11/38. The role of [18F]fluoro-2-deoxyglucose-PET scanning in the diagnosis of paraneoplastic neurological disorders.The detection of an occult tumour in a patient with a suspected paraneoplastic neurological disorder (PND) may be difficult because of the limitations of conventional imaging techniques. [(18)F]fluoro-2-deoxyglucose-PET (FDG-PET) can visualize a small tumour anywhere within the body. We retrospectively reviewed the case notes of 43 unselected patients with suspected PND referred for FDG-PET scanning to determine how useful this technique was when conventional imaging was negative. All patients had undergone standard radiological investigations and bronchoscopy (where appropriate) prior to PET scanning. There were discrete areas of hypermetabolism suggestive of malignancy (positive) in 16 patients (37%). A tissue diagnosis of cancer was subsequently made in seven patients (two at post-mortem), further radiological studies were suggestive of cancer in one patient, one patient subsequently presented with a metastatic deposit which was biopsied, and four patients died shortly afterwards without a post-mortem. In three patients, subsequent investigations were negative for cancer. serum anti-neuronal antibodies were present in 43% and CSF oligoclonal bands were present in 46% of patients with positive PET scans compared with 16 and 26%, respectively, in PET-negative patients, but this was not significant. Only one patient with a negative scan has been diagnosed subsequently as having malignancy on prolonged follow-up. These findings confirm that FDG-PET scanning is a useful technique in the detection of small tumours in patients with suspected PND. False positives and false negatives do occur, but at a sufficiently low frequency to justify the clinical usefulness of this technique.- - - - - - - - - - ranking = 1keywords = paraneoplastic (Clic here for more details about this article) |
12/38. 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 = 1keywords = paraneoplastic (Clic here for more details about this article) |
13/38. Quantification of circulating anti-Hu antibody in serial samples from patients with paraneoplastic neurological syndromes: possible correlation of antibody concentration and course of neurological symptoms.Serial samples, spanning an observation period of 4 to 10 years, from five patients with anti-Hu associated paraneoplastic neurological syndromes (PNS) were investigated with an enzyme linked immunosorbent assay (ELISA) employing recombinant HuD protein as antigen. In one patient the anti-Hu antibody concentration converted from negative to highly positive levels after the onset of neurological symptoms. These findings argue in favour of the idea that an autoimmune process, which is generated at the beginning of the neurological disease, causes the anti-Hu associated PNS. serum samples obtained shortly after the beginning of the PNS were available from two other patients. The anti-Hu antibody levels in these latter patients increased from modest to highly positive anti-Hu antibody in follow up samples. In two patients a clear decrease of the anti-HuD antibody concentration together with an improvement of paraneoplastic neurological symptoms after successful tumour treatment were seen. overall these data suggest that there might be a correlation between the anti-HuD antibody level and the clinical course of paraneoplastic neurological symptoms which supports further the hypothesis that an autoimmune process is responsible for the anti-Hu associated paraneoplastic neurological symptoms.- - - - - - - - - - ranking = 1.6keywords = paraneoplastic (Clic here for more details about this article) |
14/38. Tumor progression and serum anti-HuD antibody concentration in patients with paraneoplastic neurological syndromes.The aim of this study was to investigate whether there are clues for a correlation between tumor progression and serum anti-Hu antibody concentration in patients with anti-Hu-associated paraneoplastic neurological syndromes (PNS). 19 patients with anti-Hu-associated PNS were assigned to three groups according to the course of tumor progression. Group 1 corresponds to patients with rapid tumor progression [n = 5; mean survival in months/standard deviation (SD); 24/10]; in group 2 patients with a favorable tumor prognosis were included (n = 7; mean/SD 79/25, 6 patients still alive); group 3 consisted of patients in whom tumor progression could not be assessed (n = 7; mean/SD 23/20). The anti-Hu antibody concentrations in sera were measured in a recombinant HuD-ELISA. In sera from patients of group 1 the anti-Hu antibody concentration was modest (mean OD 0.56, SD 0.08) whereas a significantly higher anti-Hu antibody concentration was detected in sera from patients with a favorable tumor prognosis (group 2, mean OD 1.86, SD 0.34). These results hint at a negative correlation between tumor progression and the anti-Hu antibody concentration in sera from patients with PNS. These findings confirm and extend previous reports of more indolent tumor growth in patients with an anti-Hu immune response.- - - - - - - - - - ranking = 1keywords = paraneoplastic (Clic here for more details about this article) |
15/38. Diffuse large B-cell lymphoma with infiltration-associated peripheral neuropathy and paraneoplastic myopathy with a prolonged course over seven years.Rarely lymphoproliferative disorders (LPD) may be associated with peripheral neuropathy, based on different mechanisms of pathogenesis. Here, we report a case of a primary extranodal diffuse large B-cell lymphoma which showed a prolonged course with several recurrences manifesting as neuropathies, and long term remissions achieved by chemotherapy and involved field radiation. Repeated pathohistologic evaluations showed spread of lymphoma growth predominantly along nerve structures, suggesting neurotropism of the lymphoma cells.- - - - - - - - - - ranking = 0.8keywords = paraneoplastic (Clic here for more details about this article) |
16/38. Amphiphysin paraneoplastic syndrome: a delayed diagnosis of breast carcinoma.We present a rare case of the paraneoplastic disorder, amphiphysin autoimmunity, which is associated with breast cancer. Amphiphysin is a presynaptic protein to which the body can form antibodies usually in cases of carcinoma, particularly of the breast and lung. The diagnosis of malignancy was delayed because of the complexity of the presenting neurologic symptoms related to the autoimmunity. The confusing picture and the rarity of the entity can result in delays in routine investigations, such as screening mammography. We advocate routine screening and physical examination in all patients, even in the presence of perplexing clinical symptoms.- - - - - - - - - - ranking = 1keywords = paraneoplastic (Clic here for more details about this article) |
17/38. 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 = 0.2keywords = paraneoplastic (Clic here for more details about this article) |
18/38. 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 = 1keywords = paraneoplastic (Clic here for more details about this article) |
19/38. Complicated paraneoplastic neurological syndromes: a report of two patients with small cell or non-small cell lung cancer.Paraneoplastic neurological syndromes are frequently associated in patients with small cell lung cancer (SCLC) and antineuronal antibodies are involved in the autoimmune mechanism. Multiple syndromes are sometimes complicated in a single patient with SCLC. However, little is known about non-SCLC-associated neurological manifestations. We report two patients with complicated paraneoplastic neurological syndromes. Patient 1 showed paraneoplastic limbic encephalitis (PLE), paraneoplastic sensory neuropathy (PSN) and lambert-eaton myasthenic syndrome (LEMS) associated with SCLC. Patient 2 developed opsoclonus-ataxia and probable PLE associated with non-SCLC. Analysis of various antineuronal antibodies revealed that anti-Hu and P/Q-type voltage-gated calcium channel (VGCC) antibodies were positive in Patient 1 but any antibodies were not in Patient 2. Brain MRI demonstrated high intensity signals in temporal lobes particularly on fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted images. These findings suggest that complicated paraneoplastic neurological syndromes occur in non-SCLC as well as SCLC and that unidentified antineuronal autoantibodies may underlie the pathophysiology.- - - - - - - - - - ranking = 1.6keywords = paraneoplastic (Clic here for more details about this article) |
20/38. Paraneoplastic opsoclonus-myoclonus syndrome associated with malignant fibrous histiocytoma: neuropathological findings.paraneoplastic syndromes related to soft tissue tumors are very uncommon and an association of paraneoplastic diseases with a malignant fibrous histiocytoma (MFH) has not been reported so far. Opsoclonus-myoclonus is a rare paraneoplastic nervous system syndrome, that was well documented in adult patients with neoplasms particularly of the lung and breast. A 77-year-old woman developed typical opsoclonus-myoclonus syndrome and rapidly progressed to coma. The opsoclonus and generalized myoclonus continued until the patient's death seven months later. An autopsy examination identified a pleomorphic MFH in the retroperitoneum without a metastatic spread. Microscopic examination of the cerebellum revealed an atrophy of the granular layer along with a marked patchy loss of purkinje cells, and a loss of neurons in the cerebellar nuclei accompanied by distinct peridental demyelination and astrogliosis. A moderate loss of neurons and neuronal chromatolysis were observed also in the inferior olivary nuclei. However, the omnipause neurons of the nucleus raphe interpositus, that are supposed to be responsible for opsoclonus generation under pathological conditions, as well as ocular brain stem nuclei were all intact. We failed to prove the presence of known antineuronal anti-Yo, anti-Hu or anti-Ri autoantibodies in both serum and cerebrospinal fluid (CSF) of the patient. However, the confirmation of the intrathecal IgG synthesis by oligoclonal bands in CSF and the immunohistochemical detection of IgG deposits on membranes of purkinje cells, neurons of cerebellar granular layer and in cerebellar nuclei in our case support the presumed autoimmune nature of the disease.- - - - - - - - - - ranking = 0.4keywords = paraneoplastic (Clic here for more details about this article) |
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