1/38. opsoclonus-myoclonus syndrome in a child with neuroborreliosis.Opsoclonus-myoclonus is a rare neurological syndrome affecting children and adults. In children it occurs as a parainfectious process or a paraneoplastic syndrome in association with neuroblastoma. Here we report it presenting as an unusual neurological manifestation of Lyme borreliosis. To our knowledge, this is the first report which describes recovery from this syndrome in a child.- - - - - - - - - - ranking = 1keywords = paraneoplastic (Clic here for more details about this article) |
2/38. Olivopontocerebellar atrophy: paraneoplastic syndrome of brain tumour?We describe a patient who, three years after the onset of an olivopontocerebellar atrophy, developed a right cerebral tumour. The cerebellar symptomatology also included, as in other cerebellar patients previously described, a peripheral dysgraphia. Because this deficit of writing is generally reported in patients with right cerebral lesion, the authors hypothesized that functional alterations of supratentorial structures preceding the tumour by years may be able to damage the neural substrates connecting cerebral and cerebellar structures and to produce cerebellar atrophy.- - - - - - - - - - ranking = 4keywords = paraneoplastic (Clic here for more details about this article) |
3/38. association of herpes simplex virus encephalitis and paraneoplastic encephalitis - a clinico-pathological study.A 57 year-old woman developed acute limbic encephalitis and brainstem dysfunction. Anti-HU antibodies were repeatedly detected in serum and CSF. Postmortem examination showed necrotic and hemorrhagic lesions in the temporal lobes characteristic of herpes simplex virus encephalitis, which was confirmed by immunocytochemistry, and Purkinje cell loss with proliferation of Bergman glia and myelin loss in the external aspect of the dentate nuclei characteristic of paraneoplastic encephalitis. PCR-assay performed on temporal tissue extracts was positive for HSV-1. There was no identifiable neoplasm. This unusual association raises the possibility of a link between the two diseases.- - - - - - - - - - ranking = 5keywords = paraneoplastic (Clic here for more details about this article) |
4/38. Morphometric evaluation of paraneoplastic neuropathies associated with carcinomas, lymphomas, and dysproteinemias.Paraneoplastic peripheral neuropathies are caused by indirect effects of carcinomas, mainly small cell bronchogenic carcinomas, lymphoproliferative disorders (lymphomas, myelomas, polycythemia vera), and dysproteinemias (benign monoclonal paraproteinemia, Waldenstrom's macroglobulinemia) including cryoglobulinemias. Those associated with carcinomas are usually considered as severe, those associated with benign gammopathies (monoclonal gammopathies of unknown significance, MGUS) as mild, and those with cryoglobulinemias as of variable severity. In a larger series of 104 autopsy and biopsy cases, we noted a wide range of severity concerning various morphometric parameters of peripheral nerve fibers by evaluating sural nerves. There were no apparent morphometric differences between the groups of disorders. The most valuable parameter of optic-electronic evaluation and comparison turned out to be the myelin area expressed as a percentage of the endoneurial area because this measure comprises the relative number, size, and myelin thickness of the myelinated nerve fibers. In the 104 cases of the three disease groups, most of the cases (38 cases; 36.5%) showed a moderate reduction of the myelin sheath area per endoneurial area of sural nerves. This was followed by 34 cases (32.7%) with severe and very severe reduction. Twenty-nine cases (27.9%) presented with mild reduction. It is concluded that the severity of the neuropathy depends largely on the stage of the disease and the time of progression rather than on the type of the underlying disorder.- - - - - - - - - - ranking = 4keywords = paraneoplastic (Clic here for more details about this article) |
5/38. Paraneoplastic syndrome in the course of lung adenocarcinoma: morphological picture and immunohistochemical analysis of the inflammatory infiltrates and PECAM-1 expression.We examined sections of brain, spinal cord, spinal roots, and peripheral nerves from a patient with paraneoplastic syndrome in the course of lung adenocarcinoma. Morphological examination showed marked loss of myelin fibers in peripheral nerves, severe brain tissue edema, and paraneoplastic degeneration involving cerebrum and cerebellum with inflammatory components. Inflammatory infiltrates examined immunohistochemically using antibodies against antigens CD 3, CD 4, CD 8, and CD 20 turned out to be composed of cytotoxic T lymphocytes. The expression of platelet-endothelial cell adhesion molecule-1 (PECAM-1) in blood vessels was increased in comparison with control material, which may facilitate transendothelial lymphocyte migration triggering a cascade of biochemical and morphological reactions observed in paraneoplastic syndrome.- - - - - - - - - - ranking = 3keywords = paraneoplastic (Clic here for more details about this article) |
6/38. Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody.A woman developed brain stem encephalopathy in association with serum anti-Ma2 antibodies and left upper lobe lung mass. T2 weighted MRI of the brain showed abnormalities involving the pons, left middle and superior cerebellar peduncles, and bilateral basal ganglia. Immunohistochemical analysis for serum antineuronal antibodies was confounded by the presence of a non-neuronal specific antinuclear antibody. Immunoblot studies showed the presence of anti-Ma2 antibodies. A premortem tissue diagnosis of the lung mass could not be established despite two CT guided needle biopsies, and the patient died as a result of rapid neurological deterioration. The necropsy showed that the lung lesion was an adenocarcinoma which expressed Ma2 immunoreactive protein. Neuropathological findings included prominent perivascular inflammatory infiltrates, glial nodules, and neuronophagia involving the brain stem, basal ganglia, hippocampus and the dentate nucleus of the cerebellum. Ma2 is an autoantigen previously identified in patients with germ cell tumours of the testis and paraneoplastic brain stem and limbic encephalitis. Our patient's clinical and immunopathological findings indicate that this disorder can affect women with lung adenocarcinoma, and that the encephalitic changes predominate in those regions of the brain known to express high concentrations of Ma proteins.- - - - - - - - - - ranking = 1keywords = paraneoplastic (Clic here for more details about this article) |
7/38. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: histopathologic features-a report from the Children's Cancer Group.BACKGROUND: Opsoclonus-myoclonus-ataxia (OMA) is a paraneoplastic syndrome that occurs in about 2-3% of all cases of neuroblastoma. The histopathologic characteristics of neuroblastoma tumors associated with this syndrome were evaluated in a series of cases and controls. PROCEDURE: pathology slides from a total of 54 neuroblastoma tumors were reviewed blindly. They included 13 tumors associated with opsoclonus-myoclonus and 41 age- and stage-matched controls. All tumors were classified into either the favorable (FH) or unfavorable histology (UH) group according to the International neuroblastoma pathology classification (the Shimada system). Grade of lymphocytic infiltration was evaluated and presence or absence of lymphoid follicles was recorded in the individual tumor tissues. RESULTS: Twelve of 13 cases with opsoclonus-myoclonus were in the FH group. Twelve of 13 cases had diffuse (found in every section prepared from the multiple portions of the primary tumor) and extensive (occupying more than 50% of a single of multiple microscopic fields with x 100 magnification) lymphocytic infiltration with lymphoid follicles. Of the 41 control cases (27 FH and 14 UH tumors), 18 had focal areas of lymphocytic infiltration and six showed lymphoid follicles, but none had diffuse or extensive infiltration in their primary tumors. CONCLUSIONS: Diffuse and extensive lymphocytic infiltration with lymphoid follicles is a characteristic histologic feature of neuroblastic tumors with opsoclonus-myoclonus. This observation suggests an immune-mediated mechanism for this rare paraneoplastic syndrome.- - - - - - - - - - ranking = 2keywords = paraneoplastic (Clic here for more details about this article) |
8/38. Hodgkin's disease preceded by unique neurological symptoms.This is the first case report of Hodgkin's disease (HD) which showed both remission and exacerbation of neurological signs before a confirmed diagnosis of HD. The episodes occurred three times and multiple lesions were involved. Immunoabsorption plasmapheresis and double filtration plasmapheresis were effective for the first episode, whereas, corticosteroids partly improved the second and third episodes. fever and lymph node swelling were apparent afterward and she was diagnosed as having HD from a supraclavicular lymph node biopsy. The remaining neurologic deficits responded to chemotherapy and radiotherapy. The neurological symptoms were considered as a paraneoplastic syndrome of HD.- - - - - - - - - - ranking = 1keywords = paraneoplastic (Clic here for more details about this article) |
9/38. ANNA-3 anti-neuronal nuclear antibody: marker of lung cancer-related autoimmunity.Two anti-neuronal nuclear antibodies (ANNA-1 and ANNA-2) are markers of paraneoplastic neurological autoimmunity related to small-cell carcinoma. ANNA-2 is also related to breast carcinoma. Here we define a third IgG specificity (ANNA-3), identified in 11 patients (10 adults) by immunofluorescence screening of sera from approximately 68,000 patients with suspected paraneoplastic neurological syndromes. ANNA-3 binds prominently to nuclei of cerebellar Purkinje neurons, not to cytoplasm, granular neurons, or enteric neurons, but distinctively to renal glomerular podocytes. Western blots revealed an approximately 170 kDa antigen, in cerebellum and small-cell carcinoma. IgG eluted from this protein reproduced Purkinje and podocyte nuclear staining. ANNA-2 in 8 of 32 cases bound to podocyte nuclei but not to the 170 kDa protein. Healthy subjects and control neurological and cancer patients lack ANNA-3. Neurological accompaniments, subacute and usually multifocal, included sensory/sensorimotor neuropathies, cerebellar ataxia, myelopathy, brain stem and limbic encephalopathy. All of 9 adults followed had an intrathoracic neoplasm, seven biopsied within 7 months (five small-cell lung carcinomas and two adenocarcinomas, one lung, one esophagus) and two imaged, one early, the other 3 years later. Thus, immunohistochemical and Western blot criteria can now identify six IgG markers of neurological autoimmunity related to small-cell carcinoma, their frequency being ANNA-1 > collapsin response-mediator protein-5 > amphiphysin > Purkinje cell cytoplasmic antibody-2 = ANNA-2 = ANNA-3.- - - - - - - - - - ranking = 2keywords = paraneoplastic (Clic here for more details about this article) |
10/38. Phenotypic and neuropathologic heterogeneity of anti-Hu antibody-related paraneoplastic syndrome presenting with progressive dysautonomia: report of two cases.The anti-Hu antibody (HuAb) is directed against rna-associated neuronal proteins and is known to cause paraneoplastic encephalomyelitis/sensory neuronopathy syndrome mostly when associated with small cell lung cancer (SCLC). Paraneoplastic encephalomyelitis/sensory neuronopathy syndrome with concurrent autonomic neuropathy has been reported to occur in paraneoplastic syndromes, although its occurrence concomitant with acute pandysautonomia is less frequent. The authors describe the clinical, neuropathologic, and serologic features of two cases with an anti-Hu-related paraneoplastic syndrome presenting with progressive autonomic neuropathy. Both patients showed features of dysautonomia, including postural dizziness, abdominal pain, and diarrhea, and symptoms of sensory neuropathy. Investigations disclosed severe sensory and autonomic neuropathy and positive HuAb titers. The disease of patient 1 had a very rapid progression, and the patient died of cardiac arrest within 2 months of the onset of symptoms. The autopsy revealed SCLC. In contrast, the disease of patient 2 had a less aggressive course. An extensive tumor search disclosed SCLC only 28 months after onset of symptoms, and the patient died 1 month later of cardiorespiratory arrest. Autopsies in both cases showed inflammation involving the intermediolateral columns and the dorsal root ganglia. These two cases illustrate the association of early dysautonomia with HuAb-related paraneoplastic syndrome and the variations of clinical, neuropathologic, and serologic findings in these types of cases.- - - - - - - - - - ranking = 8keywords = paraneoplastic (Clic here for more details about this article) |
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