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1/67. Neuroendocrine lung tumors and disorders of the neuromuscular junction.

    We report four cases of lambert-eaton myasthenic syndrome (LEMS) or myasthenia gravis (MG) associated with pulmonary neuroendocrine carcinoma having prolonged survival. The tumors were atypical carcinoid or large cell neuroendocrine carcinoma. LEMS is associated with several neuroendocrine carcinomas. Because some neuroendocrine carcinomas have a better prognosis, aggressive tissue diagnosis of lung cancer in LEMS is warranted. Whether the association between MG and atypical carcinoid is a significant co-occurrence is uncertain. ( info)

2/67. lambert-eaton myasthenic syndrome associated with idiopathic thrombocytopenic purpura and diffuse panbronchiolitis: long-term remission after a course of intravenous immunoglobulin combined with low-dose prednisolone.

    We report a case of lambert-eaton myasthenic syndrome (LEMS) associated with idiopathic thrombocytopenic purpura (ITP) and diffuse panbronchiolitis (DPB). An extensive search for malignancy yielded negative results. Interestingly, ITP and DPB developed simultaneously when the patient suffered from myasthenic symptoms. This is the first report in the Japanese or English literature of an association of LEMS, ITP, and DPB. The use of cholinesterase blocker alone did not improve the myasthenic symptoms, and the symptoms and signs relapsed with the tapering of prednisolone (PSL) dosage. However, after administration of immunoglobulin (IVIG) (0.4 g/kg/day x 5 days), low-dose PSL (20 mg/day) alleviated the LEMS and ITP, and the diseases have remained in remission for 8 months without additional IVIG. We suspect that there is a synergistic relationship between IVIG and PSL. ( info)

3/67. lambert-eaton myasthenic syndrome with ophthalmoparesis and pseudoblepharospasm.

    We report a patient initially diagnosed as having ocular myasthenia gravis who showed progressive ophthalmoparesis and pseudoblepharospasm together with positive acetylcholine receptor antibodies. Repeated evaluation with high-frequency repetitive stimulation revealed an incremental response and elevated titers of antibodies against presynaptic calcium channels, confirming lambert-eaton myasthenic syndrome. Systemic evaluation revealed no malignant neoplasm but revealed euthyroid Hashimoto's disease. Immunomodulative therapy including plasma exchange and administration of an immunosuppressent (azathioprine) combined with a potassium-channel blocker (3,4-diaminopyridine) reduced the ocular abnormalities. We conclude that the ocular manifestations in this patient were probably caused by lambert-eaton myasthenic syndrome. ( info)

4/67. Poorly differentiated carcinoma of the lung presenting with Lambert--Eaton myasthenic syndrome.

    lambert-eaton myasthenic syndrome commonly seen in small-cell lung cancer represents an autoimmune reaction against antigens coexpressed by tumor and neurons. It is rarely seen with other histologic subtypes. Symptoms antedate the appearance of the neoplasm by weeks to years. Therapeutic options range from immunosuppression, plasmapheresis, pharmacologic facilitation of neuromuscular transmission, and definitive therapy of the primary tumor. This case report describes the rare association of lambert-eaton myasthenic syndrome with non-small-cell lung cancer. ( info)

5/67. lambert-eaton myasthenic syndrome in association with transitional cell carcinoma: a previously unrecognized association.

    The lambert-eaton myasthenic syndrome is known to occur with, or precede, a variety of malignancies, most commonly oat cell carcinoma of the lung. We report the first case of this syndrome associated with transitional cell carcinoma of the bladder and ureter. A brief review of published reports on the presentation, diagnosis, and treatment is included. ( info)

6/67. myasthenia gravis with a paraneoplastic marker.

    Ocular manifestations of myasthenia gravis are very common. myasthenia gravis may be associated with lung carcinoma. Lambert-Eaton syndrome is also commonly associated with lung carcinoma and can have ocular manifestations. Overlap of these two entities has been described. The case of a patient with fatigable diplopia and ptosis 3 years after removal of a large-cell lung carcinoma is presented. Tests results for acetylcholine receptor binding and modulating antibodies were positive for myasthenia gravis. Test results for presynaptic voltage-gated calcium channel antibodies of the N-type were also positive. However, test results for the P/Q-type voltage-gated calcium channel antibodies, which are consistent with Lambert-Eaton syndrome, were negative. autoantibodies can be used to serologically distinguish paraneoplastic myasthenia gravis from Lambert-Eaton syndrome. ( info)

7/67. Chronic multiple paraneoplastic syndromes.

    A patient presented with symptoms of limbic and brainstem encephalitis, motor and sensory neuronopathy, cerebellar dysfunction, and highly positive anti-Hu antibodies. He also harbored P/Q-type calcium channel antibodies and manifested the lambert-eaton myasthenic syndrome (LEMS). Small-cell lung cancer was found, and he received both antineoplastic therapy and intravenous immunoglobulin (IVIg). Remission of the malignancy was achieved. Although the anti-Hu-related manifestations improved after therapy, LEMS has persisted, leading to IVIg dependency. ( info)

8/67. Neurogenic bladder in lambert-eaton myasthenic syndrome and its response to 3,4-diaminopyridine.

    Autonomic dysfunction, as well as neuromuscular involvement, is a common manifestation of lambert-eaton myasthenic syndrome (LEMS). Dry mouth and impotence have been described as typical features of autonomic dysfunction, but neurogenic bladder is infrequent or subclinical in LEMS. We report a patient with neurogenic bladder secondary to LEMS whose condition responded to 3,4-diaminopyridine (3,4-DAP). In this patient's serum, results of repeated measurement with P/Q-type VGCC antibodies proved positive, but not with N-type VGCC and synaptotagmin antibodies. A review of the literature turned up a few patients with voiding dysfunction related to LEMS, but no urodynamic studies were done on these patients. Ours is the first case in which 3,4-DAP was efficacious in treating LEMS and neurogenic bladder. Responses of 3,4-DAP in urodynamic studies suggest that in this LEMS patient neurogenic bladder was caused by defective neurotransmission both in the autonomic detrusor and skeletal abdominal muscles. ( info)

9/67. Paraneoplastic and metastatic neurologic complications of Merkel cell carcinoma.

    Merkel cell carcinoma is a rare primary cutaneous neuroendocrine tumor that is locally aggressive and frequently accompanied by distant metastases. Neurologic complications of Merkel cell carcinoma are rare. We describe a 69-year-old man who presented with lambert-eaton myasthenic syndrome and was found to have Merkel cell carcinoma. The paraneoplastic syndrome improved with initial treatment of the malignancy. He subsequently developed a solitary brain metastasis and died of leptomeningeal carcinomatosis. ( info)

10/67. Lambert-Eaton myaesthenic syndrome: a possible association with Hodgkin's lymphoma.

    lambert-eaton myasthenic syndrome is a presynaptic neuromuscular junction disorder typically associated with small cell lung carcinoma. The characterstic electrophysiological abnormality is a low amplitude compound muscle action potential that shows a marked increment after maximal voluntary contraction or brief tetanic nerve stimulation. We describe a patient who had LEMS in association with Hodgkin's disease. A 61 year old woman presented with proximal muscle weakness 6 years following successful treatment of Hodgkin's disease. Her symptoms responded well to treatment with diaminopyridine. 9 additional patients have been described with LEMS in association with lymphoproliferative diseases. A systemic malignancy is usually found within 2 years of LEMS diagnosis but may present later. LEMS should be considered in patients with Hodgkin's disease presenting with muscle weakness. ( info)
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