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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyotrophic lateral sclerosis is a progressive dengenerative neuromuscular disease of insidious onset. It involves upper and lower motor neurons and causes both spastic and atrophic muscular symptoms. More than one fourth of patients have complaints relating to the head and neck (bulbar palsy); thus, the otolaryngologist may be the first physician to see them. Predominant symptoms are slurred speech, hoarseness, dysphagia, and dyspnea. Muscular weakness, atrophy, and fasciculation are noted on examination. The course is relentless, and only 20% of patients survive five years after diagnosis.
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PMID:The otolaryngologic presentation of amyotrophic lateral sclerosis. 11 40

A polygraphic study of nocturnal sleep was carried out on 12 patients suffering from amyotrophic lateral sclerosis (ALS), 6 patients suffering from olivio-ponto-cerebellare-atrophia (OPCA) and 9 patients suffering from the Steele-Richardson disease (SR). A disturbance of sleep--if it exists--always runs parallel with the course of the disease. No disturbances were registered in the group of patients suffering from ALS, in the OPCA group a specific disease of paradoxal sleep is observed. The sleep disturbances are more global in the group of patients suffering from the SR disease. A particular electrooculogram was taken from the patients from the awakening and from the paradoxal sleep. Respiratory difficulties were registered on certain patients of each of the three groups. These sleep disturbances, registered by the polygraph, were compared with those observed on patients presenting a cortical disease.
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PMID:[Polygraphic study of nocturnal sleep in three degenerative diseases: ALS, oligo-ponto-cerebellar atrophy, and progressive supranuclear palsy]. 22 77

To confirm our impression that a high percentage of patients with amyotrophic lateral sclerosis are initially misdiagnosed, we reviewed records of 33 patients with a definitive diagnosis of amyotrophic lateral sclerosis seen over 10 years. Fourteen patients (43%) were initially misdiagnosed. Mean time to correct diagnosis was significantly greater for the misdiagnosed group (16.0 +/- 9.3 months) than for the rest of the patients (7.6 +/- 4.1 months). Two of three patients with an initial symptom of dyspnea were misdiagnosed. Three patients underwent laminectomies because of misdiagnosis. Age, stage of disease, and unusual presenting symptoms were not identified as causes of misdiagnosis. Most likely causes were physicians' failure to consider the diagnosis and lack of familiarity with the common clinical presentations of amyotrophic lateral sclerosis. Earlier diagnosis of amyotrophic lateral sclerosis may help prevent medical mismanagement and may benefit patients both medically and psychologically.
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PMID:Misdiagnosis in patients with amyotrophic lateral sclerosis. 224 38

In myasthenia gravis and amyotrophic lateral sclerosis the ENT specialist or the phoniatrician may be consulted first, because in about 30 percent of all cases the initial symptoms are dysarthria, dysphagia or dyspnea. Three typical cases of each condition are presented. The quality of life of the patients can be improved considerably by early diagnosis and treatment. Special diagnostic and therapeutic procedures are described.
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PMID:[Dysarthria, dysphagia or dyspnea as a reason for the initial consultation in pseudoparalytic myasthenia gravis and amyotrophic lateral sclerosis]. 231 Apr 61

A trial of Thyrotropin Releasing Hormone (TRH) 5.0 mg/kg body weight subcutaneously every other day for two weeks produced transient increased tone in muscles, along with other (side-) effects in patients with Amyotrophic Lateral Sclerosis (ALS). One patient's extensor plantar transiently changed to a flexor plantar reflex after injection, probably due to disproportionate increase in tone of the calf muscles. No significant changes in F-waves or H-reflexes were seen. No increase in useful voluntary strength, or in strength measured by Medical Research Council (MRC) testing or strain gauge isometric strength testing was seen. However, dyspnea was seen within 10 minutes of TRH injection.
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PMID:TRH-induced reflex changes in patients with ALS. 251 91

In amyotrophic lateral sclerosis (ALS), respiratory problems are usually late complications. However, ALS may occasionally present with exertional dyspnea as the primary symptom, as was the case in the patient reported here. On the basis of this case and the literature, the diagnostic and therapeutic aspects of the neurologic causes of dyspnea are discussed. Where there is a discrepancy between severity of blood gas alterations, changes in lung volume determinations and severity of dyspnea, a neuromuscular disorder must be considered in differential diagnosis.
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PMID:[Exertional dyspnea as the initial manifestation of amyotrophic lateral sclerosis]. 372 5

A 59-year-old man presented with dyspnoea, hypersomnia followed by acute respiratory failure necessitating mechanical ventilation. There were no signs of cardiopulmonary disease and on the first few days, extubation was impossible. Further neurological evaluation supported the diagnosis of amyotrophic lateral sclerosis. The neuropathological examination corroborated this diagnosis, and showed a preponderance of lesions in the phrenic nuclei and axonal alterations associated with a fast evolution.
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PMID:Amyotrophic lateral sclerosis presenting with respiratory insufficiency as the primary complaint. Clinicopathological study of a case. 397 16

In the case of three patients an acute respiratory failure with alveolar hypoventilation is related to bilateral diaphragmatic paralysis apparently isolated from any other neurologic abnormalities. The current initial diagnosis of pulmonary embolism leading to admission in an intensive respiratory care unit, because of the severity of the acute respiratory failure, has to be rectified then. Bilateral diaphragmatic paralysis is suspected on account of the absence of any patent etiology, on increasing dyspnea in supine position and paradoxic movements of the upper abdomen (whether spontaneously or in attempted weaning of ventilation support). Bilateral diaphragmatic paralysis is asserted by electromyogram with measurement of nerve conduction velocities of the two phrenic nerves. In the first case, it appears early in the course of an amyotrophic lateral sclerosis; in the second case, it occurs before the presence of a herpes-zoster becomes patent. In the third case, paralysis seems to be idiopathic. Evolution is promising in the last two cases, owing to the reversibility of the lesions. The difficulty of diagnosis, the varying nature of etiology and prognosis encountered in these three cases are also apparent in the 15 cases published in medical literature. The small number of cases published up to now, contrasting with the cases we have witnessed over the last 3 years, leads us to think that this disease must exist more often and may remain unknown to us.
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PMID:[Acute respiratory failure disclosing bilateral diaphragmatic paralysis]. 717 Nov 80

A 59-year-old man with ALS developed dyspnea and was performed tracheostomy in September 1987. The cuff volume of a tracheostomy tube increased gradually in four years of mechanical ventilation. His chest X-ray in December 1992 showed a marked enlargement of the cuff. In March 1993, massive hemorrhage suddenly occurred through the tracheostomy site. This was controlled by hyperinflation of the cuff. He was diagnosed as tracheo-innominate artery fistula. An emergent operation was done by median sternotomy and right oblique cervical incision. The innominate artery, the subclavian artery and the common carotid artery were severed and occluded with sutures. The tracheal fistula was closed with a suture of monofilament. The postoperative course was satisfactory, however he died of respiratory failure ten months after the surgery.
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PMID:[Successful surgical repair of tracheo-innominate artery fistula in a case of ALS patient with long-term mechanical ventilation]. 763 24

A 68-year-old man with severe dyspnea was admitted as an emergency case. He had no past history of any respiratory or neuromuscular diseases. Immediately after insufflation of oxygen, respiratory arrest occurred. The blood gas analysis showed hypoxemia and severe hypercapnia (PaO2; 32 mmHg, PaCO2; 127 mmHg). We diagnosed as CO2 narcosis, and he was treated with a respirator in the ICU. He showed nonflaccid bilateral diaphragmatic paralysis and muscle atrophy of the upper extremities. As the EMG showed giant spikes of neurogenic pattern, he was diagnosed as ALS. Weaning from the respirator failed because of his respiratory muscle fatigue. He was given rehabilitation during the day time and ventilatory support with the respirator during the night. We conclude that if we meet with an emergency patient with CO2 narcosis without any pulmonary disorder, we have to suspect neuromuscular diseases, e.q. ALS. In some of such cases, mechanical ventilation supports social rehabilitation.
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PMID:[A case of emergency admission for CO2 narcosis in a patient with amyotrophic lateral sclerosis]. 852 59


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