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)

Only a minority of patients who have ALS require, request, and receive assisted or supported ventilation. Usually, when a mechanical ventilator is needed, nonsurgical methods can be used for prolonged periods of time. Appropriately timed discussions can reduce the need for emergency management of breathing failure. The doctrine of informed consent applies to decisions about life support. It involves both the physician (to exercise clinical judgment on behalf of the patient) and the patient (to make personal decisions). They must interact. The patient's firm decision must be clear but need not be in the form of a "living will," and it does not need to be sought repeatedly or reiterated endlessly. Just as a considered decision cannot be arbitrarily overthrown in a time of crisis, neither can a change of mind be willfully ignored. In practice, this may test the capability of even the most experienced and understanding physician, and may result in less-than-ideal outcomes, as our examples show. As in any other area of medical practice, personal experience teaches valuable lessons. Unfortunately, even extended publications discussing clinical management of ALS have failed to address the subject of discontinuing ventilatory support, and ethicists have not always been helpful. Bernat and Beresford have, however, successfully summarized the ethical issues involved. Failure to sustain breathing mechanically or withdrawing artificial support of breathing from a requesting patient who, in the terminal stage of ALS, has become unable to breathe without a mechanical ventilator cannot be called assisted suicide, mercy killing, or either passive or active euthanasia. It is allowing a competent person to die naturally of the incurable illness that afflicts him. The state has no legal interests to be served by intervening in the process just described, which bears no relationship to issues of malpractice, much less to criminal negligence or homicide. Neurologists have not uniformly understood these points, as demonstrated by previous publications addressing the issue and by the findings of our own survey of neurologists who have special experience in the area of neuromuscular diseases. In regard both to starting and to stopping the ventilator, we believe strongly that it is time to lay aside the moral, legal, and ethical conflicts that have needlessly delayed or prevented physicians from complying with the resolute decisions that competent patients have made about their own lives. We urge doctors to act in these cases, as in all others, with their best medical judgment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Starting and stopping the ventilator for patients with amyotrophic lateral sclerosis. 258 1

Transcript of the December 17, 1992 testimony of Sue Rodriguez before the Supreme Court of British Columbia. Rodriguez, who had been diagnosed with amyotrophic lateral sclerosis, was petitioning the court to allow her the option of physician-assisted suicide.
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PMID:Rodriguez vs. Attorney General of Canada (petitioners testimony). 750 1

The Supreme Court of British Columbia dismissed the petition of Sue Rodriguez, who suffered from amyotrophic lateral sclerosis. Rodriguez had sought an order from the court declaring invalid a section of the Criminal Code of Canada to the extent that it prohibits a terminally ill person from committing physician-assisted suicide.
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PMID:Rodriguez vs. Attorney General of Canada (trial court opinion). 810 38

Above-average exposure to electromagnetic fields has been associated with certain nonmalignant medical conditions such as amyotrophic lateral sclerosis, other neurologic diseases, depressive symptoms, and suicide. The authors conducted a nationwide mortality study in Denmark of 21,236 men employed in utility companies between 1900 and 1993. The causes of death were ascertained for January 1, 1974, through December 31, 1993, and cause-specific mortality was analyzed by latency and estimated levels of exposure to 50-Hz electromagnetic fields. Overall, 3,540 deaths were observed as compared with 3,709 expected from national mortality rates, yielding a standardized mortality ratio of 0.96 (95% confidence interval 0.93-0.99). A slight excess in mortality from cancer was due to deaths from cancers of the lung and pleural cavity, probably because of exposure to asbestos. A twofold increase in mortality from amyotrophic lateral sclerosis and a tenfold increase in mortality from electrical accidents were seen on the basis of 14 and 10 deaths, respectively, the former increasing with time since first employment in a utility company. The excess mortality from amyotrophic lateral sclerosis seems to be associated with above-average levels of exposure to electromagnetic fields and may be due to repeated episodes with electric shocks.
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PMID:Mortality from amyotrophic lateral sclerosis, other chronic disorders, and electric shocks among utility workers. 971 80

Legal developments in assisted dying have focused on assisted suicide for mentally competent, terminally ill adults. Requests for assisted dying are likely to represent broader concerns, but studies have been limited to surveys of specific patient groups or recollections of physicians. To describe the nature of inquiries by a broad range of persons seeking assisted dying, a retrospective review was performed of confidential client memoranda summarizing telephone inquiries regarding assisted dying to a counseling service of a national, not-for-profit, consumer-based organization. The review evaluated the underlying medical condition prompting the inquiry, evidence of patient's decisional capacity, and relationship of caller to the patient. Of 125 assisted suicide calls, 111 with medical illness were analyzed. Among 111 inquiries, 71 (64%) were made by someone other than the person suffering from the illness ("the patient"); 52 (47%) of these were family members, 14 (13%) were friends, and 5 (7%) were professionals. Cancer accounted for 25% of cases, HIV/AIDS and amyotrophic lateral sclerosis (ALS) for 10 and 9%, respectively, other neurological diseases for 23%, and chronic or other medical conditions for approximately 17%. In an additional 10 cases, there was no medical illness. Within the group of callers inquiring on behalf of others, 18 (25%) stated the patient lacked ability to communicate his or her wishes and 7 (10%) stated there was uncertainty. Inquiries about assisted dying represent a broader range of concerns than represented by legal initiatives. More study is needed to determine if enhanced knowledge about alternatives, such as palliative care, reduces requests for assisted dying.
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PMID:Pursuit of assisted dying: a pilot study of inquiries made to a national consumer-based organization. 1064 65

Neurodegenerative processes are generally characterized by the long-lasting course of neuronal death and the selectivity of the neuronal population or brain structure involved in the lesion. Two main common forms of cell death that have been described in neurons as in other vertebrate tissues i.e., necrosis and apoptosis. Necrosis is the result of cellular "accidents", such as those occurring in tissues subjected to chemical trauma. The necrotizing cells swell, rupture and provoke an inflammatory response. Apoptosis, on the other hand, is dependent on the cell's "decision" to commit suicide and die, and therefore is referred to as "programmed cell death" (PCD). The course of apoptotic death is characterized by a massive morphological change, including cell shrinkage, nuclear (chromosome) condensation and DNA degradation. Activation of PCD in an individual cell is based on its own internal metabolism, environment, developmental background and its genetic information. Such a situation occurs in most of the neurodegenerative disorders such as Alzheimer's, Parkinson's and Huntington's diseases and amyotrophic lateral sclerosis (ALS). In these pathological situations, specific neurons undergo apoptotic cell death characterized by DNA fragmentation, increased levels of pro-apoptotic genes and "apoptotic proteins" both, in human brain and in experimental models. It is of utmost importance to conclusively determine the mode of cell death in neurodegenerative diseases, because new "anti-apoptotic" compounds may offer a means of protecting neurons from cell death and of slowing the rate of cell degeneration and illness progression.
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PMID:Apoptosis as a general cell death pathway in neurodegenerative diseases. 1112 5

In 1979 the first study was published which indicated that environmental exposure to power frequency, electric and magnetic fields (EMF), might increase the risk of chronic disease. This was a study on cancer. However, this research area has gradually evolved and come also to include outcomes other than cancer. The purpose of this paper is to provide a better understanding of the literature on neurodegenerative diseases and on suicide and depressive symptoms in relation to EMF by using a meta-analysis technique. It is concluded that for amyotrophic lateral sclerosis, there are relatively strong data indicating that electric utility work may be associated with an increased risk. However, EMF exposure is only one of several possible explanations to this. For Alzheimer's disease the combined data on an association with EMF are weaker than that for ALS. For suicide an overall assessment yields the conclusion that the support for an association is weak. For depressive symptoms the assessment is more complex, but the overall conclusion is nevertheless that the evidence is relatively weak. For other diseases, such as Parkinson's, there is not enough information for an assessment.
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PMID:Neurodegenerative diseases, suicide and depressive symptoms in relation to EMF. 1117 Jan 23

Exposures to extremely low-frequency electric and magnetic fields (EMF) emanating from the generation, transmission, and use of electricity are a ubiquitous part of modern life. Concern about potential adverse health effects was initially brought to prominence by an epidemiologic report two decades ago from Denver on childhood cancer. We reviewed the now voluminous epidemiologic literature on EMF and risks of chronic disease and conclude the following: a) The quality of epidemiologic studies on this topic has improved over time and several of the recent studies on childhood leukemia and on cancer associated with occupational exposure are close to the limit of what can realistically be achieved in terms of size of study and methodological rigor. b) Exposure assessment is a particular difficulty of EMF epidemiology, in several respects: i) The exposure is imperceptible, ubiquitous, has multiple sources, and can vary greatly over time and short distances. ii) The exposure period of relevance is before the date at which measurements can realistically be obtained and of unknown duration and induction period. iii) The appropriate exposure metric is not known and there are no biological data from which to impute it. c) In the absence of experimental evidence and given the methodological uncertainties in the epidemiologic literature, there is no chronic disease for which an etiological relation to EMF can be regarded as established. d) There has been a large body of high quality data for childhood cancer, and also for adult leukemia and brain tumor in relation to occupational exposure. Among all the outcomes evaluated in epidemiologic studies of EMF, childhood leukemia in relation to postnatal exposures above 0.4 microT is the one for which there is most evidence of an association. The relative risk has been estimated at 2.0 (95% confidence limit: 1.27-3.13) in a large pooled analysis. This is unlikely to be due to chance but, may be, in part, due to bias. This is difficult to interpret in the absence of a known mechanism or reproducible experimental support. In the large pooled analysis only 0.8% of all children were exposed above 0.4 microT. Further studies need to be designed to test specific hypotheses such as aspects of selection bias or exposure. On the basis of epidemiologic findings, evidence shows an association of amyotrophic lateral sclerosis with occupational EMF exposure although confounding is a potential explanation. Breast cancer, cardiovascular disease, and suicide and depression remain unresolved.
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PMID:Review of the epidemiologic literature on EMF and Health. 1174 9

The Supreme Court of Canada dismissed the petition of Sue Rodriguez, for a waiver of Section 241 of the Canadian Criminal Code, which makes it unlawful to aid or abet anyone in committing suicide. Rodriguez, who suffered from amyotrophic lateral sclerosis, a disease with an average life expectancy of three years, sought the waiver in order to make it permissible for her to be assisted in committing suicide when her illness became unbearable. The Court, in agreeing with two lower courts which had dismissed her application, held that section 241 did not infringe improperly upon the rights guaranteed by the Canadian Charter of Rights and Freedoms, including autonomy over one's person, freedom from cruel and unusual punishment, or deprivation of any benefit because of physical disabilities.
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PMID:Rodriguez v. British Columbia (Attorney-General). 1204 Nov 3

Studies of dying patients have shown that about half would like the option of physician-assisted suicide (PAS) to be available for possible future use. Those percentages decrease significantly with each step patients take toward action. Studies show that although about 10% of patients seriously consider PAS, only 1% of dying patients specifically request it, and 1 in 10 of those patients actually receive and take a lethal prescription. However, most patients' desires for PAS diminish as their underlying concerns are identified and addressed directly. To help identify concerns motivating a patient's request for PAS, physicians should talk with patients about their expectations and fears, options for end-of-life care, goals, family concerns and burdens, suffering or physical symptoms, sense of meaning and quality of life, and symptoms of depression. A patient with advanced amyotrophic lateral sclerosis (ALS) who requested PAS illustrates how a hasty response may adversely affect patient care and the health care team. Although physicians should remain mindful of their personal, moral, and legal concerns, these concerns should not override their willingness to explore what motivates a patient to make this request. When this approach is taken, suffering can be optimally alleviated and, in almost all cases, the patient's wishes can be met without PAS.
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PMID:Responding to requests for physician-assisted suicide: "These are uncharted waters for both of us...". 1242 12


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