1/241. Distal myasthenia gravis.myasthenia gravis (MG) characteristically involves ocular, bulbar, and proximal extremity muscles. Distal extremity muscles are typically spared or less prominently involved. The authors performed a retrospective chart review of MG patients treated at two university-based neuromuscular clinics. From a total population of 236, nine patients (3%) had distal extremity weakness exceeding proximal weakness by at least one Medical research Council grade during their illness. hand muscles, particularly finger extensors, were involved more frequently than were distal leg and foot muscles.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/241. Treatment of severe myasthenia gravis with protein A immunoadsorption and cyclophosphamide.myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction caused by antibodies against the nicotinic acetylcholine receptor (AChR-Ab). We report a 16-year-old girl with severe MG who showed a poor response to plasma exchange and tryptophan-linked polyvinylalcohol gel immunoadsorption. Further improvement of muscle strength and decline of AChR-Ab could be achieved after initiation of protein-A immunoadsorption (PA-IA). maintenance therapy with PA-IA and intravenous pulses of cyclophosphamide resulted in a stabilisation of the disease, with a complete remission during the follow-up period of six months. We suggest that PA-IA may be valuable and safe in the management of patients with severe MG, and maintenance therapy with PA-IA and cyclophosphamide may prevent serious and potentially life-threatening relapses of the disease.- - - - - - - - - - ranking = 0.25keywords = muscle (Clic here for more details about this article) |
3/241. Development of myasthenia gravis after interferon alpha therapy.Interferon (IFN) alpha is now used in the treatment of some malignant diseases and chronic viral hepatitis. There have been several reports of development of autoantibodies and autoimmune diseases or the deterioration of preexisting disorders in patients under treatment. We enclose a case of myasthenia gravis (MG) which developed after six weeks of treatment as fluctuating bilateral ptosis, intermittent diplopia, and mild weakness of limb and neck muscles. A test dose of edrophonium chloride was administered, resulting in improved muscle strength. Elevated anti acetylcholine receptor (AChR) antibody titer was found. Single fiber electromyography showed an increased jitter from extensor digitorum communis, frequently accompanied by transmission blocking. Repetitive electric 3 Hz stimulation of the abductor pollicis brevis muscle, revealed an abnormal decrement of 28% in compound motor action potential. myasthenia gravis was diagnosed and the patient was given pyridostigmine, immunoglobulines and prednisone with benefit. Six months latter he developed an acute myasthenic crisis with severe respiratory failure and high anti AChR antibody titer. IFN-alpha can induce MG or simply manifests a preexisting subclinical disease, but otherwise its therapeutic efficacy in MG has been shown in experimental and clinical studies. Autoimmune mechanisms, as the release of different cytokines as IFN, by immunocompetent cells, may be involved in the pathogenesis of both MG and chronic active hepatitis. Autoantibody production against postsynaptic membrane structures by IFN-alpha could be the underlying pathophysiology.- - - - - - - - - - ranking = 0.75keywords = muscle (Clic here for more details about this article) |
4/241. edrophonium-induced macrosaccadic oscillations in myasthenia gravis.In a myasthenia gravis patient with profound ophthalmoplegia who was given edrophonium during forward fixation or horizontal/vertical saccades, horizontal macrosaccadic oscillations occurred that were conjugate, 2 to 3 Hz, and of peak-to-peak amplitude up to 50 degrees. The intersaccadic interval averaged 129.5 msec as measured by electro-oculography. eye closure or the use of Frenzel goggles markedly reduced the amplitude and frequency of the macrosaccadic oscillations. This short latency is similar to that of a corrective saccade, and suggests that edrophonium induces macrosaccadic oscillations through a non-visually guided reflex saccade by temporarily unmasking the adaptively increased saccadic gain to overcome myasthenic ocular paresis.- - - - - - - - - - ranking = 0.079809642750229keywords = paresis (Clic here for more details about this article) |
5/241. chiropractic management of a patient with myasthenia gravis and vertebral subluxations.OBJECTIVE: The chiropractic management of a patient with myasthenia gravis and vertebral subluxation is described. We discuss the pathophysiology, clinical features, and treatment of patients with these diseases. CLINICAL FEATURES: The 63-year-old male patient suffered from complaints associated with the disease myasthenia gravis along with signs of vertebral subluxation. The patient had an initial complaint of dysphagia. In addition, the patient experienced swelling of the tongue, nausea, digestive problems, weakness in the eye muscles, difficulty breathing, myopia, diplopia, and headaches. Balance and coordination problems resulted in walking difficulties. INTERVENTION AND OUTCOME: Contact specific, high-velocity, low-amplitude adjustments were applied to sites of patient subluxation. myasthenia gravis is no longer debilitating to the patient; he is medication free and has resumed a "normal life." CONCLUSION: The clinical aspects of the disease, including the possible role of chiropractic intervention in the treatment of patients suffering from myasthenia gravis, are also discussed. This case study encourages further investigation into the holistic approach to patient management by chiropractors vis-a-vis specific adjustments of vertebral subluxation.- - - - - - - - - - ranking = 0.25keywords = muscle (Clic here for more details about this article) |
6/241. Exacerbation of myasthenia gravis in a patient after interferon-beta treatment for chronic active hepatitis c.We report the case of a 53-year-old female patient, who developed bilateral blepharoptosis, limb weakness, dysphagia, and dyspnea several days after human natural interferon-beta (IFN-beta) treatment for chronic active hepatitis c. A positive edrophonium test, an elevated anti-acetylcholine receptor antibody titer, and decrements in the amplitude of muscle action potentials evoked by repetitive stimulation confirmed the diagnosis of myasthenia gravis (MG). Since she had been suffering from drooping of her right eyelid, fluctuating diplopia and easy fatiguability of limbs before receiving IFN-beta, her symptoms of MG were considered to be exacerbated by IFN-beta. It is recommended that IFN-beta should be used with particular care in patients with known MG or its compatible symptoms.- - - - - - - - - - ranking = 0.25keywords = muscle (Clic here for more details about this article) |
7/241. Pseudo-myasthenia gravis and thymic hyperplasia in Graves' disease.BACKGROUND: Diagnostic confusion between thyroid disease and myasthenia gravis (MG) can arise because the two may have similar clinical features, and also because of the more frequent coexistence of these autoimmune disorders in the same individual. In MG, autoantibodies directed against the acetylcholine receptor result in muscle weakness. Thymic pathology is well recognized in MG, with thymic hyperplasia frequent in early onset MG and thymoma more common in later onset MG. In Graves' disease, autoantibodies against thyroid antigens result in hyperthyroidism. A seldom-recognized feature of Grave's disease is the occurrence of an enlarged thymus (thymic hyperplasia) on chest CT, or of thymic lymphoid hyperplasia pathologically. CASE STUDY: This report describes a case in which the discovery of a mediastinal mass during imaging of the thyroid, and the presence of myasthenic-like symptoms, in a patient with Graves' disease prompted investigations into whether the patient also had MG. RESULTS: Despite symptoms which strongly suggested MG, subsequent investigations did not confirm the diagnosis, and treatment of Grave's lead to a resolution of the symptoms and regression of the thymic enlargement seen on CT. CONCLUSIONS: The case study highlighted clinical similarities between Grave's disease and myasthenia gravis which might cause diagnostic confusion, and also the investigations which are useful in order to differentiate the two diseases. In addition to common clinical features, the autoimmune diseases Grave's disease and myasthenia gravis may both produce radiological thymic enlargement.- - - - - - - - - - ranking = 0.25keywords = muscle (Clic here for more details about this article) |
8/241. Myasthenia, thymoma, presynaptic antibodies, and a continuum of neuromuscular hyperexcitability.BACKGROUND: autoantibodies specific for the nicotinic acetylcholine receptor (AChR) of skeletal muscle impair neuromuscular transmission in myasthenia gravis (MG). autoantibodies specific for alpha3 neuronal AChRs or voltage-gated potassium channels have been reported in patients with isaacs syndrome, an acquired disorder of continuous muscle fiber activity characterized by neuromyotonia. OBJECTIVE: To report the neuromuscular autoantibody profiles of three patients with a syndrome of MG and neuromuscular hyperexcitability. RESULTS: All three patients reported here had clinical and electrophysiologic evidence of MG and neuromuscular hyperexcitability. None had neuromyotonia. thymoma was proven in two patients and suspected in the third. One had MG and thymoma and subsequently developed cramp-fasciculation syndrome; MG and rippling muscle syndrome appeared simultaneously in the other two. All patients had muscle and neuronal AChR binding antibodies and striational antibodies. Only one had antibodies reactive with alpha-dendrotoxin-complexed potassium channels. CONCLUSIONS: The coexistence of cramp-fasciculation syndrome and acquired rippling muscle syndrome with MG, thymoma, and neuronal AChR autoantibodies suggests that there is a continuum of autoimmune neuromuscular hyperexcitability disorders related pathogenically to isaacs syndrome. Manifestations of neuromuscular hyperexcitability may be altered and less apparent in the context of MG because of the coexisting defect of neuromuscular transmission.- - - - - - - - - - ranking = 1.25keywords = muscle (Clic here for more details about this article) |
9/241. 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.- - - - - - - - - - ranking = 0.39904821375115keywords = paresis (Clic here for more details about this article) |
10/241. Immunosuppressive treatment of rippling muscles in patients with myasthenia gravis.Rippling muscle disease is a rare autosomal dominant disorder that may occur sporadically. In this report two patients presenting with rippling muscles followed by myasthenia gravis are described. Our first patient developed rippling muscles about 1 month after infection with yersinia enterocolitica. Two years later myasthenia gravis appeared. Our second patient had a 2-year history of asthma prior to the onset of rippling muscles which preceded the myasthenic symptoms by 4-8 weeks. acetylcholine receptor and anti-skeletal muscle antibody titers were positive in both patients. In both patients the rippling phenomena worsened with pyridostigmine treatment but markedly improved after immunosuppression with azathioprine.- - - - - - - - - - ranking = 2.25keywords = muscle (Clic here for more details about this article) |
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