1/100. myasthenia gravis and peripheral neuropathy in an Amazon indigenous female.A 15 year-old female presented with anti-acetylcholine receptor antibody-positive myasthenia gravis and electrophysiological signs of sensory peripheral neuropathy. The repetitive stimulation test showed decremental response of 31% in the median nerve. The clinical picture improved with prostigmine, corticosteroids, plasmapheresis and thymectomy. This is the first case report of an Amazon indigenous patient with myasthenia gravis.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/100. Coexistence of primary biliary cirrhosis and myasthenia gravis: a case study.We present a case that suggests a relationship between primary biliary cirrhosis and myasthenia gravis. A 43-year-old Japanese woman was admitted to the Nagoya City University Medical School, First Department of internal medicine with abnormal liver function in August 1991. She had had ptosis of the right eye since 1990. She had not been treated for liver disease. Ptosis of the right eye and hepatomegaly were present. serum laboratory examinations revealed elevated biliary enzymes and IgM levels; tests were positive for antimitochondrial antibody and antiacetylcholine antibody. liver histology revealed chronic non-suppurative destructive cholangitis and led to a diagnosis of primary biliary cirrhosis. The tensilon test was positive. electromyography with repetitive motor nerve stimulation revealed a neuromuscular junction defect; i.e., the primary characteristic of myasthenia gravis. The patient was diagnosed with myasthenia gravis. Although the development of myasthenia gravis has previously been reported in patients with primary biliary cirrhosis during D-penicillamine administration, this is a very rare case of the coexistence of both diseases before such treatment.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/100. A case of myasthenia gravis proven by ultrastructural study.Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/100. Neuromuscular interaction of sevoflurane--cisatracurium in a myasthenic patient.PURPOSE: To describe the influence of sevoflurane anesthesia on cisatracurium neuromuscular block in a myasthenic patient undergoing thymectomy. CLINICAL FEATURES: A myasthenic patient (Osserman IIB) was managed for one year before surgery with 60 mg pyridostigmine qid, 50 mg immuran tid and 30 mg prednisone therapy. Pyridostigmine was interrupted three months before surgery, and five sessions of plasmapheresis were done within 13 days before surgery. The neuromuscular response was monitored by Datex electromyographic response to train-of-four stimulation of the ulnar nerve. Sevoflurane 4% decreased the T1/C ratio by 20%. Administration of 0.025 mg x kg(-1) cisatracurium, during sevoflurane anesthesia, was followed by complete neuromuscular block for 45 min. Discontinuation of sevoflurane resulted, after 10 min, in recovery of the T which reached T1/C ratio of 50% after 30 min. CONCLUSION: The marked sensitivity of this myasthenic patient to 0.5 x ED95 of cisatracurium can be attributed to potentiation of cisatracurium neuromuscular block by sevoflurane, as evidenced by the reappearance of the first twitch of the train-of-four response 10 min after sevoflurane was discontinued.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
5/100. Graft-versus-Host disease-like syndrome in malignant thymoma.Allogenic transfusion of immunocompetent T lymphocytes into an immunodeficient recipient is necessary for the development of graft-versus-host-disease (GVHD). The gastrointestinal tract is one of the most involved organs in human GVHD, and single-cell necrosis with apoptotic change and crypt abscess are characteristic histopathologic features. The thymus is important in immune regulation, and dysregulation of the immune system can be expected once its microenvironment is disrupted. We report the case of a 38-year-old woman with malignant thymoma without transplantation or transfusion history who initially presented with myasthenia gravis and clinically developed a GVHD-like syndrome with characteristic GVHD-like colitis on colonoscopy. We propose that disruption of the thymic microenvironment caused a dysregulated immune system and development of a GVHD-like syndrome.- - - - - - - - - - ranking = 0.013210243325268keywords = organ (Clic here for more details about this article) |
6/100. Gabapentin may be hazardous in myasthenia gravis.A patient with painful neuropathy developed ocular, facial, and masticatory weakness and fatigue after 3 months of gabapentin (GBP) treatment (400 mg/day). An elevated level of serum acetylcholine receptor antibodies (AChR-Ab) was detected. The patient recovered following pyridostigmine therapy and withdrawal of GBP and, 2 years later, is practically asymptomatic despite positive AChR-Ab. Because of this clinical observation, we gave 150 mg/kg GBP to rats with experimental autoimmune myasthenia gravis (EAMG). Repetitive nerve stimulation at 3-Hz was performed, and the 5th/1st amplitude ratio was used to calculate the decremental response. In all EAMG rats, GBP induced a transient, abnormal decrement (7-20%) 90 to 240 min after administration. No decrement was induced by GBP in normal rats. Thus, GBP aggravates the decrement in EAMG. The mechanism involved in the hitherto unreported possible unmasking of myasthenia gravis (MG) by GBP is unknown. Gabapentin should be used with caution in this disease.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/100. Dysphagia with multiple autoimmune disease.myasthenia gravis (MG) and polymyositis (PM) are organ-specific autoimmune diseases. Occasional reports describe patients with clinical and pathologic features of both. Achalasia is idiopathic in nature, but autoimmune and inflammatory mechanisms have been proposed for this disorder as well. We describe a patient with dysphagia who was diagnosed at different points in time with all these three rare conditions. Despite at least putatively having immune mechanisms in common, an association between the three has not been previously described.- - - - - - - - - - ranking = 0.013210243325268keywords = organ (Clic here for more details about this article) |
8/100. myasthenia gravis in conjunction with Graves' disease: a diagnostic challenge.OBJECTIVE: To describe an association between Graves' disease and myasthenia gravis and discuss the clinical features and laboratory tests that may help distinguish these two diseases. methods: The clinical, laboratory, and electrophysiologic findings in a patient with Graves' disease and myasthenia gravis are presented. RESULTS: A 28-year-old African American man was admitted to the University of Louisville Hospital with generalized muscle weakness, exophthalmos, diplopia, weight loss, and mild dysphagia. The diagnosis of Graves' disease with ophthalmologic involvement was suspected clinically and confirmed by an undetectable thyrotropin level (<0.03 mIU/mL), high total thyroxine (20.5 mg/dL), and increased homogeneous 123I thyroid uptake. Because of the generalized muscle weakness and mild dysphagia, assessment was done by a neurology team, and severe thyrotoxic myopathy was diagnosed. He was treated with 131I and b-adrenergic blocking agents and scheduled for follow-up as an outpatient. Two weeks later, the patient presented in acute respiratory failure. The neurology team was reconsulted because of suspected myasthenic crisis. Anti-acetylcholine receptor antibodies were undetectable in the serum, and computed tomography of the chest showed no thymic enlargement. Repetitive nerve stimulation testing, however, showed findings consistent with an abnormality of the neuromuscular junction. The patient responded dramatically to an anticholinesterase agent and corticosteroids. CONCLUSION: The overlapping clinical features may cause diagnostic confusion when myasthenia gravis and Graves' disease coexist, and numerous tests may be needed to distinguish these two conditions, which have differing treatments and prognoses.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
9/100. myasthenia gravis presenting as a unilateral abducens nerve palsy.A 48-year-old man with no significant medical history presented to the emergency department of a large, urban, tertiary-care hospital complaining of double vision and unsteady gait. physical examination was remarkable for an isolated, unilateral sixth nerve palsy. After comprehensive testing, a final diagnosis of myasthenia gravis was made. The patient's unsteady gait was a manifestation of sensory distortion from the diplopia and corrected when the patient closed his eyes. The presentation of myasthenia gravis as an isolated sixth nerve palsy is unprecedented in the emergency medicine literature.- - - - - - - - - - ranking = 6keywords = nerve (Clic here for more details about this article) |
10/100. myasthenia gravis and lymphoblastic lymphoma involving the thymus: a rare association.myasthenia gravis (MG) is an autoimmune neuromuscular junction disease. An association between thymic epithelial neoplasms and MG is well known. However, it is rarely associated with hematologic malignancies. In particular, very few cases of lymphoblastic lymphoma involving the thymus and MG have been reported. Here we report a case T-cell lymphoblastic lymphoma involving the thymus who developed MG after the initial diagnosis. The patient initially presented with a mediastinal mass which was diagnosed as lymphoblastic lymphoma. MG was diagnosed during leukemic relapse in this patient and was based on clinical presentation and neurophysiologic studies including single fiber electromyography (EMG) and repetitive nerve stimulation tests. In contrast to the other cases with such an association, the myasthenic symptoms presented nine months after the diagnosis of lymphoma by thymectomy. The patient had a highly aggressive clinical course and was resistant to various chemotherapy regimens.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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