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)

We report a 56-year-old woman with parkinsonism and dementia who died of respiratory failure. The patient was well until the age of 41 when she noted insidious onset of difficulty in moving around. Soon after, she noted tremor in both her hands and gait disturbance. She received stereotaxic right thalamotomy when she was 46-year-old; after thalamotomy, improvement was noted in her tremor, rigidity, and in gait. However, a few months later, she started to experience motor fluctuations with worsening of her symptoms in the afternoon. This worsening was temporarily relieved by increasing her levodopa/benserazide dose. She started to show visual hallucination and agitation when she was 54-year-old. Her symptoms had progressively become worse with marked motor fluctuations and she was admitted to our hospital when she was 56-year-old. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed that she was disoriented to time and place; memory was markedly disturbed and calculation was poor. Hasegawa dementia scale was 7/30. Higher cerebral functions appeared intact. She showed masked face, small voice, and some dysphagia. Other cranial nerves were intact including ocular movements. She was unable to walk by herself; when supported she walked in small steps with marked disturbance in the righting reflex. Mixed rigidity and Gegenhalten was noted in her four limbs and in the neck. Tremor was absent. She showed marked akinesia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A 56-year-old woman with parkinsonism and dementia with the age of onset at 41 years]. 754 42

Massive hemoptysis and/or recurrent expectoration of measurable amounts of blood are common complications of chronic bronchopulmonary infections in cystic fibrosis (CF). When conservative treatment fails to control bleeding, surgery or bronchial artery embolization (BAE) is frequently considered. We present our experience and long-term follow up of BAE in 14 CF patients (age range 15-39 years) with massive (6 subjects) and/or recurrent (8 subjects) hemoptysis not responsive to medical treatment. Seven had chronic hypercapnic respiratory failure. After angiographic evaluation, polyvinyl alcohol particles (Ivalon) were injected to embolize obviously enlarged bronchial arteries. Seventeen procedures were performed in 14 patients and 36 bronchial arteries were embolized. All the patients stopped bleeding immediately upon BAE. Most of the patients had postembolization fever, dysphagia, and transient chest pain which were managed symptomatically. After a median follow-up period of 10.5 months (range 0.5-38 months), no recurrence of hemoptysis was observed in 8 patients who are still alive. In 3 patients hemoptysis recurred and they underwent reembolization after 3, 22, and 25 months, respectively. Three subjects died of respiratory failure within 5 months from BAE. Presently, 50% of patients studied had a > or = 1 year interval free of major hemoptysis after the first BAE. Our experience indicates that massive and/or recurrent hemoptysis in CF patients can be safety and effectively managed by BAE if the procedure is performed by a skilled practitioner. The procedure was well tolerated and resulted in prolonged and satisfactory bleeding control in most patients.
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PMID:Bronchial artery embolization in the management of hemoptysis in cystic fibrosis. 756 13

Between 1985 and 1993, 32 patients (24 male and 8 female) underwent colon interposition for replacement of the esophagus at the Mayo Clinic. Median age was 58.5 years (range, 1 to 79 years). The colon was used because of an inadequate stomach in 27 patients (84%) and as the conduit of choice in 5 (16%). Esophageal cancer was present in 15 patients (47%). The left colon was used in 20 patients (63%) and the right, in 12 (38%). The colon was placed substernally in 19 patients (59%) and in the esophageal bed in 13 (41%). The operative mortality was 9%; cause of death was ischemic necrosis of right colon conduits in 2 patients and adult respiratory distress syndrome in 1 patient. Major complications occurred in 4 additional patients and included ischemic colitis of a right colon conduit, Roux-en-Y limb obstruction, chylothorax, and an anastomotic leak. Follow-up was complete for all patients and ranged from 15 months to 7 years (median follow-up, 2.3 years). Eleven patients died during follow-up. The cause of death was metastatic esophageal cancer in 9 patients, myocardial infarction in 1 patient, and respiratory failure in 1 patient. At last follow-up, 26 of the 29 operative survivors had little or no difficulty eating. Two patients had dumping symptoms, and 1 patient had severe dysphagia. Seven patients required dilation of the esophagocolonic anastomosis. We conclude that colon interposition for esophageal replacement provides acceptable long-term function; however, early morbidity and mortality are considerable.
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PMID:Esophageal replacement by colon interposition. 777 14

Central alveolar hypoventilation syndrome (CAH), or Ondine's curse, is a very rare disease characterized by dysfunction of respiratory center in the brain stem. Here, we report a case of CAH associated with cerebral infarction. A 59-year-old man developed right facial sensory deficit at age 56. Then, the facial sensory deficit spread to the left side and dysarthria and dysphagia also developed. Since age 58, he often developed respiratory failure and consciousness disturbance. Arterial blood gas analysis revealed alveolar hypoventilation and respiratory acidosis. Disorders of peripheral organs such as lung, airway, thorax and neuromuscular diseases were ruled out. Brain MRI showed cerebral infarction in the brain stem. We diagnosed him as CAH associated with brain stem infarction.
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PMID:[A case of central alveolar hypoventilation syndrome associated with cerebral infarction]. 832 22

We describe the clinical course of two cases of envenoming by the many-banded krait (Bungarus multicinctus). A man developed generalized paralysis and respiratory failure with transient hypertension. Nerve conduction studies revealed normal motor and sensory conduction velocities with reduced motor unit action potential amplitudes consistent with neuromuscular blockade. He showed a slight transient response to the banded krait (B. fasciatus) antivenom but required ventilatory support for 8 days. After the fourth day, there was some response to treatment with anticholinesterase. Another man developed diplopia, dysphagia and leg weakness but recovered spontaneously after 48 hours.
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PMID:Evenoming by Bungarus multicinctus (many-banded krait) in Hong Kong. 854 31

Although the genetic basis of myotonic dystrophy (MD) has recently been clarified, data on prognosis and causes of death are few. To assess the relation between the age of onset and the degree to which activities of daily living (ADL) were affected, we studied statistically 586 patients with MD, who were registered in the database in hospitals of Japan in 1985. Many of the young onset patients had a long duration of illness, and many of the long-term patients showed the more severely affected ADL. When we adjusted the duration of illness, using the Mantel extension method, many patients whose age of onset was older had poorer ADL than the younger onset ones. To clarify the causes of death in MD, we collected data on 72 patients who succumbed to it from answers to questionnaires sent to all councilors of the Japanese Society of Neurology and the Japanese Society of Child Neurology in 1994. Before their deaths, among 32 MD cases unable to sit up, 16 (50%) died from respiratory involvement (respiratory failure and pneumonia), 8 (25%) from dysphagia (i.e., aspiration pneumonia and choking) and 2 (6%) from cardiac involvement. Moreover, among 25 cases that could sit up, 9 cases (36%) died from dysphagia, 4 (15%) from respiratory involvement and 2 (8%) from cardiac involvement. Dysphagia and cardiac involvement are often the causes of death in the early stage. To avoid these complications, management of swallowing and careful cardiac control is essential in patients with MD.
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PMID:[Prognosis for myotonic dystrophy]. 875 40

Noninvasive positive pressure ventilation (NPPV) is used for respiratory support in a number of diseases causing acute or chronic respiratory failure. We describe a novel use of NPPV to provide respiratory support during sedation for percutaneous placement of a gastrostomy tube in a patient with Duchenne muscular dystrophy (DMD). The patient had severe respiratory insufficiency, progressive dysphagia, and undernutrition. In addition to the case in this report, we have used NPPV to provide respiratory support to DMD patients during five other gastrointestinal endoscopies without complication. The technique is highly labor intensive and requires physicians and respiratory therapists familiar with NPPV. The primary risk associated with this technique is lack of definitive airway protection during the procedure, which must be balanced against the risks of intubation in an anesthetized patient with neuromuscular disease. The potential benefit to selected patients is substantial, such as initiation of gastrostomy tube feeding in our patient, with subsequent improvement in his quality of life and nutritional status.
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PMID:Noninvasive ventilation during percutaneous gastrostomy placement in Duchenne muscular dystrophy. 922 May 32

The incidence of wound botulism is increasing and the epidemiology of the disease is changing. The majority of new cases are associated with injection drug use, in particular, the use of Mexican black tar heroin. This case report and discussion of wound botulism illustrate the following important points: Dysphagia, dysphonia, diplopia, and descending paralysis, in association with injection drug use, should alert the treating physician to the possibility of wound botulism. In such patients, the onset of respiratory failure may be sudden and without clinically obvious signs of respiratory weakness. For the reported patient, maximum inspiratory force measurements were the only reliable indicator of respiratory muscle weakness. This is a measurement not routinely performed in the ED, but may prove essential for patients with suspected wound botulism. To minimize the effect of the botulinum toxin and to decrease length of hospital stay, antitoxin administration and surgical wound debridement should be performed early.
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PMID:Wound botulism associated with black tar heroin. 926 1

We report a 49-year-old man with progressive bulbar palsy and respiratory failure. He was well until his 48 years of the age (December 1994) when he noted a difficulty in speaking in loud voice. In February, 1995, he noted regurgitation of foods to his nose and difficulty in his speech. He was admitted to our service in May 29, 1995. On admission, he was alert and oriented to all spheres and he was not demented. His higher cerebral functions were normal. In cranial nerves, he showed dysarthria and dysphagia; muscle atrophies were seen in the tongue, the bilateral sternocleidomastoid, supraspinatus, and infraspinatus muscles. Fasciculations were seen in these muscles. He showed no muscle weakness in his limbs except for the upper limb girdle muscles, no ataxia, no reflex abnormalities, nor sensory changes. EMG showed neurogenic changes in the affected muscles. MRI of the brain and the spinal cord was entirely normal. He was discharged for out patient follow-up, however, in October of 1995, he noted difficulty in swallowing solid foods. Gastrostomy was placed and he was discharged to his home. In February 11th of 1996, he was found unresponsive and brought into the ER of our hospital. On admission, he was comatose without spontaneous respiration. BP could not be obtained. He was immediately intubated and artificial ventilation was started. On the following morning, he became alert and he was not demented. He continued to show marked dysarthria and dysphagia; again no weakness was noted in the distal parts of the upper and lower extremities. Laboratory examination showed increase in serum CK to 2,173 IU/L and amylase to 2,032 IU/L. He was extubated on February 15th, however, his spontaneous respiration was not suffice to maintain his blood gas. According to his will, he was not placed on respirator and he died on February 24th, 1996. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had ALS. Although no upper neuron signs were observed clinically, it is not uncommon to see degeneration in the corticospinal tract in post-mortem examination. The question was what might have been the cause of increase in CK and amylase. Many participants thought that they were secondary to multiple organ failure due to prolonged hypoxic state at his last admission; other possibilities raised included acute myocardial infarction and acute bowel necrosis. Post-mortem examination revealed muscle atrophy in the facial, lingual, cervical, intercostal, and the upper limb girdle areas. The lungs were unremarkable except for old organized pneumonic foci in the right middle and lower lobes. Marked to moderate congestion was seen in many internal organs, however, no other gross abnormality was found. It was thought that respiratory palsy itself was the direct cause of his agonal event. In the spinal cord, the anterior horns showed various degree of neuronal loss and gliosis. No clear evidence of pyramidal tract degeneration was seen at the light microscope level. Lower brain stem motor neurons were markedly reduced. But no Bunina body was found. The substantia nigra showed moderate degree of neuronal loss and extraneuronal neuromelanins. The locus coeruleus showed similar but milder changes. The degree of nigral degeneration appeared to be well beyond those which could be seen in usual ALS patients. The question was whether or not this patient might have been in an early stage of the extended form of ALS.
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PMID:[A 49-year-old man with progressive bulbar palsy and respiratory failure]. 949 5

Although respiratory failure is the primary cause of death in patients with amyotrophic lateral sclerosis (ALS), the management of nutritional status is important to enhancing the quality of life and optimising the timing of interventive techniques. Progressively weakening muscles impair the patient's ability to eat, and nearly all patients with ALS develop severe dysphagia. If nutritional support is not provided, food and fluid consumption may be greatly restricted, leading to weight loss and malnutrition. This may be compounded by impaired respiratory functions, which place increased energy demands on the patient. This paper describes the nutritional needs of ALS patients from a worldwide and cross-cultural perspective. In particular, the differences between a paternalistic and a patient-centred approach to treatment are addressed. The need for further study into the nutritional status of ALS patients and the issue of parenteral and enteral nutritional therapy, particularly percutaneous endoscopic gastrostomy, are discussed.
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PMID:Nutritional management in amyotrophic lateral sclerosis: a worldwide perspective. 974 29


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