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)

Myotonic dystrophy or Steinert's disease may be discovered during acute respiratory failure, sometimes caused by a general anaesthetic. It complicates chronic respiratory failure which is present in almost all cases, both restrictive and obstructive, the clinical signs of which progress with the myopathy. Apart from myotonic degeneration of the respiratory muscles, a hypoventilation syndrome of central origin has been described, but the etiology of this respiratory failure is dominated by repeated aspiration pneumonia favoured by constant dysphagia and passage of food into the trachea and poor cough reflex. One should attempt to correct this.
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PMID:[Myotonic dystrophy and acute respiratory insufficiency]. 19 96

Three cases of a painful, inflammatory, nodular myopathy in young and middle-aged adult males are described. In two of the cases the nodular process gradually evolved into a diffuse proximal myopathy accompanied by dysphagia, and manifesting the so-called "facioscapulohumeral syndrome". The electromyogram (EMG) in all three cases was "myopathic" in type and in two cases abnormalities consistent with an inflammatory muscle disorder were seen. Muscle biopsy revealed a consistent pattern of intense, predominantly interstitial, pleomorphic infiltration of muscle with scattered foci of muscle fibre destruction and regeneration, which resembled muscle infarcts. It is suggested that this syndrome, whilst readily recognizable from both the clinical and pathological standpoints, is not a nosological entity in its own right but rather an unusual presentation of polymyositis.
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PMID:Localised nodular myositis: a clinical and pathological variant of polymyositis. 59 1

Twelve relatives of a 15-year-old girl with idiopathic intestinal pseudoobstruction were studied with esophageal manometry or cine-esophagography to determine whether the disease was genetically transmitted. Four maternal relatives, including the patient's mother, 13-year-old brother, one aunt, and one of that aunt's children had mild dysphagia and esophageal motor dysfunction. In addition, the patient's mother and 13-year-old brother had a flaccid bladder and bilateral ureteral reflux, respectively. The brother had abnormal bladder smooth muscle by light microscopy. We conclude that idiopathic intestinal pseudoobstruction in this family is secondary to a generalized disease of smooth muscle which is transmitted as a dominant trait of variable expressivity, manifested in some family members as a mild disorder of esophageal smooth muscle dysfunction, at times accompanied by bladder dysfunction. We suggest that this form of idiopathic intestinal pseudoobstruction be called "hereditary hollow visceral myopathy." Esophageal manometry may prove useful as a tool for studying the inheritance of this disorder.
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PMID:Studies of idiopathic intestinal pseudoobstruction. II. Hereditary hollow visceral myopathy: family studies. 87 35

Two patients are described with distinctive clinical features including an insidious onset, slow progression, bilateral ptosis, weakness of facial muscles, dysphagia, muscle atrophy, and weakness with a distal distribution in the extremities, and cardiomopathy with conduction system disorders. Electromyographic studies and muscle biopsy showed features highly suggestive of a myopathic disorder. One case is considered to be sporadic. The other seems to be a familial disorder, because of the presence of a mild atrioventricular block and right incomplete bundle branch block in the patient's son and the presence of eyelid ptosis in his sister. This may be a variant of oculopharyngeal myopathy with distal and cardiomyopathy. It will be necessary to perform long-term follow-up studies in these families.
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PMID:Oculopharyngeal myopathy with distal and cardiomyopathy. 90 74

A 23-year-old ex-bicycle racer with vague gastrointestinal symptoms had an acute weight loss of 13.6 kg (30 lb). During the period of maximal weight loss, he experienced muscle weakness, dysphagia, bradycardia, and T wave changes on the electrocardiogram. His skeletal muscle biopsy showed a severe vacuolar myopathy devastating the atrophic type II fibers. Without treatment, he recovered completely and has remained well. This may be an exaggerated or acute form of type II atrophy not previously reported, or it may represent an acute muscular degeneration.
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PMID:Reversible vacuolar myopathy of type II fibers. 120 33

Inclusion body myositis is an inflammatory myopathy in which dysphagia has been considered a rare finding. However, recent literature finds dysphagia an increasingly common symptom as more cases of inclusion body myositis are identified. Unlike some inflammatory myopathic disorders, inclusion body myositis is resistant to treatment with corticosteroids, and therefore, the otolaryngologist may be consulted regarding surgical options for relief of dysphagia. A patient is described in whom severe progressive dysphagia associated with inclusion body myositis developed. Impaired pharyngeal wall motion and cricopharyngeal achalasia were demonstrated by videofluoroscopic evaluation, and the patient was successfully treated by cricopharyngeal myotomy. The pathophysiologic nature of inclusion body myositis and the mechanisms of cervical dysphagia in the inflammatory myopathies are reviewed.
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PMID:Management of dysphagia in inclusion body myositis. 131 47

A 2-year-old girl with reducing body myopathy was reported. She had no family history of neuromuscular disease. She developed normally with no delay in milestones during infancy. She had no muscle weakness or hypotonia up to 2 years of age when she received mumps vaccination. Three days after the injection, she was first noticed to have limb muscle weakness. The muscle weakness progressed rapidly with increasing difficulty in gait and raising the upper arms, particularly the left. Four months later, she had difficulty in keeping her head up and could no longer climb the stairs. On physical examination, she had proximal dominant generalized muscle weakness, with a preferential neck muscle involvement. She walked waddlingly and stood up with Gowers' maneuver. Facial and ocular muscles were intact. No dysarthria, dysphagia or respiratory difficulty was noted. EMG showed myopathic pattern. Serum creatine kinase level was moderately elevated to 739 IU/l. In the biopsied left biceps muscle, there was marked variation in fiber size, but no apparent necrotic or regenerating fibers. The most striking feature was the presence of numerous eosinophilic inclusions which reduced nitroblue tetrazolium (NBT) and were, therefore, stained dark with menadione-linked alpha-glycerophosphate dehydrogenase even without the substrate of menadione, showing the histochemical characteristics of "reducing" body. The bodies were predominantly seen in fibers with disorganized intermyofibrillar networks and with high acid phosphatase activity. On electron microscopy, the reducing bodies consisted of fine granular material with the similar electron density to the chromatin granules and were located mostly around the degenerated nuclei, suggesting the nuclear degeneration playing a role in forming the reducing bodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Reducing body myopathy--a case report]. 132 Oct 16

Upper functional dysphagia, occurring in the absence of tumoral obstruction, includes swallowing disorders caused by a disturbance of the reflex, velopharyngoesophageal time of deglutition. Thirty-three patients with such disorders have been studied. Fourteen suffered from a neurologic affection (including 4 brainstem tumors and 6 cerebrovascular neurologic disease), 10 from myopathy, 9 from a localized affection of the upper sphincter of the esophagus. Diverticula were excluded from this study. The exploration resorted to fiberendoscopy of esophageal follow-through, pharyngoesophageal manometry, radiocinema, and MRI for some of the latter patients. Cricopharyngeal myotomy was carried out in 16 of these patients, with good results in 10 and failures in 6. The role of the various additional examinations and of myotomy is discussed.
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PMID:[Upper functional dysphagia. Apropos of 33 cases]. 152 60

Thyroid disease in the elderly can be easily overlooked. Symptoms too often are explained away as normal processes of aging. Development of unstable illness, especially cardiac disease, is a frequent mode of presentation. One symptom or one clinical feature of thyroid disease in the elderly may be overwhelming in its presentation, as in apathetic hyperthyroidism, thyroid myopathy, depression and dementia. Physical examination of the thyroid gland can be helpful but in a high percentage of older patients the gland is normal to palpation. The treatment of hypothyroidism is straightforward. Only myxedema coma requires large doses of levothyroxine parenterally; all other forms of hypothyroidism are treated with oral levothyroxine. The dose is started very low and increased gradually over months. The euthyroid state is achieved gradually and safely. Hyperthyroidism can be treated by several modalities. In the unstable elderly patient, antithyroid medication can quickly produce a euthyroid state. When the patient is stable, further decisions can be made regarding definitive therapy. Radioactive iodine therapy is well-tolerated and effective. On occasion, a second course of therapy is needed to suppress hyperthyroidism. Close follow-up of all patients ever having received this therapy is needed to identify the development of hypothyroidism. Surgical thyroid ablation may be necessary in patients who fail to respond to radioactive iodine therapy. Abnormalities associated with unresolved thyromegaly, dysphagia, or tracheal compression may require surgical intervention. If suspicion exists that the gland is cancerous, surgical intervention is warranted.
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PMID:Hypothyroidism and hyperthyroidism in the elderly. 158 94

The case of a 41 year old woman with amyloid myopathy is reported. Clinical involvement consisted of limb girdle muscle weakness, mild scapular muscle atrophy and dysphagia. In contrast with the published cases, abnormal firmness, pseudohypertrophy of the musculature and macroglossia were absent. Muscle biopsy showed endo- and perimysial amyloid deposits but also inflammatory infiltrates. Inflammatory cells typing was studied by immunocytochemical methods and revealed a predominant T-helper cell infiltration. Free kappa light chains were present in serum and urine. Serum immunoglobulin levels were reduced. Bone marrow examination revealed mild plasmocytosis without abnormal cells. Immunofluorescence and immunoperoxidase techniques for identification of the type of amyloid fibrils showed positivity with antisera to kappa light chains. A 4-year follow-up revealed a progressive worsening of muscle weakness despite immunosuppressive treatment. No malignant plasmocytosis occurred. The unusual inflammatory muscle infiltration observed in this case may suggest an associated polymyositis.
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PMID:[Non hypertrophic amyloid myopathy with muscular inflammation in plasma cell dyscrasia]. 160 34


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