1/51. Paraneoplastic limbic encephalitis associated with small cell carcinoma of the prostate.A 76-year-old man with primary small cell carcinoma of the prostate died after a subacute illness marked by memory loss and truncal ataxia Post-mortem examination of the central nervous system was consistent with limbic encephalitis and cerebellar degeneration. Although limbic encephalitis is a known complication of small cell carcinoma of the lung, this seems to be the first reported case of limbic encephalitis associated with small cell carcinoma of the prostate. Implications with respect to diagnosis and therapy are discussed.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/51. Primary angiitis of the central nervous system as a first presentation in Hodgkin's disease: a case report and review of the literature.OBJECTIVE AND IMPORTANCE: Granulomatous angiitis of the central nervous system is a rare cause of neurological deterioration. It is often diagnosed posthumously, and a high index of suspicion is necessary to make the correct diagnosis on a timely basis. CLINICAL PRESENTATION: A 27-year-old woman presented to the emergency room with complaints of worsening headache, nausea, and vomiting for 10 days, which were preceded by swelling of her tongue. At the examination, she had mild ocular tenderness, but no cranial nerve abnormalities. Radiographic examination revealed a right temporal lobe area with edema, and mild contrast enhancement was noted on computed tomography and magnetic resonance imaging. A similar but smaller region was present in the left frontal lobe. INTERVENTION: Stereotactic biopsy of the left temporal lobe revealed granulomatous angiitis. Further workup revealed Hodgkin's disease in the mediastinum. dexamethasone as well as chemotherapy for Hodgkin's disease was initiated. The patient's symptoms resolved, and she returned to work with her disease in remission. CONCLUSION: Previous reports of central nervous system angiitis have shown an association with sjogren's syndrome, herpes zoster infection, human immunodeficiency virus, and Hodgkin's disease. A review of the literature revealed a total of 12 patients with central nervous system angiitis and Hodgkin's disease. As a group, these patients had very poor outcomes. However, of six patients who presented with central nervous system angiitis and concurrent Hodgkin's disease and who underwent aggressive treatment for both conditions, three had a full recovery, two had a partial recovery, and one died.- - - - - - - - - - ranking = 8keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/51. Ballistic-choreic movements as the presenting feature of renal cancer.BACKGROUND: The paraneoplastic syndromes can involve multiple areas of the central nervous system and result in a variety of neurological symptoms. To our knowledge, severe, rapidly progressive, and drug-resistant ballistic-choreic movements have not been previously described as the presenting feature of renal cell carcinoma. PATIENT AND methods: A previously healthy 55-year-old man developed limb ballismus and involuntary choreic movements of his face over several weeks. Extensive laboratory, diagnostic, and radiographic studies failed to reveal a cause, until an abnormality on a chest x-ray film prompted a search for a primary neoplasm and a final diagnosis of renal cell carcinoma. High doses of medications traditionally used to treat choreic disorders had no effect on the abnormal movements. A biopsy specimen of the basal ganglia showed focal encephalitic changes but no malignant neoplasm. CONCLUSIONS: Whereas prior cases of paraneoplastic syndromes with chorea have been reported in other forms of cancer, our case was significant because, to our knowledge, renal cell carcinoma has not been previously reported in association with this syndrome. Furthermore, the chorea was categorically resistant to pharmacological treatment, and the movement disorder was the initial and only focal neurological feature of the primary illness.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/51. Negative-antibody paraneoplastic syndrome complicating small cell carcinoma.The neurological paraneoplastic syndromes represent nonmetastatic complications of cancer and may affect several levels of the nervous system. They are thought to be immunologically-mediated. The syndrome predates the diagnosis of cancer by months to years in two thirds of cases. We report the case of a female patient presenting with a cerebellar syndrome and a sensory neuronopathy on a background of severe weight loss. We searched for occult malignancy and later diagnosed her to be suffering from a paraneoplastic syndrome secondary to small cell carcinoma of the lung. Paraneoplastic antibodies were negative. She was subsequently treated with chemotherapy.- - - - - - - - - - ranking = 0.46209326397225keywords = nervous system (Clic here for more details about this article) |
5/51. Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients.We studied 71 patients with "paraneoplastic" encephalomyelitis, sensory neuronopathy, or both associated with the presence of the anti-Hu antibody in their serum. Most (78%) had small-cell lung cancer. In 9 patients no tumor was detected. Fifty-two patients (73%) had signs and symptoms of multifocal involvement of the nervous system; in 28 (39%), 2 areas, and in 24 (34%), 3 or more areas were clinically affected. Sensory neuronopathy was present in 52 patients (74%), but in only 44 (62%) did it dominate the course of the disease. Other predominant findings were: motor neuron dysfunction (14 patients, 20%), limbic encephalopathy (14, 20%), cerebellar symptoms (11, 15%), brainstem encephalopathy (10, 14%), and autonomic nervous system dysfunction (7, 10%). The presence of the anti-Hu antibody prompted a search for the tumor in 60% of the patients; the tumor when found was usually small and remained localized until death, or was demonstrated only at autopsy. Treatment using steroids and plasmapheresis, immunosuppressants, or both, did not improve the paraneoplastic symptoms. Autonomic and respiratory failure, either of central origin or secondary to neuromuscular weakness, were the principal causes of death. patients with rapidly developing sensory neuropathy or symptoms of encephalomyelitis should be studied for the presence of the anti-Hu antibody; if the antibody is found, the possibility of small-cell lung cancer should be investigated. If a tumor is not found in the initial search, one may become evident in several months.- - - - - - - - - - ranking = 0.9241865279445keywords = nervous system (Clic here for more details about this article) |
6/51. rhabdomyolysis and paraneoplastic stiff-man syndrome with amphiphysin autoimmunity.Stiff-Man syndrome (SMS) is a rare disease of the central nervous system characterized by chronic muscle rigidity and autoimmunity directed against synaptic antigens. In a subset of patients, generally positive for antiamphiphysin autoantibodies, SMS has an autoimmune paraneoplastic origin. Amphiphysin isoforms are expressed at high levels in brain and skeletal muscle and often are overexpressed in breast cancer. We report here the occurrence of rhabdomyolysis in a patient with SMS, breast cancer, and antibodies that recognize both brain and muscle amphiphysin isoforms. immunotherapy induced a remission of both rhabdomyolysis and SMS symptoms. Autoimmune rhabdomyolysis may represent a paraneoplastic complication of cancer patients with amphiphysin autoimmunity.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/51. chorea, eosinophilia, and lupus anticoagulant associated with acute lymphoblastic leukemia.A child is reported with chorea as the initial presentation of acute lymphoblastic leukemia. Subsequent laboratory studies revealed marked eosinophilia and a lupus anticoagulant. No peripheral or central nervous system lymphoblasts were observed. The chorea, eosinophilia, and lupus anticoagulant all resolved once remission of the acute lymphoblastic leukemia was induced. It is suggested that acute lymphoblastic leukemia be included in the differential diagnosis of chorea and eosinophilia in childhood.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/51. Paraneoplastic myelopathy at diagnosis in a patient with pathologic stage 1A hodgkin disease.The case of a patient with pathologic Stage 1A hodgkin disease is reported in whom subacute paraneoplastic myelopathy developed before treatment. myelography, computed tomography, magnetic resonance imaging, and lumbar puncture examination showed no evidence of central nervous system involvement. The patient was treated with combination chemotherapy (to avoid spinal cord exposure to radiation) without neurologic improvement. The patient's neurologic condition responded well to intrathecal dexamethasone.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/51. Localization of antibody in the central nervous system of a patient with paraneoplastic encephalomyeloneuritis.We report a patient with severe paraneoplastic encephalomyeloneuritis, occult small-cell carcinoma of the lung, and high titers of circulating antineuronal antibody who died shortly after developing limbic encephalitis. The antibody was of IgG class and reacted specifically with nuclei and cytoplasm of all neurons in the pattern typical for encephalomyelitis and subacute sensory neuropathy associated with small-cell carcinoma (type II, anti-Hu). At autopsy, perivascular inflammatory infiltrates were prominent. All samples of serum, CSF, and postmortem peritoneal and pleural fluid contained high titers of antibody. Direct immunofluorescence of frozen tissue revealed IgG bound to most remaining neurons in multiple brain regions in a pattern similar to indirect immunofluorescence of normal brain tissue. IgG was also bound to tumor. Attempts to elute antibody from tissue decreased background staining but did not remove neuronal immunofluorescence. These results indicate that antibody can access and bind specifically to neuronal antigens in the brain during the course of paraneoplastic disease.- - - - - - - - - - ranking = 4keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/51. Encephaloradiculopathy: a non-metastatic complication of hepatocellular carcinoma.We describe a patient with a silent hepatocellular carcinoma in whom a progressive dysfunction developed in the brainstem, cerebrum, and cerebellum. This clinical manifestation was also associated with polyradiculopathy. The changes were microscopically unlike those seen in hepatic encephalopathy, progressive multifocal leukoencephalopathy and other metabolic encephalopathy. The findings seem to have occurred as a paraneoplastic syndrome of the nervous system. Although in the present case, the cause-and-effect relationship between the hepatocellular carcinoma and the encephaloradiculopathy is only hypothetical, we suggest that the demyelinating process which involves both central and peripheral nervous system in our case resulted from interaction of tumor with the host-immunological mechanism. The neurological presentation in our case is unique and has never been reported before.- - - - - - - - - - ranking = 0.9241865279445keywords = nervous system (Clic here for more details about this article) |
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