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)

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, causes a progressive wasting and loss of the upper and lower motor neurons that facilitate the movement of body parts. At onset, ALS patients may show symptoms such as muscle weakness, atrophy, hyperreflexia, or bulbar symptoms such as dysphagia or dysarthria. Deterioration progresses rapidly, and the later stages of ALS are characterized by severely limited mobility and respiratory failure, which is the primary cause of death. There is no specific diagnostic test for ALS, and there are a number of other conditions that may resemble ALS, making a diagnosis difficult. The variability of the initial presentation combined with the broad differential diagnosis may result in significant delays in diagnosis or, in some cases, misdiagnosis, which in turn have a negative impact on patient outcomes. There is no cure for ALS; however, many of the symptoms are treatable, and the physical and psychological symptoms are best managed through the efforts of a coordinated, multidisciplinary team. Nurses play a critical role in the clinical management of ALS and may be involved in coordinating the activities of the team, facilitating treatment, and helping patients and caregivers in making informed treatment and end-of-life decisions. Drug therapy for ALS is currently limited to riluzole; however, patients may be treated with a number of nonpharmacologic methods on the basis of their symptoms. A number of other treatment modalities, such as stem-cell-based therapy or gene therapy, and an array of neuroprotective clinical trials are currently under development for the treatment of ALS. Nurses may also have a key role in these various ALS studies.
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PMID:Clinical recognition and management of amyotrophic lateral sclerosis: the nurse's role. 2179 43

In late December 2010, a male resident of Wisconsin, aged 70 years, sought treatment for progressive right shoulder pain, tremors, abnormal behavior, and dysphagia at an emergency department (ED). He was admitted for observation and treated with benzodiazepines and haloperidol, a neuroleptic, for presumed alcohol withdrawal syndrome. The next day, he had rhabdomyolysis, fever, and rigidity, and neuroleptic malignant syndrome was diagnosed. The neuroleptic was discontinued, but the patient's clinical status worsened, with encephalopathy, respiratory failure, acute renal failure requiring hemodialysis, and episodes of cardiac arrest. With continued clinical deterioration, additional causes were considered, including rabies. On hospital day 12, rabies virus antigens and nucleic acid were detected in the nuchal skin biopsy and rabies virus nucleic acid in saliva specimens sent to CDC. A rabies virus variant associated with silver-haired bats (Lasionycteris noctivagans) was identified. The patient died on hospital day 13. His spouse reported that they had been selling firewood, and bats had been present in the woodpile; however, the man had not reported a bat bite. Two relatives and five health-care workers potentially exposed to the man's saliva received postexposure prophylaxis. This case highlights the variable presentations of rabies and the ease with which a diagnosis of rabies can be missed in a clinically challenging patient with comorbidities. Clinicians should consider rabies in the differential diagnosis for patients with progressive encephalitis or neurologic illness of unknown etiology and caregivers should take precautions to avoid exposure to body fluids. Continued public education regarding risks for rabies virus exposure during interactions with wildlife, particularly bats, is important.
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PMID:Human rabies--Wisconsin, 2010. 2188 47

Radiotherapy may lead to late-onset, rare, but sometimes life-threatening complications that need to be recognized for timely management. We report the case of a 39-year-old man who presented with a 20-kg weight loss with severe dysphagia and respiratory failure. His medical history was noticeable for Hodgkin's lymphoma that was treated 20 y previously. The physical examination and electroneuromyography indicated vagal and phrenic neuropathies. We concluded that the patient had late-onset esophageal motor disorder and bilateral phrenic paralysis secondary to the radiotherapy received 20 y previously for the lymphoma. The patient's management included long-term nutritional support. Although late-onset vagal and phrenic nerve injuries have been described separately after radiotherapy, we report the first case of paralysis at both sites. Another striking feature of this observation is the subsequent severe malnutrition that accompanied these paralyses.
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PMID:Late onset malnutrition from esophageal and phrenic dysfunction after radiotherapy for Hodgkin's lymphoma: a case report. 2196 95

Fusobacterium necrophorum is a non-spore-forming gram-negative anaerobic bacillus that may be the causative agent of localized or severe systemic infections. Systemic infections due to F.necrophorum are known as Lemierre's syndrome, postanginal sepsis or necrobacillosis. The most common clinical course of severe infections in humans is a progressive illness from tonsillitis to septicemia in previously healthy young adults. A septic thrombophlebitis arising from the tonsillar veins and extending into the internal jugular vein leads to septicemia and septic emboli contributing to the development of necrotic abscesses especially in lungs and other tissues such as liver, bone and joints. In this case report, a previously healthy man with pneumonia and empyema due to F.necrophorum has been presented. A 22 year-old man suffering from sore throat for seven days was admitted to emergency department with ongoing fever and dysphagia for three days. On admission he was already taking amoxicillin-clavulanic acid and his complaints were relieved with continuation of therapy to a total of 10 days. However, five days after the cessation of treatment he developed productive cough, fever and generalized myalgia. On physical examination, there were crackles on right lower lung, and chest X-ray revealed pulmonary consolidation on the right middle lobe. Levofloxacin therapy was started based on the diagnosis of pneumonia. While polymorphonuclear leucocytes and intracellular gram-negative bacilli were seen in Gram stained sputum smear, sputum culture was reported as normal flora. Although the patient's status had started to improve with treatment, his condition deteriorated with development of fever and dyspnea. Chest X-ray revealed consolidation, pulmonary infiltrates, pleural effusion and air-fluid level on the right. Meropenem, clarithromycin and linezolid were initiated and a chest tube was inserted with the preliminary diagnosis of necrotizing pneumonia, empyema and type-1 respiratory failure. While there was no growth on bronchoalveolar lavage fluid culture, thoracentesis material inoculated into thioglycolate broth revealed turbidity. Further inoculation onto Schaedler agar which was incubated under anaerobic conditions, yielded growth of catalase negative, indol positive, gram-negative anaerobic bacilli identified as F.necrophorum by BBL Crystal system (Becton Dickinson, USA). The detailed history of the patient revealed that fish bone had stuck in his throat a week ago. Clarithromycin and linezolid were discontinued and he was recovered within six weeks of meropenem treatment. F.necrophorum infection should be considered in the differential diagnosis of persistent head and neck infections with rapidly progressive metastatic necrotic lesions especially in healthy young adults and clindamycin or metranidazol should be added to the treatment protocols.
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PMID:[Pneumonia caused by Fusobacterium necrophorum: is Lemierre syndrome still current?]. 2209 Mar 4

Polymyositis is a systemic autoimmune disorder characterised by inflammatory myopathy of the skeletal muscles predominantly affecting the proximal muscles and associated with extra-muscular manifestations like dysphagia and skin involvement. In this case report, we describe the occurrence of diaphragmatic weakness and respiratory failure due to polymyositis with relatively well preserved power in limb muscles.
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PMID:Polymyositis presenting with respiratory failure. 2212 23

We present a rare cause of hypercapneic respiratory failure through this case report of a 72-year-old man presenting with progressive dyspnea and dysphagia over two years. Hypercapneic respiratory failure was acute on chronic in nature without an obvious etiology. Extensive workup for intrinsic pulmonary disease and neurologic causes were negative. Laryngoscopy and diagnostic imaging confirmed the diagnosis of diffuse idiopathic skeletal hyperostosis, also known as DISH, as the cause of upper airway obstruction leading to hypercapneic respiratory failure.
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PMID:An unusual cause of acute hypercapneic respiratory failure. 2217 94

Neurodegenerative diseases are often associated with life-threatening declines in respiratory and swallowing mechanisms. We report the case of a 70-year-old man who had postoperative dysphagia and respiratory failure that required reintubation after coronary artery bypass surgery. Impairment of the patient's speech, swallowing, and respiratory mechanisms identified during postoperative clinical and instrumental examinations was suggestive of a neurodegenerative disease. Genetic testing confirmed a diagnosis of spinal-bulbar muscular atrophy (Kennedy disease). This case report aims to highlight increased morbidity in patients with undiagnosed neuromuscular disorders in the critical care setting and the benefits of vigilant postoperative monitoring and multidisciplinary involvement throughout the care of complex patients.
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PMID:Complications after cardiovascular surgery in a case of undiagnosed spinal-bulbar muscular atrophy (Kennedy disease). 2238 92

Otolaryngologists commonly evaluate patients with findings suspicious for deep space soft tissue infections of the neck. In this case, a woman with a history of injection drug use (IDU) presented with dysphagia, odynophagia, and neck pain. Multiple neck abscesses, too small to drain, were seen on imaging. Despite broad-spectrum intravenous antibiotics, she unexpectedly and rapidly developed respiratory failure requiring intubation. Further work-up diagnosed wound botulism (WB). To our knowledge, this is the first report of WB presenting as a deep neck space infection, and illustrates the importance of considering this deadly diagnosis in patients with IDU history and bulbar symptoms.
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PMID:Wound botulism presenting as a deep neck space infection. 2264 53

Amyotrophic lateral sclerosis (ALS) is the most common form of progressive motor neuron disease and the most devastating neurodegenerative disorder. ALS is characterized by progressive paralysis and respiratory failure leading to death within 3 to 5 years after its onset. Protein-energy malnutrition is a frequent finding in ALS. The pathogenesis of protein-energy malnutrition in ALS is multifactorial. Muscle atrophy, hypophagia, dysphagia, and hypermetabolism play a role in determining the deterioration of nutritional status. A multidisciplinary approach is crucial to set an appropriate plan for metabolic and nutritional support in ALS. Nutritional management incorporates a continuous assessment and implementation of dietary modifications throughout the duration of the disease. The nutritional and metabolic approaches to ALS should start when the diagnosis of ALS is made and should become an integral part of the continuous care to the patient, including nutritional surveillance, dietary counseling, management of dysphagia, and enteral nutrition when needed. Parenteral nutrition is rarely indicated. Standard polymeric enteral formulas are routinely used, usually providing 25 to 30 kcal/kg and protein 0.8 to 1.2 g /kg per day. The use of fiber-enriched formulas may help prevent constipation. However, considering the complex metabolic abnormalities of ALS, standard and/or fiber-enriched formulas might not be sufficient to achieve optimal metabolic and nutritional support. Based on the most recent clinical and experimental evidence, it is tempting to hypothesize that personalized nutritional support including specific nutritional substrates could act on disease progression and improve the quality of life and the response to the few and yet scarcely effective, currently available pharmacologic therapies.
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PMID:Nutritional and metabolic support in patients with amyotrophic lateral sclerosis. 2267 56

Thoracic aortic aneurysms are life threatening because of the risk of rupture. Moreover, aneurysm enlargement can lead to additional complications, including bronchial and esophageal obstruction. We report an 80-year-old man with a 7-cm diameter thoracic aortic aneurysm resulting in near-complete left main bronchial obstruction and significant dysphagia. He had a number of intensive care unit admissions for respiratory failure and had lost more than 10 kilograms. Under spinal anesthesia, he underwent endovascular thoracic aortic aneurysm repair. Postoperatively, he had left main bronchial total obstruction treated with a bronchial stent. He then recovered uneventfully and was discharged to home.
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PMID:Endobronchial and endovascular management of bronchial compression by a thoracic aortic aneurysm. 2273 93


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