1/82. Pulmonary manifestations of poems syndrome: case report and literature review.phrenic nerve paresis is an unusual complication of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein spike and skin changes) syndrome. In this report, we describe a case of poems syndrome in which a 56-year-old woman presented with dyspnea and ventilatory failure due to bilateral phrenic nerve paralysis. To our knowledge, only one other case of phrenic neuropathy in poems syndrome has been reported.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
2/82. Inspiratory pressure threshold training for glottal airway limitation in laryngeal papilloma.A single-subject design was used to determine if inspiratory pressure threshold training increases inspiratory muscle strength and reduces the sensation of dyspnea during exercise and speech. The subject was a 23-year-old female with congenital juvenile papilloma which has been in remission for 10 years. A 4-week inspiratory muscle training program was implemented using an inspiratory pressure threshold trainer. The pressure threshold of the trainer was set by the experimenter. The pressure threshold setting of the trainer was based on a percentage of the subject's maximum inspiratory pressure measured prior to training. The average range of the pressure threshold was 40 to 70 cmH2O. In order for inspiratory air to flow, the subject generated inspiratory pressure, independent of airflow rate. Maximum inspiratory pressure (MIP) was the dependent variable used as the index of inspiratory muscle strength. exercise dyspnea was a dependent variable rated by the subject during a progressive treadmill test. dyspnea associated with speech was rated following production of a comfortable and loud speech task. MIP increased by 57% following the training program with a 2-scale point reduction in the perception of dyspnea during exercise. dyspnea during loud speech decreased from moderate to mild. The changes in dyspnea, both during exercise and speech, are directly related to inspiratory muscle strengthening. The results suggest that inspiratory muscle training may improve respiratory related function in patients with restrictive upper airway disorders.- - - - - - - - - - ranking = 0.025881746472095keywords = muscle (Clic here for more details about this article) |
3/82. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by peribronchial, perivascular, and perilymphatic proliferation of abnormal smooth muscle cells leading to cystic lesions. The hypothesis of hormonal dependence and the effectiveness of hormonal therapy have not yet been demonstrated conclusively, and the prevalence of extrathoracic manifestations and the survival of patients with LAM are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features, the diagnostic procedures, the associated lesions, and, above all, the management and course of LAM in a large homogeneous series of 69 stringently selected patients, with a majority of cases diagnosed since 1990. The aim of the study, based on a review of the literature, also was to provide a comprehensive view of this uncommon disease. The clinical features were in keeping with previous studies, but we found that exertional dyspnea and pneumothorax were the most common features, and chylous involvement was less frequent. LAM was diagnosed after menopause in about 10% of cases. The onset of LAM occurred during pregnancy in 20% of cases, and a clear exacerbation of LAM was observed in 14% of cases during pregnancy. Pulmonary LAM was diagnosed on lung histopathology in 83% of cases, but renal angiomyolipoma, observed in 32% of our patients, may be a useful diagnostic criterion when associated with typical multiple cysts on chest CT scan or with chylous effusion. Chest CT scan was more informative than chest X-ray (normal in 9% of cases), and may be indicated in spontaneous pneumothorax or renal angiomyolipoma in women of childbearing age. About 40% of the patients had a normal initial spirometry, while an obstructive ventilatory defect (44%), a restrictive ventilatory defect (23%), was observed in other patients. Initial diffusing capacity for carbon monoxide was frequently decreased (82%). Hormonal therapy was administered in 57 patients, but a clear > or = 15% improvement of FEV1 was observed in only 4 evaluable patients, treated with tamoxifen and progestogens (n = 2), progestogen (n = 1), and oophorectomy (n = 1). Probably 1 of the most urgent needs for clinical research in LAM is to test the currently available hormonal treatments in the context of international multicenter prospective controlled studies. pleurodesis was performed in 40 patients. lung transplantation was performed in 13 patients, 7.8 /- 5.2 years after onset of LAM, in whom the mean FEV1 was 0.57 /- 0.15 L. After a follow-up of 2.3 /- 2.2 years, 9 patients were alive. Mean follow-up from onset of disease to either death or closing date was 8.2 /- 6.3 years. overall survival was better than usually reported in LAM, and Kaplan-Meier plot showed survival probabilities of 91% after 5 years, 79% after 10 years, and 71% after 15 years of disease duration.- - - - - - - - - - ranking = 0.005176349294419keywords = muscle (Clic here for more details about this article) |
4/82. cricoid cartilage necrosis after arytenoidectomy in a previously irradiated larynx.Several open and endoscopic surgical techniques are available to provide an adequate airway for patients with bilateral vocal cord paralysis. Transoral laser arytenoidectomy has repeatedly been reported to be a reliable and effective minimally invasive procedure for airway restoration. To our knowledge, there have been no previous reports of serious complications, other than poor vocal results, aspiration, and failed decannulation in individual patients, that have resulted from this intervention. We report a case in which arytenoidectomy led to severe complications and death. Prior irradiation is suspected to be a causative factor. To prevent such an outcome, we believe that operative settings should be chosen that avoid deep thermal injury of the laryngeal framework.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
5/82. Rapidly progressive polymyositis with elevated antiacetylcholine receptor antibody activity.We report a 51-year-old woman with polymyositis accompanied by a high titer of antiacetylcholine receptor antibody. The patient presented with weakness of grip strength followed by rapidly progressive dyspnea, which required mechanical ventilation. She was treated with a glucocorticoid and came off the respirator one week later. Antiacetylcholine receptor antibody activity was elevated in the acute phase and decreased during recovery, although other signs of myasthenia gravis were negative. This patient suggested that in cases of rapidly progressive bulbar palsy and limb muscle weakness, it is necessary to include polymyositis associated with elevated antiacetylcholine receptor antibody activity in the differential diagnosis.- - - - - - - - - - ranking = 0.005176349294419keywords = muscle (Clic here for more details about this article) |
6/82. Perspectives on care at the close of life. Management of dyspnea in patients with far-advanced lung disease: "once I lose it, it's kind of hard to catch it... ".dyspnea is a common problem among patients with interstitial fibrosis, lung cancer, cystic fibrosis, and chronic obstructive pulmonary disease. The slow but steady progression of such diseases, often punctuated by acute exacerbations or secondary illnesses, can lead to decision-making dilemmas among patients and their caregivers, such as when to accept mechanical ventilation, when to forgoe aggressive therapies, and when to make formal end-of-life care plans. Two cases, a 74-year-old woman with dyspnea secondary to emphysema and a 65-year-old woman with recurrent lung cancer and severe exertional fatigue and dyspnea, illustrate how dyspneic patients approaching the end of life can be evaluated and treated. Four management strategies for dyspnea are discussed: reducing ventilatory impedance, reducing ventilatory demand, improving respiratory muscle function, and altering central perception. physicians should encourage end-stage lung disease patients and their families to discuss issues such as hospitalization and mechanical ventilation, to prepare advance directives, and to participate in a plan to manage their dyspnea.- - - - - - - - - - ranking = 40.420542101126keywords = respiratory muscle, muscle (Clic here for more details about this article) |
7/82. meige syndrome secondary to basal ganglia injury: a potential cause of acute respiratory distress.BACKGROUND: meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on meige syndrome and the relationship between dystonias and respiratory compromise is presented. methods: A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. RESULTS: Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated. CONCLUSION: This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.- - - - - - - - - - ranking = 0.005176349294419keywords = muscle (Clic here for more details about this article) |
8/82. Symptomatic palatal myoclonus: an unusual cause of respiratory difficulty.A 67-year-old man presented with dysphagia and difficulty breathing. physical examination revealed palatal myoclonus. In this patient, the respiratory difficulty was caused by the fragmentation of breathing. Electromyographic examination of the cricothyroid muscle demonstrated rhythmic myoclonic jerks. magnetic resonance imaging (MRI) yielded a pontine midline and right sided tegmental infarct. The patient responded to sodium valproate.- - - - - - - - - - ranking = 0.005176349294419keywords = muscle (Clic here for more details about this article) |
9/82. myotonic dystrophy type 1 (DM1) presenting with laryngeal stridor and vocal fold paresis.myotonic dystrophy type 1 (DM1) is the most common inherited muscle disorder and may present in numerous ways due to characteristic multisystem involvement. We report a 47-year-old man who presented with an 8-year history of slowly progressive dyspnea and episodic stridor. The laryngeal paresis was documented with videostroboscopy and laryngeal electromyography, and treated with tracheostomy and antimyotonia agents.- - - - - - - - - - ranking = 0.005176349294419keywords = muscle (Clic here for more details about this article) |
10/82. Idiopathic bilateral diaphragmatic paralysis.A 41-year-old man complained of subacute onset of dyspnea and pain in the neck and chest. He was diagnosed with bilateral diaphragmatic paralysis, based on clinical inspection of the breathing pattern and transdiaphragmatic pressure recording, and was trained to use a portable bi-level positive airway pressure apparatus (BiPAP). Needle electromyography showed profuse fibrillation potentials and positive waves in the diaphragm, more abundant on the right than left side, and no response to phrenic nerve stimulation. Other muscles were not involved. Follow-up examinations, performed at 9 and 12 months after onset of paralysis, demonstrated a slow but progressive improvement of the patient's respiratory function, together with the appearance of reinnervation potentials in the diaphragm, and polyphasic, long-latency responses to phrenic nerve stimulation. The subacute onset of the paralysis associated with local pain, and its subsequent recovery, suggest bilateral proximal lesions in the phrenic nerves. In the absence of traumatic or metabolic causes, these findings suggest that the phrenic nerve can be a target in idiopathic neuritis.- - - - - - - - - - ranking = 356.44612473188keywords = diaphragmatic paralysis, paralysis, muscle (Clic here for more details about this article) |
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