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

Inclusion body myositis has been recognized as a major form of idiopathic inflammatory myopathy. An old male patient with insidious onset and slowly progressive muscular weakness and artrophy has been reported in this article. The duration of symptom before biopsy was 23 years. The first symptom was dysphagia, and muscular weakness developed seven years later. Muscular atrophy was predominant symmetrically and proximally, particularly the quadriceps femoris muscles. Cervical and abdominal muscles were also affected. Myalgia was absent. Electromyogrophy showed myopathic alterations. Erythrocyte sedimentation rate, creatine kinase, immunoglobulins G increased slightly or moderately. Rheumatoid factor was positive, and he had been diagnosed as having rheumatoid arthritis for 23 years. Inclusion body myositis was ultimately diagnosed based on the muscle biopsy which showed mononuclear cell invasion of nonnecrotic muscle fibers, the characteristic rimmed vacuoles in cryostat sections and cytoplasmic inclusion bodies consisted of plenty of tubulofilaments by electron microscope.
 ...
PMID:[Inclusion body myositis: clinical and myopathological features]. 1043 72

Since 1951, when it was first used as a treatment for post-poliomyelitis dysphagia, cricopharyngeal myotomy (CPM) has been used in the treatment of various neurogenic, myogenic, structural, and idiopathic disorders. Yet, the efficacy of CPM in treating patients with upper esophageal sphincter (UES) disorders remains controversial. Despite favorable reports regarding its success, too few studies about indications, complications, and outcomes of CPM have been conducted to quell the controversy. Swallowing is accomplished when three primary conditions exist: (1) the cricopharyngeus muscle (CP) relaxes--that is, it is not tonically contracted, (2) the laryngo-hyoid complex elevates in an anterior-superior direction to open the sphincter, and (3) pharyngeal pressure is sufficient to propel a bolus through the open sphincter. CPM is indicated when the second and third conditions are "adequate" but the first is inadequate, thus resulting in pharyngeal dysphagia associated with a defective opening of the UES. UES dysfunction is determined most often through patient history, physical examination, and testing. Patients with Zenker's (pharyngoesophageal) diverticulum, oculopharyngeal dystrophy, or inclusion body myositis are among those reported to have the most positive responses to CPM. Modified barium swallow is the most common measurement of UES dysfunction; manometry also is used, but to a lesser degree because of catheter motion during swallowing. There are four approaches to CPM, including: (1) the external technique, which is indicated when a muscle biopsy or neck exploration is needed; (2) the endoscopic approach, which is reported to work best with patients with Zenker's diverticulum and offers the choice of electrocautery, laser, or the surgical stapler--the last option being the best choice for high-risk patients; (3) balloon dilatation of the UES, a low-risk option that reportedly works best in patients with fibrosis of the CP; and (4) botulinum toxin injection of the CP transcervically or endoscopically, which offers low risk and minimal or no anesthesia. This approach best serves cases of failed relaxation of the CP. Each approach has potential complications, but reports of such are few and rarely severe. In all cases, massive reflux should be controlled before CPM and the patient should be medically stable. Patient selection for CPM remains inadequate. To assess the efficacy of CPM, more multi-institutional outcome studies need to be conducted. In the meanwhile, clinical judgment and selective testing via modified barium swallow are the best methods for identifying patients who may derive the most benefit from CPM.
 ...
PMID:Management of upper esophageal sphincter disorders: indications and complications of myotomy. 1071 51

Hypertrophy of the cricopharyngeal muscle is a serious clinical condition that can cause severe dysphagic symptoms, including prolonged deglutition and postdeglutitive aspiration. Although the therapeutical concepts are well established, the pathogenic mechanism of cricopharyngeal hypertrophy remains unclear. We present a patient with a ten-year history of progressive dysphagia. The neurological and MRI findings were normal. However, videocineradiography showed severe hypertrophy of the cricopharyngeal muscle. This condition was first treated by injections of botulinum toxin, which did not alleviate the symptoms. Next, myotomy and muscle biopsy were performed. Histological evaluation disclosed lymphoplasmacellular florid myositis, single-fiber atrophy, and muscle fiber necrosis with phagocytosis. There were no signs of inclusion body myositis or oculopharyngeal muscular dystrophy. Our finding of severe cricopharyngeal muscle hypertrophy associated with myositis has been published previously (n = 34). The study presented here shows cricopharyngeal dysphagia associated with various systemic diseases, including motor neuron disease, general granulomatous disease, dermatomyositis, or inclusion body myositis. Isolated changes of the cricopharyngeal muscle were described in 65% of the cases.
Dysphagia 2001
PMID:Cricopharyngeal muscle hypertrophy associated with florid myositis. 1172 Mar 99

We present the case of a 71-year-old man with inclusion body myositis combined with subacute cutaneous lupus erythematosus and dysphagia. Although inclusion body myositis is usually resistant to immunosuppressive therapy, this patient improved under treatment with corticosteroids. The presented case is discussed in the context of earlier reports of inclusion body myositis and lupus erythematosus.
 ...
PMID:Association of inclusion body myositis with subacute cutaneous lupus erythematosus. 1173 63

Inclusion body myositis (IBM) is a primary inflammatory myopathy characterized by an older age at presentation. We describe four IBM cases fulfilling Mendell's diagnostic criteria. All patients were older than 60 years at diagnosis and the mean length of time from onset to diagnosis was 5.7 years. Two of them complained of leg weakness with unsteady gait and the other two, of upper limb weakness. Three patients had dysphagia, one of them had diaphragmatic paralysis and another had bilateral blepharoptosis. Histological sections of the muscle biopsy showed mononuclear cell invasion of nonnecrotic muscle fibers, rimmed vacuoles, intracellular amyloid deposits and 16-21 nm tubulofilaments by electron microscopy. Mitochondrial anomalies were found in two cases. Only one patient had transient response to steroid therapy. Our serie shows that clinical presentation of inclusion body myositis includes a broader spectrum than the classical description.
 ...
PMID:[Inclusion body myositis. Report of 4 cases]. 1196 48

Familial occurrence of inclusion body myositis is extremely rare, and only a few cases in Western countries have been reported. In these reports, a strong association of this disease with DR3 (DRB1*0301/0302) and the efficacy of immunosuppressants suggested that an immune pathomechanism is involved in the disease. We, for the first time, report two Japanese sisters who suffered myopathy clinicopathologically similar to inclusion body myositis. One sister received corticosteroid and azathioprine and the therapy relieved dysphagia. Both of our patients had DR15(2)/4 (DRB1*1502/0405), suggesting a distinct genetic association with the disease in the Japanese population.
 ...
PMID:Familial inclusion body myositis: a report on two Japanese sisters. 1460 2

Inclusion body myositis (IBM) is a progressive degenerative skeletal muscle disease leading to weakening and atrophy of both proximal and distal muscles. Dysphagia is reported in up to 86% of IBM patients. Surgical cricopharyngeal myotomy may be effective for cricopharyngeal dysphagia and there is one published report that botulinum toxin A, injected into the cricopharyngeus muscle using a hypopharyngoscope under general anesthesia, relieved IBM-associated dysphagia. This report presents the first documentation of botulinum toxin A injection into the upper esophageal sphincter using a flexible esophagogastroduodenoscope under conscious sedation, to reduce upper esophageal sphincter pressure and successfully alleviate oropharyngeal dysphagia in two IBM patients.
 ...
PMID:Injection of botulinum toxin A to the upper esophageal sphincter for oropharyngeal dysphagia in two patients with inclusion body myositis. 1519 Mar 96

The clinical features of inclusion body myositis (IBM) were of minor importance in the design of consensus diagnostic criteria, mainly because of controversial views on the specificity of signs and symptoms, although some authors reported "typical" signs. To re-assess the clinical spectrum of IBM, a single investigator using a standard protocol studied a cohort of 64 patients cross-sectionally. Symptom onset was before the age of 50 years in 20% of cases. Only a few patients (14 %) started with weakness other than that of quadriceps, finger flexor or pharyngeal muscles. The sequence of power loss was erratic, but onset of symptoms with quadriceps weakness predicted an earlier onset of dysphagia in older patients (> or = 56 years) compared with younger ones (< 56 years) (p = 0.02). Despite widespread weakness patients had favourable scores on three commonly used function scales and they kept their employment. Complete wheel-chair dependency was rare (3 %). A dominant characteristic was the anatomical distribution of afflicted muscles: ventral extremity muscle groups were more affected than dorsal muscle groups and girdle muscles were least affected, the latter preserving postural stability. Ankylosis, especially in extension of the fingers,was frequently present. Together with the sparing of intrinsic hand muscles it was helpful in the preservation of many skillful movements. IBM has a unique distribution of muscle weakness. Ankylotic contractures are common. We feel that their joint impact on daily functioning is characteristic for the disease.
 ...
PMID:Inclusion body myositis. Clinical features and clinical course of the disease in 64 patients. 1594 3

The case is reported of a patient with progressive proximal and distal weakness, dysphagia, respiratory weakness, calcifications, ptosis and ophthalmoparesis with inflammation, rimmed vacuoles and positive amyloid and ubiquitin on muscle biopsy. The histopathological features fit best with inclusion body myositis, but ophthalmoparesis and ptosis have not previously been described. The clinical phenotype fits best with hereditary inclusion body myopathy or distal-oculopharyngeal muscular dystrophy, but the degree of inflammation seen is unusual. None of these are associated with calcinosis.
 ...
PMID:Oculopharyngeal myopathy with inflammation and calcinosis: an unusual phenotype. 1827 Feb 38

Inclusion body myositis is an acquired inflammatory muscle disease belonging to the family of idiopathic inflammatory myopathy with vacuole formation. Approximately 15-28% of idiopathic inflammatory myopathy patients suffer from inclusion body myositis. Early diagnosis is very important due to the slowly progressive disease course and consecutive muscle atrophy. Inclusion body myositis is the most common chronic progressive muscle disease over the age of 50 years. Both degenerative processes including beta-amyloid accumulation and inflammatory processes, such as CD8 positive T-lymphocytes mediated cellular cytotoxicity take part in the pathomechanism of the inclusion body myositis. These findings are well demonstrated by the parallel presence of vacuolized muscle fibers rarely invaded by T cells and intact muscle fibers invaded by inflammatory T-cells in biopsy specimens. MHC-I/CD8 complex was introduced into the newly revised diagnostic criteria as a specific immune marker which helps to differentiate inclusion body myositis from aspecific inflammation present in other muscle dystrophies. Clinically both proximal and distal muscle weakness, respiratory muscle weakness and dysphagia are present. Interstitial lung disease is infrequent. Inclusion body myositis responds poorly to antiinflammatory treatment due to the predominant degenerative processes and it often results in only biochemical response instead of clinical. Diagnosis and differential diagnosis of inclusion body myositis are a very special challenge for the physician due to the diagnostic procedures which need immunohistochemical background. New therapeutic targets, monoclonal antibodies against the costimulatory molecules, anticytokine therapy may provide further improvement in the quality of life of inclusion body myositis patients.
 ...
PMID:[Inclusion body myositis pathomechanism and therapy]. 1862


<< Previous 1 2 3 4 5 6 Next >>