1/20. 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 = 1keywords = brain (Clic here for more details about this article) |
2/20. 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 = 0.25keywords = brain (Clic here for more details about this article) |
3/20. 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 = 0.5keywords = brain (Clic here for more details about this article) |
4/20. 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 = 2.25keywords = brain (Clic here for more details about this article) |
5/20. 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 = 0.25keywords = brain (Clic here for more details about this article) |
6/20. opsoclonus-myoclonus syndrome as a paraneoplastic manifestation of renal cell carcinoma. a case report and review of the literature.Opsoclonus is a rare but distinctive disorder of ocular motility, characterized by irregular, continual and conjugated chaotic saccades of the eyes. It is increased with eye closure and fixation, and it persists during sleep. Opsoclonus appears more commonly in children and in half of such cases in association with neuroblastoma. In adults the most frequent causes are idiopathic (50%) and paraneoplastic (20%). Among the second group, different types of tumors involving a wide variety of organs have been reported. Opsoclonus when accompanied by other symptoms of central nervous system involvement (head, appendicular myoclonus and truncal ataxia) constitutes a clinical picture, known as opsoclonus-myoclonus syndrome. We report the case of a young patient affected by renal cell carcinoma associated with opsoclonus-myoclonus syndrome which did not respond to medical therapy but dramatically improved after cancer ablation.- - - - - - - - - - ranking = 0.89290717987875keywords = nervous system, central nervous system (Clic here for more details about this article) |
7/20. Metaiodobenzylguanidine total-body scintigraphy required for revealing occult neuroblastoma in opsoclonus-myoclonus syndrome.A girl aged 13 months presented with clinical features of subacute progressive ataxia leading to abasia, astasia, loss of unsupported sitting and apraxia. In addition, an opsoclonus, myoclonia and introvert behaviour developed. MRI of the brain, EEG, extensive tests of blood, urine and CSF showed no abnormalities. Based on clinical symptoms only, the diagnosis of opsoclonus-myoclonus syndrome (OMS) could be made. Under the suspicion of a neuroblastoma, further investigations were performed: a lateral and antero-posterior X-ray examination of the chest showed no tumour; neither did ultrasound of the abdomen. Concentrations of catecholamines and their metabolites in 24 h urine were normal and none of five tested anti-neuronal antibodies were found. However, a total-body scintigraphy with [I(123)] metaiodobenzylguanidine (MIBG) revealed a paravertebral hot spot on the left side compatible with a neural crest tumour. A MRI scan of the abdomen confirmed the supraphrenic lesion. [I(123)]MIBG uptake was sufficient for [I(131)]MIBG therapy. The response of the tumour to this therapy was favourable. The neurological symptoms of the patient slightly improved under steroid treatment. CONCLUSION: opsoclonus-myoclonus syndrome is a serious disease in infants, sometimes associated with occult neuroblastoma for which a full oncological work-up, including metaiodobenzylguanidine total-body scintigraphy is required.- - - - - - - - - - ranking = 0.25keywords = brain (Clic here for more details about this article) |
8/20. Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma.A 65-year-old male patient developed truncal ataxia, opsoclonus and myoclonus. In the serum anti-Ri antibodies were found, which led to the detection of a small adenocarcinoma of the breast. Other prominent clinical features were an excessive startle response and behavioral disorders, such as anxiety and impatience. These features suggest an immune response against both Nova-1 and Nova-2 antigens throughout the central nervous system.- - - - - - - - - - ranking = 0.89290717987875keywords = nervous system, central nervous system (Clic here for more details about this article) |
9/20. Paraneoplastic encephalitis associated with cystic teratoma is detected by fluorodeoxyglucose positron emission tomography with negative magnetic resonance image findings.The role of fluorodeoxyglucose (FDG) positron emission tomography (PET) as a functional imaging technique used in the evaluation of a variety of malignancies has been well known. Paraneoplastic encephalitis is a rare central nervous complication, which has been reported in some tumors. Traditionally, magnetic resonance imaging of the brain is performed to aid in its diagnosis. The authors report a case of paraneoplastic encephalitis, associated with cystic teratoma, which had positive FDG-PET findings but appeared normal on magnetic resonance imaging.- - - - - - - - - - ranking = 0.25keywords = brain (Clic here for more details about this article) |
10/20. Anti-Hu antibodies may indicate a positive response to chemotherapy in paraneoplastic syndrome secondary to small cell lung cancer.Paraneoplastic encephalomyelitis (PEM) is a form of neurological dysfunction caused by the remote effect of small cell lung cancer (SCLC) on the brain. In certain cases it is associated with the presence of Anti-Hu antibodies. Up to 65% of patients with SCLC and PEM, who have Anti-Hu antibodies present, die of neurological complications rather than tumour progression. Chemotherapy is not believed to be beneficial. We describe a 58-year-old lady who was bed-bound and significantly disabled from paraneoplastic cerebellar degeneration. Her serology confirmed the presence of Anti-Hu antibodies and SCLC was confirmed at bronchoscopy. Following six courses of chemotherapy her neurological symptoms have remarkably improved, both subjectively and objectively, such that she is now independently mobile with a walking frame. Her tumour is still evident on bronchoscopy. We suggest that people with SCLC who are significantly disabled by neurological symptoms, should be tested for serum anti-neuronal antibodies and if Anti-Hu antibody positive, anti-neoplastic treatment should be considered despite poor performance status.- - - - - - - - - - ranking = 0.25keywords = brain (Clic here for more details about this article) |
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