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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with inclusion body myositis (IBM) is presented. Unusual aspects of this case include a myopathy of 36 years duration, severe dysphagia due to cricopharyngeus muscle dysfunction, improvement with cricopharyngeus myotomy, and a diagnostic cricopharyngeus muscle biopsy.
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PMID:Inclusion body myositis with cricopharyngeus muscle involvement and severe dysphagia. 131 Jan 56

We report the case of a patient who presented with symptoms of dysphagia, muscle weakness, and photophobia. The diagnosis of sarcoidosis was made by the histologic evidence of widespread noncaseating epithelioid cell granulomas in more than one organ (anterior mediastinal node and right quadriceps muscle). The disease was proven to be active by elevated angiotensin-converting enzyme (ACE) level and gallium-67 imaging. Esophageal dysfunction was demonstrated by barium swallow and manometric study. A review of the literature on sarcoidosis involving the esophagus and the muscular system is presented. Dysphagia and acute symptomatic myopathy are rare presentations of sarcoidosis. The combination of symptoms is unique, and clearly demonstrates the protean multisystemic nature of sarcoidosis. Patients presenting with dysphagia and myopathy should be investigated for sarcoid granulomas in these organs for appropriate treatment.
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PMID:Sarcoidosis: a unique presentation of dysphagia, myopathy, and photophobia. 195 Dec 51

Nineteen cases are described, including 12 cases from three different families and 7 nonfamilial cases, in which multisystem neurological disease was associated with acanthocytosis in peripheral blood and normal plasma lipoproteins. Mild acanthocytosis can easily be overlooked, and scanning electron microscopy may be helpful. Some neurologically asymptomatic relatives with significant acanthocytosis were identified during family screening, including some who were clinically affected. The mean age of onset was 32 (range 8-62) yrs and the clinical course was usually progressive but there was marked phenotypic variation. Cognitive impairment, psychiatric features and organic personality change occurred in over half the cases, and more than one-third had seizures. Orofaciolingual involuntary movements and pseudobulbar disturbance commonly caused dysphagia and dysarthria that was sometimes severe, but biting of the lips or tongue was rarely seen. Chorea was seen in almost all symptomatic cases but dystonia, tics, involuntary vocalizations and akinetic-rigid features also occurred. Two cases had no movement disorder at all. Computerized tomography often demonstrated cerebral atrophy. Caudate atrophy was seen less commonly, and nonspecific focal and symmetric signal abnormalities from the caudate or lentiform nuclei were seen by magnetic resonance imaging in 3 out of 4 cases. Depression or absence of tendon reflexes was noted in 13 cases and neurophysiological abnormalities often indicated an axonal neuropathy. Sural nerve biopsies from 3 cases showed evidence of a chronic axonal neuropathy with prominent regenerative activity, predominantly affecting the large diameter myelinated fibres. Serum creatine kinase activity was increased in 11 cases but without clinical evidence of a myopathy. Postmortem neuropathological examination in 1 case revealed extensive neuronal loss and gliosis affecting the corpus striatum, pallidum, and the substantia nigra, especially the pars reticulata. The cerebral cortex appeared spared and the spinal cord showed no evidence of anterior horn cell loss. Two examples of the McLeod phenotype, an X-linked abnormality of expression of Kell blood group antigens, were identified in a single family and included 1 female. The genetics of neuroacanthocytosis are unclear and probably heterogeneous, but the available pedigree data and the association with the McLeod phenotype suggest that there may be a locus for this disorder on the short arm of the X chromosome.
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PMID:Neuroacanthocytosis. A clinical, haematological and pathological study of 19 cases. 199 79

Swallowing disorders have been studied in seven patients with oculopharyngeal myopathy. All patients presented with symptoms dominated by solid food dysphagia and false passages. Two of them had a considerably deteriorated general and pulmonary condition. Manometry demonstrates a decrease in pharyngeal populsion in six patients but does not detect any anomaly in the function of the upper sphincter of the esophagus. Three patients were operated with myotomy of the cricopharyngeus. The procedure led to the disappearance of the disorders in two of them, while it was a failure in the third. Swallowing disorders, the difficulties of manometric exploration and the role of myotomy are discussed in this article.
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PMID:[Dysphagia in oculopharyngeal myopathies. Report of 7 cases]. 208 57

We report a case of a woman of Mexican origin with oculopharyngeal muscular dystrophy (OMD). This is the first OMD reported in Mexico. She was healthy until the age of 30, when she noticed slowly progressive ptosis and dysphagia. She developed dermatitis and polyneuritis which we attribute to a deficiency of nutrients due to her dysphagia. In contrast to most previous reported cases this patient had also a distal myopathy. It is recommended in this type of patients a strict dietary control in order to avoid complications. It is also recommended to perform biopsies of several muscles to complete the diagnosis and prognosis.
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PMID:[Oculopharyngeal muscular dystrophy: 1st report in Mexico]. 223 75

A 68-year-old man known to have inclusion body myositis underwent a cricopharyngeal myotomy in an attempt to improve his progressive dysphagia. Morphological studies from tissues obtained during this procedure showed the diagnostic features typical of this chronic inflammatory myopathy. To our knowledge this is the first pathological demonstration of inclusion body myositis involving the pharyngeal skeletal musculature.
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PMID:Inclusion body myositis associated with progressive dysphagia: treatment with cricopharyngeal myotomy. 255 29

This report deals with a middle aged man in whom the presenting symptom of the disorder was dysphagia. The clinical approach to the final diagnosis of thyrotoxic myopathy causing dysphagia is outlined and the pathophysiology of dysphagia then discussed. The need to include thyrotoxicosis in the differential diagnosis of an otherwise unexplained case of dysphagia is stressed.
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PMID:Dysphagia as a primary manifestation of thyrotoxicosis: a case report. 262 17

Tubular aggregates (TA) are unusual intramuscular structures stained basophilic on hematoxilin and eosin (HE) staining and red on modified Gomori trichrome (GT) staining. The structures are said to be originated from sarcoplasmic reticulum and are collections of tubules with double membranes on electron microscopic studies. The TA are usually seen in biopsy muscles from patients with muscle pain and cramps but without muscle weakness, periodic paralysis or alcoholic myopathy. In addition, there are five reports on families with progressive myopathy and tubular aggregates in the literature. We presented here a 48-year-old postman without any family history, who had had progressive muscle weakness for 17 years. He had never noticed pain or cramps in his muscles, not taken any particular medicine, and not had regular alcoholic beverages. There was no ptosis, facial weakness, masticatory muscle weakness or dysphagia. Muscle wasting, started from the proximal part of four extremities had progressed to the distal part of them. He could not walk on heels or toes and walked with waddling gait. He stood up with Gowers' maneuver. Serum GOT, GPT and CK were elevated. EMG showed myogenic pattern and MCV was normal. The muscle biopsies were performed; the first one taken from quadriceps femoris muscle at 42 years old showed myopathic changes including marked variation in fiber sizes, with scattered necrotic fiber splitting and TA in type 2B fibers. The second biopsy from biceps brachii muscle at the age of 48 years, showed densely proliferated fibrous tissues, marked variation fiber sizes and scattered split fibers. The TA were rarely seen and type 2B fibers were decreased in number.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of progressive myopathy with tubular aggregates]. 268 70

An infant presented with congenital hypotonia, poor sucking, apathy, and areflexia. Muscle biopsy at two months of age revealed numerous nemaline rods, suggesting congenital nemaline myopathy. During the ensuing months, familial dysautonomia was suggested by recurrent pulmonary infections, dysphagia, alacrima, hyperhydrosis, emotional lability, and unexplained episodes of hyperthermia and breath-holding spells. The diagnosis was confirmed by positive intradermal histamine and ocular mecholyl tests. The finding of nemaline rods adds a new facet to the recognized polymorphic presentation of familial dysautonomia.
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PMID:Familial dysautonomia manifesting as neonatal nemaline myopathy. 285 35

We describe a patient who had scleromyxedema associated with proximal myopathy. The histopathologic and electron microscopic features are presented. In addition, we review the clinical and pathologic features of all 9 previously reported patients with documented scleromyxedema and myopathy. Proximal (pharyngeal) or distal dysphagia (7 of 7 patients), elevated creatine kinase (5 of 8), elevated aldolase (3 of 3), and electromyographically demonstrated myopathy (7 of 7) were usual features. Four patients had muscle biopsies that showed myofibril vacuolar changes, but inflammation was infrequent (2 cases). Our patient responded to oral prednisone and weekly intravenous methotrexate with improvement of the erythroderma, papular rash, and muscle strength.
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PMID:Scleromyxedema myopathy: case report and review of the literature. 305 23


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