Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myasthenia gravis is an infrequent disease seen at the neonatal stage. Two main groups are identified: a) one with a genetic origin which can be secondary to pre- or postsynaptic defects and b) of an acquired origin, as transitory neonatal myasthenia seen in 10-15% of those children with myasthenic mothers. Few cases with a genetic origin have been reported in the literature; the most common symptoms being bilateral eyelid ptosis, ophtalmoparesis, easy fatigability, respiratory and feeding difficulties. This is a report of a genetic neonatal case of myasthenia gravis with dysphagia as the only clinical manifestation seen since the first days of the child's life, confirming the diagnosis using a repetitive supramaximal stimulation test and obtaining excellent results with pyridostigmine. We conclude that, although this a rare form of the disease, genetic neonatal myasthenia gravis should be considered in the differential diagnosis of newborns with difficulties to swallow. The repetitive supramaximal stimulation test is the diagnostic procedure of first choice to be used in the neonatal period.
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PMID:[Congenital myasthenia gravis. Presentation of a case with dysphagia as the only clinical manifestation]. 209 41

The purposes of this paper are to evaluate degree of dysphagia at the pharyngeal stage of swallowing in patients with myasthenia gravis (Osserman IIB). A catheter with three diode transducers 5 cm apart was swallowed through the nose into the upper esophagus. Firstly a patient was commanded to drink water of 1 ml at about five second intervals, and the swallowing pressures were recorded on condition that the speed of paper recording and catheter pull-through is the same 1 mm/sec. Secondly the swallowing pressures were recorded on condition that the middle transducer is fixed at the level of the upper esophageal sphincter and the speed of paper recording is 5 mm/sec before and after intravenous injection of 10 mg edrophonium chloride. Thirdly the resting pressure of upper esophageal sphincter was recorded on the same condition of the first recording. The results were as follows: (1) All seven patients showed extremely low swallowing pressure at the all level of the pharynx compared with healthy men. (2) At the edrophonium test all seven patients revealed increased percentage of swallowing pressure. (3) One patient showed poor relaxation of the upper esophageal sphincter. (4) The resting pressure of the upper esophageal sphincter was low compared with control. In myasthenia gravis manometric study of the pharynx and the pharyngoesophageal sphincter is a useful method for evaluating swallowing function.
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PMID:[Manometric study of the pharynx and pharyngoesophageal sphincter in myasthenia gravis]. 226 3

In myasthenia gravis and amyotrophic lateral sclerosis the ENT specialist or the phoniatrician may be consulted first, because in about 30 percent of all cases the initial symptoms are dysarthria, dysphagia or dyspnea. Three typical cases of each condition are presented. The quality of life of the patients can be improved considerably by early diagnosis and treatment. Special diagnostic and therapeutic procedures are described.
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PMID:[Dysarthria, dysphagia or dyspnea as a reason for the initial consultation in pseudoparalytic myasthenia gravis and amyotrophic lateral sclerosis]. 231 Apr 61

Dysarthria, dysphagia and repeated aspiration in a 54-year-old woman diagnosed and treated for myasthenia gravis 7 years earlier were initially thought to represent a late exacerbation of myasthenia. A cervical mass invading the jugular foramen and causing multiple lower cranial nerve palsies was biopsied and found to represent invasive ectopic thymoma.
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PMID:Late pseudo-exacerbation of myasthenia gravis due to ectopic thymoma invading lower cranial nerves. 231 Oct 16

A case of myasthenia gravis accompanied with polymyositis and malignant thymoma, detected immune complexes in the sera and around the muscle fibers, was described. A 37-year-old woman was admitted to Shinshu University Hospital in September, 1987 because of dyspnea, dysphagia and muscle weakness. She first noticed her right blepharoptosis 3 weeks before admission. Weakness of all four limbs and myalgia of lower extremities were noticed one week later. These symptoms got worse and nocturnal dyspnea, dysphagia and easy fatigability at mastication appeared. On admission, she looked ill and neurological examination revealed left blepharoptosis, bilateral facial weakness, weakness of all four limbs, more prominent in proximal muscles and tenderness of lower extremities. Edrophonium test was positive, improving her muscle weakness. Laboratory examination revealed the elevated serum levels of CK, the increased titre of circulating immune complexes and high titres of acetylcholine receptor antibodies and anti-skeletal muscle antibodies. Electromyographic study showed myogenic pattern and Harvey-Masland test revealed waning at low frequency stimulation. Muscle biopsy showed marked perivascular infiltration of lymphocytes, accompanied by phagocytosis and interstitial fibrosis. IgG deposits were shown around the muscle fibers exclusively around the infiltrates of mononuclear cells. Granular deposits of C3 were also shown specifically around the muscle fibers exclusively around the infiltrates of mononuclear cells. Thymectomy was performed on September 21, 1987. Invasion of thymoma, predominantly lymphocytic type, to right lung and pericardium was observed histologically. After thymectomy, she got better. Immunological data and immunohistochemical examination of the present case suggest that in the case of myasthenia gravis accompanied with polymyositis and malignant thymoma, immune complexes may play a primary role on the pathogenesis of myositis.
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PMID:[Detection of immune complexes in the sera and around the muscle fibers in a case of myasthenia gravis and polymyositis]. 253 18

A number of reports describe the otolaryngologic manifestations of myasthenia gravis. Symptoms such as dysphagia, dysarthria, and dysphonia are quite common to myasthenia gravis, yet the usual onset of this disorder is insidious in nature. We report a case of an 18-year-old woman, previously undiagnosed, who presented with acute dysphagia followed by the rapid onset of respiratory failure. The diagnosis and treatment of myasthenia gravis are discussed.
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PMID:Respiratory failure as the initial presentation of myasthenia gravis. 274

Seven patients with myasthenia gravis (MG) unresponsive to thymectomy and steroid treatment (Osserman group III) underwent plasma perfusion (PP). All patients showed palpebral ptosis, diplopia, dysphonia, dysphagia, and muscle weakness; five of them had impaired ventilatory function. Separated plasma was perfused onto a column to adsorb anti-AChR-Ab. Each patient received a treatment cycle of six PP sessions. Clinical conditions were assessed before and after the treatment, with evaluation of muscular strength, ventilatory function, and electromyographic testing (RSS). Immunologic markers were tested before and after each PP. Clinical improvement in bulbar symptoms and respiratory function was noted in all patients after one to three PP. Limb muscle strength began to improve later. Serum concentration decreased (mean % +/- SD) after each PP:anti-AChR-Ab 36.47 +/- 17.43; IgA 20.44 +/- 11.26; IgG 21.24 +/- 32.56; IgM 23.22 +/- 11.40; C3 36.78 +/- 10.15; C4 42.69 +/- 14.82. In five of seven patients the improvement continues (follow-up 1 to 10 months). In one patient it lasted only 1 month, and in another a relapse occurred after 10 months of benefit, but was successfully reversed by retreatment.
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PMID:Plasma perfusion in myasthenia gravis. 319 71

Dysphagia is often present early, with varying degrees of involvement, in patients with myasthenia gravis. Twenty-five patients with a clinical status of myasthenia gravis were graded according to a modified Osserman classification and the esophageal manometric results were reported according to their clinical status. The results were also compared with those in 20 normal control subjects. Twenty-four patients (96%) had abnormal motility. The only patient who was in class I had a normal tracing. There was no significant difference in function of the lower esophageal sphincter, but a significant decrease in mean amplitude (15.1 +/- 6.1 versus 29.7 +/- 4.7 mm Hg) and a prolongation in mean duration of the peristaltic wave (7.4 +/- 2.8 versus 4.5 +/- 0.2 seconds) was noted in the upper esophagus. The cricopharyngeal sphincter pressure also showed a significant decrease (23.4 +/- 9.5 versus 43.1 +/- 3.1 mm Hg), but relaxation and coordination were good. The mean amplitudes, mean duration of peristaltic waves, and cricopharyngeal pressures between the myasthenic and control populations were statistically significant, with a p value of 0.001. Besides this, frequent spontaneous contractions, simultaneous contractions, and biphasic repetitive contractions typical of peristaltic dysfunction were also found in myasthenic patients. Motor dysfunction of the esophagus as assessed by manometric study correlates well with Osserman's modified clinical classification.
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PMID:Esophageal manometric studies in patients with myasthenia gravis. 333 94

Experimental autoimmune myasthenia gravis (EAMG) was induced in rhesus monkeys using purified acetylcholine receptor (AChR) from Torpedo california. A single dose of 80 micrograms induced antibody formation two weeks after injection. Two subsequent doses at two-week intervals caused clinical signs (anorexia, fatigability, weight loss, ptosis and dysphagia) which initially responded to treatment with neostigmine. Histologic examination of post-mortem tissues revealed lesions characteristic of myasthenia gravis in man: muscular atrophy, fibrous degeneration and lymphocytic infiltration. Antibodies were quantitated in the sera of three other monkeys which received only 60 micrograms of purified AChR. Abnormally high titers persisted for two years (60-200 micrograms /ml versus 0-10 micrograms/ml for controls). A monkey injected with 60 micrograms AChR as part of reconstituted membrane vesicles had lower titers (30-50 micrograms/ml) than those which received purified receptor. Only those monkeys with antibody titers exceeding 800 micrograms/ml developed overt disease. These titers were 4-100 times higher than those reported for myasthenic humans. The antibody-antigen molar ratios were higher for monkeys with disease than for asymptomatic animals. These data suggest that the diversity of antibody molecules synthesized by the sensitized monkeys determined the appearance of clinical signs, and that the cross reaction of anti-torpedo antibodies with monkey receptor was primarily responsible for the development of EAMG.
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PMID:Induction of experimental myasthenia gravis in rhesus monkeys: a model for the study of the human disease. 379 23

The patient who has multiple cranial neuropathies may pose a diagnostic dilemma. The neurologic disorders of amyotrophic lateral sclerosis, multiple sclerosis, myasthenia gravis, and poliomyelitis often cause bulbar dysfunctions such as diplopia, facial weakness, slurred or hypernasal speech, dysphagia, and hoarseness. In general, treatment is supportive and is directed toward restoring or aiding lost function (i.e., tracheostomy, esophagostomy, and cricopharyngeal myotomy). The relative infrequency of these disorders can lead to delays in diagnosis and rehabilitative therapy.
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PMID:Neurologic disorders: amyotrophic lateral sclerosis, myasthenia gravis, multiple sclerosis, and poliomyelitis. 710 59


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