Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We shortened single fiber electromyography examination by evaluating fiber density, jitter and block numbers in the 10th and the 20th different needle positions in the extensor digitorum communis. The results obtained in 15 normal subjects and 12 amyotrophic lateral sclerosis patients were comparated. There were no significant differences with the 10th and 20th needle positions in normal subjects or in patients. This method diminished patient discomfort and shortened the examination time.
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PMID:[Value of a rapid method to determine muscle fiber density in amyotrophic lateral sclerosis]. 772 27

Amyotrophic lateral sclerosis (ALS) is a progressive disorder of unknown origin. Respiratory involvement is the principal cause of death, and dyspnoea is a major source of discomfort. In this study, diaphragm function is described and its relationship with dyspnoea examined in 48 ALS patients (32 male, age 26-80 yrs). The detailed neurological and respiratory evaluation (clinical examination, pulmonary function tests, static pressures, mouth twitch pressures (Pm,t), electromyographic responses to phrenic nerve stimulation and cortical magnetic stimulation were analysed after stratification according to dyspnoea. Dyspnoeic (group I) and nondyspnoeic (group II) patients were similar, bulbar signs being more frequent in group I. Vital capacity was lower in group I (mean+/-SD 67.9+/-22.7 versus 87.9+/-15.6% of the predicted value, p=0.0028), as were maximal static inspiratory pressure (41+/-24 versus 60+/-27% pred, p=0.0242) maximal static inspiratory pressure (18+/-11 versus 32+/-14% pred, p=0.0042), and Pm,t (3.71+/-2.5 versus 7.26+/-3.45 cmH2O, p=0.0011). Abdominal (Abd) paradox and respiratory pulse were frequent in group I (15 of 25 and 14 of 25) but absent or rare in group II (0 of 23 and four of 23) (p<0.05). The electromyographic responses to phrenic and cortical stimulation were generally abnormal in group I but subnormal in group II. Multivariate analysis selected only signs of diaphragm dysfunction (namely, Abd paradox and abnormal electromyographic responses) as significant predictors of dyspnoea. It is concluded that dyspnoea in amyotrophic lateral sclerosis patients should prompt diaphragm function tests.
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PMID:Diaphragmatic dysfunction and dyspnoea in amyotrophic lateral sclerosis. 1070 1

A 71-year-old man developed dysarthria and difficulty of swallowing in December 1997. He was diagnosed as having the bulbar type of amyotrophic lateral sclerosis (ALS). In November 1998, he was admitted to our hospital to undergo treatment for bulbar palsy and respiratory discomfort. In January 1999, ventilatory support (synchronous intermittent mandatory ventilation) during sleep at night was initiated. Severe progressive hypotension and loss of consciousness were observed soon after the start of artificial respiration, and both symptoms disappeared after artificial respiration was discontinued. This phenomenon was observed consistently during ventilatory support, while unpleasant stimuli such as bronchoscopy and replacement of the cannula tube induced severe hypertension. To clarify the mechanism of underlying these abnormal changes in blood pressure, autonomic function tests were performed while awake during the daytime. Ventilatory support induced a drop in blood pressure accompanied by a decrease in influx speed to the right ventriculum, the latter of which suggested a reduction in venous return. These values returned to the baseline following detachment of the ventilator. A 60 degrees head-up tilt (HUT) angle and standing from a supine position produced orthostatic hypotension, the latter of which was accompanied by a compensatory increase in pulse rate. The basal supine plasma noradrenaline (NA) level was high and the HUT showed a slight elevation of NA. The basal supine plasma arginine vasopressin (AVP) level was within the normal range, whereas the AVP level did not increase during HUT. Urinary secretion rates of NA and 3-methoxy-4-hydroxy-phenylglycol were elevated. A cold pressor test demonstrated reflex hypertension. The oculovagal reflex, coefficient of variation of R-R intervals. (CVR-R) and increase in pulse rate in response to atropine administration were within the normal range. The combination of midodrine, L-dihydroxyphenylserine (DOPS) and increasing intravascular volume via continuous intravenous drip infusion relieved the circulatory collapse during artificial respiration. In conclusion, the present case of ALS had sympathetic hyperactivity, somatosympathetic reflex and dysregulation of the baroreflex arc. Degeneration of central autonomic network, including the hypothalamus and the central nucleus of the amygdala, which has been shown in some ALS patients, might underlie the autonomic abnormalities in this patient.
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PMID:[A case of amyotrophic lateral sclerosis presenting with circulatory collapse during artificial respiration]. 1125 87

To understand the factors associated with interest in assisted suicide among terminally ill patients, we surveyed 50 caregivers of decedent amyotrophic lateral sclerosis (ALS) patients from Oregon and Washington regarding perceptions of patients' interest in assisted suicide and their physical and emotional state in the last month of life. For 38 caregivers, we had baseline information from the patients themselves, gathered a median of 11 months before death, regarding depression, hopelessness, sense of burden, social support, quality of life, pain, and suffering. According to our respondents, one-third of ALS patients discussed wanting assisted suicide in the last month of life. Hopelessness and interest in assisted suicide at baseline predicted desire for assisted suicide later on. ALS patients who were interested in assisted suicide, compared to those who were not, had greater distress at being a burden to others and more insomnia, pain, and discomfort other than pain.
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PMID:Predictors and correlates of interest in assisted suicide in the final month of life among ALS patients in Oregon and Washington. 1245 12

Although oral creatine supplementation is very popular among athletes, no prospective placebo-controlled studies on the adverse effects of long-term supplementation have yet been conducted. We performed a double-blind, placebo-controlled trial of creatine monohydrate in patients with the neurodegenerative disease amyotrophic lateral sclerosis, because of the neuroprotective effects it was shown to have in animal experiments. The purpose of this paper is to compare the adverse effects, and to describe the effects on indirect markers of renal function of long-term creatine supplementation. 175 subjects (age = 57.7 +/- 11.1 y) were randomly assigned to receive creatine monohydrate 10 g daily or placebo during an average period of 310 days. After one month, two months and from then on every fourth month, adverse effects were scored using dichotomous questionnaires, plasma urea concentrations were measured, and urinary creatine and albumin concentrations were determined. No significant differences in the occurrence at any time of adverse effects due to creatine supplementation were found (23 % nausea in the creatine group, vs. 24 % in the placebo group, 19 % gastro-intestinal discomfort in the creatine group, vs. 18 % in the placebo group, 35 % diarrhoea in the creatine group, vs. 24 % in the placebo group). After two months of treatment, oedematous limbs were seen more often in subjects using creatine, probably due to water retention. Severe diarrhoea (n = 2) and severe nausea (n = 1) caused 3 subjects in the creatine group to stop intake of creatine, after which these adverse effects subsided. Long-term supplementation of creatine did not lead to an increase of plasma urea levels (5.69 +/- 1.47 before treatment vs. 5.26 +/- 1.44 at the end of treatment) or to a higher prevalence of micro-albuminuria (5.4 % before treatment vs. 1.8 % at the end of treatment).
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PMID:Few adverse effects of long-term creatine supplementation in a placebo-controlled trial. 1579 16

Amyotrophic lateral sclerosis (ALS) is a disease with progressive muscle weakness, also affecting respiratory muscles. In the terminal phase most patients experience a progression. Nutrition, speech and breathing capacity decrease. It is important to inform the patient and relatives in time and to give them a chance to decide. "Care Planning" and "Advance Directives" especially concerning ventilation reduces fear and helps the doctors and carers to decide, following the will of the patient. Nobody knows the speed of the progression. The patient in this case had few subjective symptoms at the time of the family conference. Progression till death lasted one month only. Treatment of his dyspnoe was not optimised, but during care all decisions were based on the actual will of the patient. Generally nocturnal hypoventilation, for instance non-invasive ventilation by BiPAP-mode, can relieve symptoms of dyspnoe in ALS patients. Low-dose morphine and/or benzodiazepine relieve respiratory discomfort and remove the negative spiral of dysnoe-fear-dyspnoe. Oxygen therapy is usually not needed (only in the very last stages of the disease) and is not recommended especially during the night. Hypercapnia can occur because of hypoventilation. This can cause growing unconsciousness and maybe death during sleep. Prolonging life is only possible by invasive long-term ventilation with all the problems of intensive care measures. The patient could have been given low dose morphine from the time of the family conference. Ventilation by CPAP-mode was insufficient for him.
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PMID:[Amyotrophic lateral sclerosis--when planning is almost too late]. 1683 Feb 50

A 63-year-old woman with amyotrophic lateral sclerosis (ALS) was scheduled for open reduction and internal fixation of the right tibia. Total intravenous anesthesia using propofol and remifentanil without muscle relaxant was selected as the anesthetic method, in order to avoid the possible occurrence of ventilatory depression due to abnormal responses to muscle relaxants and exacerbation of the motor neuron disease. After standard and neuromuscular monitoring devices were applied, anesthesia was induced and maintained with target controlled infusion of propofol and remifentanil in the range of 2.5-5.0 microg x ml(-1) and 2.5-5.0 ng x ml(-1), respectively. To avoid delayed neuromuscular recovery, we did not use any muscle relaxant at all. Intubation was successful and there were no remarkable events during anesthesia, except for three brief hypotensive events; there was no exacerbation of ALS itself during or after the anesthesia. She was discharged on postoperative day 3, without any discomfort.
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PMID:Total intravenous anesthesia without muscle relaxant in a patient with amyotrophic lateral sclerosis. 1901 85

A 40-year-old man with amyotrophic lateral sclerosis undergoing home non-invasive ventilation, with no risk factors for coronary artery disease, was admitted with bilateral lung infiltrates. Given the lack of a favorable clinical response, transfer to our department was scheduled. During ambulance transport the patient experienced chest discomfort. Upon arrival at our department, a diagnosis of tako-tsubo syndrome was made. In this report, the clinical aspects are taken as a basis to highlight differences with common available findings, and an international registry is proposed to help piece together fractional information present in the literature.
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PMID:[Tako-tsubo syndrome in a young man with amyotrophic lateral sclerosis. A case report]. 2159 60

We experienced an anesthetic management with rocuronium and neurostimulator for a surgical patient with amyotrophic lateral sclerosis. A 61-year-old man was scheduled for intrathecal baclofen pump implantation as treatment for his spasticity under general anesthesia. After oxygenation and totally intravenous induction with propofol and remifentanil, we administered 10 mg of rocuronium repeatedly monitoring with neurostimulator. When dosage of rocuronium reached 20 mg, train-of-four count reached 1 and his trachea was intubated without coughing or moving. Anesthesia was maintained intravenously. Train-of-four ratio recovered to 95%, 22 minutes after the first administration of rocuronium. Operation was accomplished uneventfully with no additional rocuronium. Bispectral index value recovered to 98 and the patient awoke and breathed spontaneously 19 minutes after termination of administration of anesthetic agents. We could confirm his stable and adequate respiration and trachea was extubated without reversal of rocuronium. In the postanesthesia care unit, he showed no discomfort and was returned to the ward. His symptoms did not deteriorate postoperatively and he was discharged on the 36th postoperative day.
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PMID:[Anesthetic management for a patient with amyotrophic lateral sclerosis; the neuromuscular monitoring was useful to determine appropriate dosages of rocuronium]. 2274 22

A 31-year-old woman with amyotrophic lateral sclerosis (ALS) with respiratory muscle paralysis was scheduled for tracheotomy. After applying standard neuromuscular monitoring devices, general anesthesia was induced and maintained with propofol, remifentanil, rocuronium, and sevoflurane. Sugammadex is a potent agent for reversal of neuromuscular blockade by rocuronium. The patient emerged from general anesthesia smoothly using sugammadex; however, assisted respiration was continued for possible prolongation of the effect of muscle relaxant. The postoperative course was uneventful, and she was discharged without any discomfort.
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PMID:[Anesthetic management using muscle relaxant in a patient with amyotrophic lateral sclerosis]. 2301 40


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