1/7. Anti-Ri-associated paraneoplastic cerebellar degeneration without opsoclonus in a patient with a neuroendocrine carcinoma of the stomach.We report a case of a 63-year-old man suffering from anti-Ri-associated paraneoplastic cerebellar degeneration (PCD) with gastric cancer. The neurologic presentation was limited to severe cerebellar ataxia without opsoclonus. The gastric cancer was composed of both poorly differentiated adenocarcinoma and neuro-endocrine carcinoma. The patient's serum reacted with recombinant Ri antigen and the neuroendocrine tumor component. It is thus considered that PCD without opsoclonus in the present case was related to the gastric neuroendocrine tumor and anti-Ri antibody.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/7. Extramedullary plasmacytoma of the orbit.PURPOSE: To report a case of extramedullary plasmacytoma of the orbit. methods: A 71-year-old patient presented with diplopia eyelid fullness and limitation of ocular motility in the left eye. visual acuity was counting fingers, intraocular pressure 34 mmHg and fundus eye examination showed choroidal folds in the involved eye. RESULTS: CT scan showed a mass filling the superior and external left orbit without bone destruction. A biopsy was performed revealing that the tumour was composed of plasmacytoid cells positive with immunohistochemical stains for Kappa light chains and epithelial membrane antigen. Systemic work up was negative. The diagnosis of extramedullary orbital plasmacytoma was made. The patient was treated with external beam radiotherapy (40 Gy) and has remained disease free for four years (49 months). CONCLUSION: Extramedullary plasmacytomas of the orbit are extremely rare tumours. Accurate and early diagnosis is essential for the therapeutic approach. Extensive medical work up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving the orbit is needed when the diagnosis of orbital extramedullary plasmacytoma is suspected because treatment and prognosis are very different.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/7. Anti-Ri-associated paraneoplastic opsoclonus-ataxia syndrome in a man with transitional cell carcinoma.BACKGROUND: There are several case reports describing paraneoplastic syndromes in patients with various forms of bladder carcinoma. Current immunologic analyses have enabled the identification of the antineuronal autoantibodies associated with specific syndromes. methods: A patient with a history of bladder carcinoma presented with opsoclonus and myoclonus. RESULTS: Workup confirmed the presence of anti-Ri antibodies in the patient's serum and cerebrospinal fluid. The target Ri antigen was found to be expressed by the tumor. CONCLUSIONS: To the authors' knowledge, there are few reports in the literature describing the long-term clinical follow-up and postmortem evaluation in a patient with this form of paraneoplastic syndrome. More important, the authors believe the current study represents the first time that the presence of anti-Ri antibodies has been noted in a paraneoplastic syndrome associated with transitional cell carcinoma of the bladder.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/7. Opsoclonus in three dimensions: oculographic, neuropathologic and modelling correlates.Opsoclonus is a dyskinesia consisting of involuntary, arrhythmic, chaotic, multidirectional saccades, without intersaccadic intervals. We used a magnetic scleral search coil technique to study opsoclonus in two patients with paraneoplastic complications of lung carcinoma. eye movement recordings provided evidence that opsoclonus is a three-dimensional oscillation, consisting of torsional, horizontal, and vertical components. Torsional nystagmus was also present in one patient. Antineuronal antibody study revealed the presence of anti-Ta (Ma2 onco-neuronal antigen) antibodies in one patient, which had previously been associated only with paraneoplastic limbic encephalitis and brainstem dysfunction, but not opsoclonus, and only in patients with testicular or breast cancer. Neuropathologic examination revealed mild paraneoplastic encephalitis. Normal neurons identified in the nucleus raphe interpositus (rip) do not support postulated dysfunction of omnipause cells in the pathogenesis of opsoclonus. computer simulation of a model of the saccadic system indicated that disinhibition of the oculomotor region of the fastigial nucleus (FOR) in the cerebellum can generate opsoclonus. Histopathological examination revealed inflammation and gliosis in the fastigial nucleus. This morphological finding is consistent with, but not necessary to confirm, damage to afferent projections to the FOR, as determined by the model. Malfunction of purkinje cells in the dorsal vermis, which inhibit the FOR, may cause opsoclonus by disinhibiting it.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/7. Paraneoplastic opsoclonus-myoclonus associated with anti-Hu antibody.We report a 74-year-old woman with opsoclonus, myoclonus, ataxia, and encephalopathy who had small-cell lung cancer and high titers of anti-Hu antibody in her serum. At autopsy, there were perivascular inflammatory infiltrates in the brainstem, putamen, and meninges overlying the orbital frontal cortex. Immunohistochemical studies showed the expression of the Hu antigens by the tumor and the presence of deposits of anti-Hu IgG in the patient's cortex, brainstem, and cerebellum, suggesting that anti-Hu immune response was related to the patient's clinical syndrome. This case of paraneoplastic opsoclonus, myoclonus, ataxia, and encephalopathy expands the spectrum of neurologic dysfunction associated with the anti-Hu antibody.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/7. serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues.OBJECTIVE: Opsoclonus-myoclonus (OM) is a rare neurologic syndrome affecting children and adults. In children it occurs as a parainfectious process or a paraneoplastic syndrome in association with neuroblastoma. Evidence for an immune mechanism includes the presence of serum autoantibodies to several neural antigens and improvement of symptoms with immunosuppressive therapy. We studied the neural antigenic targets of serum IgM and IgG autoantibodies from nine children with OM. DESIGN: We studied sera from nine children with OM, three with associated neuroblastoma and six with a prodromal viral illness. Control subjects (n = 77) included four children with neuroblastoma but not OM, 32 children with other neurologic disorders, and 41 with nonneurologic illnesses. We studied the neural antigenic targets of serum IgM and IgG autoantibodies by the following methods: (1) immunostaining of human cerebellar sections and peripheral nerve, and (2) Western blot analysis with human brain fractions including white matter, gray matter, and cerebellar purkinje cells and nuclei. RESULTS: Sera from all nine children with OM had IgM and IgG binding to the cytoplasm of cerebellar purkinje cells and to some axons in white matter. In peripheral nerve, IgM and IgG from all nine OM sera bound to large and small axons. Western blot analysis showed a distinctive pattern of binding to several neural proteins, including a 210 kd antigen identified as the high molecular weight subunit of neurofilament. No control serum showed a similar pattern of reactivity. CONCLUSION: opsoclonus-myoclonus syndrome in childhood is associated with a distinctive pattern of serum IgM and IgG binding to neural tissues and antigens.- - - - - - - - - - ranking = 9keywords = antigen (Clic here for more details about this article) |
7/7. Antineuronal antibody in a patient with neuroblastoma and opsoclonus-myoclonus-ataxia: a case report.We describe a case of a patient with neuroblastoma and opsoclonus- myoclonus ataxia displaying serum and CSF anti-Hu antibodies that were able to recognize antigens of the patient's own tumor.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |