lumbosacral polyradiculopathy is rare. We report on a 57-year-old man with lumbosacral polyradiculopathy, and in which a culture from the cerebrospinal fluid grew cryptococcus neoformans. A serum cryptococcal antigen study showed a positive reaction. biopsy specimens from the spinal nerve rootlet showed evidence of arachnoiditis and direct involvement of the nerve root by cryptococcus neoformans. It is important to remember that localized lumbosacral polyradiculopathy can be the sole initial manifestation of cryptococcosis.
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10/38. Toxic neuropathy after adenine arabinoside treatment in chronic HBsAg-positive liver disease.

    A 62-year-old woman with hepatitis-B-surface-antigen-positive hepatic cirrhosis presented with weakness and paresthesias over the distal part of the limbs in the course of adenine arabinoside 5'-monophosphate (ARA-AMP) treatment, and recovered spontaneously after several weeks of drug withdrawal. Electrophysiological and histological studies demonstrated axonal neuropathy. Although the patient received a relatively low total dose (120 mg/kg), her age and advanced liver disease may have played a role in the ARA-AMP neurotoxicity.
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