Amyotrophic Lateral Sclerosis

There argon a heterogeneous group of degenerative disorders that touch on destruction of the large motor neurons of the brain. Amyotrophic Lateral Sclerosis (amyotrophic squinty induration) is one. The motor effects of this disease can be devastating. The usual course is a progression to death within 3 to 4 years. In the linked Kingdom, ALS is often know as motor neuron disease. ALS is a innovative neuromuscular disease that weakens and eventually destroys motor neurons that connect the brain with the skeletal muscles.NIEHS grantee Serge Przedborski of Columbia University has pioneered the investigation of the molecular(a) mechanisms leading to the death of neurons that occurs in ALS and Parkinson disease. ALS, the most common adult- blast paralytic disease, is most commonly diagnosed in middle age, and affects men more often than women. Patients gradually lose the ability to speak, swallow, and move voluntarily. Sensory function and intellectual ability are unaffected, and de ath usually results from loss of respiratory function.The disease affects all racial, socioeconomic, and heathenish groups, and the life expectancy of ALS patients is usually three to five years after diagnosis. ALS results in progressive damage to the pyramidal motor system. There is a degeneration of both the upper and lour motor neurons leading to muscle weakness, atrophy, and losts of function. In most cases there is no loss of sensation, and intellect and anatomical sphincter control are preserved. The damage is typically bilateral, and although usually rapid in progression, the disease whitethorn on occasion proceed slowly, or stabilize after a period of progression. The reported incidence is closely 0.4 to 1. 8/100,000 (Tandan & Bradley, 1986) and prevalence estimates range from 4 to 7/100,000. The mean age of onset lies surrounded by 55 and 60 and the disease is more common in males than females (21 Hudson, 1981). Death is usually preceded by progressive respiratory fai lure. The ca handling of ALS is unknown. In ab come out of the closet 5% to 10% of cases the disease is familial in most of these cases the mode of inheritance is autosomal dominant. The disease similarly occurs amongst the Chamorros on the island of Guam at a often higher rate than elsewhere, suggesting an environmental toxin can cause the disease (Rowland, 1987).However, the majority of cases arise sporadically. Cognitive Deficits Clinicians on the job(p) with ALS patients generally conclude that the disease does non cause aberration. Although there are cases of ALS where dementia does present, this can be attributed to the concurrent effects of DAT (Caroscio, 1986). Testing ALS patients with intellectual exceeds has produced mixed results. Poloni, Capitani, Mazzini, and Ceroni (1986) order no difference on the WAIS between 21 ALS subjects and 21 comparison subjects with nondementing neurological conditions.In two other studies (Gallassi, Montagna, Ciardulli, Lorusso, Muss uto, & Stracciari, 1985 Iwaski, Kinoshita, Ikeda, Takamiya, & Shiojima, 1990) intellectual deterioration in ALS patients was strand when the test results were compared to those of healthy controls. It remains to be determined whether these cognitive changes occurred as a result of ALS, or whether the differences were due to the nonspecific effects of hospitalization and intervention. ALS is not generally regarded as a dementing disorder. Psychosocial ConsequencesThe individual with ALS depends major problems in converse because of dysarthria, a reduction in mobility, pain from muscle atrophy, and the knowledge that the disease will progress to fatuity and death. Motor dysfunctions occur in the absence of cognitive decline and so patients retain the capacity for sensation of their disabilities. The nature of the disorder is likely to provoke an emotional response. The onset of the disease provides an illustration of the working of psychosocial examine model outlined in the ne xt chapter. Although ALS patients confront the disorder with courage (B.S. Gould, 1980), the debilitation caused by the disease challenges the capacity to adjust in even the most resolute individual. Luloff (1986) describes the demands and emotional sequelae of the disease as follows Loss of resources physical, psychological, social, and economic evokes grief and depression. As the patient anticipates experiences or experiences failures in mastering problems and challenges of every daylight life, he develops feelings of helplessness. Helplessness and failure, real or anticipated, lead to decrease of self-esteem, sense of worth, dignity and confidence. pettishness becomes mixed with dismay and accentuated by limitations in ability to master everyday problems, in achieving relief from tension, and in providing oneself with gratifying experiences. Anger is often directed against oneself for being damaged, helpless, and worthless, and a failure. . . . Anger is in any case directed a gainst other persons, and at indispensable processes which appear to be increasingly harsh and threatening as the individual becomes progressively impaired and weaker. (p. 268) Although anecdotical reports of depressive and emotional reactions are common in the literature, few studies pee examined emotionality in ALS systematically.Houpt, B. S. Gould, and Norris (1977) found that the incidence of depression in ALS was corresponding to that in cancer patients. About 65% of the ALS patients scored in the nil-mild range on the BDI, 32. 5% were moderately depressed, and 2. 5% were severely depressed. Other reactions to ALS feel been cited in clinical reports including denial (Tandan & Bradley, 1985), guilt (Luloff, 1986), and diminished self-esteem (Ringel, 1987). Emotional lability and inappropriateness have also been reported on occasion (Gallagher, 1989). These symptoms have been attributed to damage to brainstem nuclei.Controlled investigations of psychiatric symptoms suggest the se are not a consequence of ALS (Houpt et al. , 1977 Peters, Wedell, & Mulder, 1977). Families and health care providers are likely also to feel distressed by the onset and progress of the disease. The physical deficits reduce the patients mobility and communication, leading to greater dependence on the family for emotional and functional support. The demands refer in caring for the ALS sufferer at home can result in the caregiver feeling alone, housebound, and unappreciated. Financial dreads and preexisting family conflict may accentuate these problems (Ringel, 1987).For spousal caregivers there are many an(prenominal) changes in role to be contemplated. Finally it is important to recognize that many community with ALS correct to their illness in a creative, positive, and stoical manner. In an insightful commentary, B. S. Gould (1980) described how many ALS patients throw up a positive aspect to the people around them. Although this may mask a deeper and realistic concern or fear, the use of some degree of denial may represent an adaptive response to the disease. In his view, the role of the counselor may be to provide a supportive environment in which this despair may be acknowledgedThe ALS patients in our series frequently maintained a schema of partial denial throughout their illness, but in a most healthy fashion. Reality was not denied as much as redefined the most distressing immutable aspects of the disease were not part of mundane conscious cognitive operation, and hope was maintained. Under safe conditions that allowed reflection and ventilation, however, the second-order denial was easily overridden distressing awareness was allowed to tape the consciousness in a controlled fashion, and considerable dysphoric tension was discharged.Treatment Highlights In view of the fact that by far there is no treatment for ALS and there is no known cure. Current theraphy consists of a daily dose of an experimental do drugs. For this reason, it is sti ll best to count on the available resources for the treatment of ALS. The drug myotrophin will be injected twice a day, alternating left abdomen, right abdomen, left thigh, right thigh to reduce scarring, as well as regular visits from occupational and physical therapists. Neither drug is genuinely thought to do much, but they are all there is right now.The physical therapy controls the rate of atrophy and minimizes edema in the extremities. It is also helpful to ingest huge amounts of vitamins. Three thousand milligrams a day of vitamin C and 800 milligrams of vitamin E, both of which are thought to be especially important in protecting the outermost nerve cell sheath. Sublingual B12, antioxidants, CoQ10 and other dietary supplements are also thought to slow the diseases progression. Ventilation is Still on Top From the patients perspectives, there are certain issues on the use of mechanically skillful ventilation for the treatment of ALS.These may include the examination of the process of and factors involved in finding making by people severely disabled by the disease by the time they face the choice of whether to use the support of mechanical ventilation. Moreover, issues that should also be addressed will include patients furbish up on family, the decision as related to level of disability, and the ventilation availability through a cadaverous mask. Research format will include some(prenominal) general questions along with a demographic culture questionnaire.These general questions will focus on obtaining information about the process of the participant in their regard of the use of mechanical ventilation, their current decision on the use of mechanical ventilation, the influence of others, and their decisions commitment. Interviews will also be utilized for the participants so that they will be rated according to their function in the areas of speech, swallowing, lower and upper extremities with the use of ALS Severity Scale as designed by Hille l et al.(1989). The scale allows for rapid, ordinal measurement of the aforementioned areas. Each symptomatic area is rated from 1 to 10, with 10 indicating normal functioning and 1 reflecting the most severe disablement. A total score of 40 would denote normal functioning in all four areas. Since several participants were unaware of available ventilatory options, the physician on the question team individually met with the participants who had not already gained knowledge concerning ventilatory methods.During these meetings, the physician imparted basic information on the following the disease process that leads to respiratory insufficiency, simple measures such as positioning in alleviating respiratory distress, the capability to discontinue use of assisted ventilation, availability of palliative measures in end-stage ALS, care needs of people with the use of mechanical ventilation (such as suctioning of excess secretions management of the ventilator itself, including arrangemen t for regular alimentation and possible necessity of a caregiver 24 hours a day), and types of mechanical ventilation.The two basic methods of mechanical ventilation are a nasal mask and a tracheostomy. A tracheostomy is a surgical opening into the trachea to which a ventilator hose is connected. The nasal mask method is noninvasive and involves tiring a plastic mask apparatus over the nose, held in place by a whip around the back of the head. The mask can be awkward and uncomfortable and may slip out of place at night. Limitations In view of the reason that only a limited time was allowed for outcome of the work, fiscal and temporal constraints were in effect, thus limiting the size of the subject sample.The principal author was also the clinical social worker for the participants. The principal authors familiarity with the participants may have influenced the selection process and the responses of the participants. Patients who had no government agency of communication were not approached to take part in the study. Participant population was unequally distributed by gender, as there were 11 men and two women, and by race, as all were white. All participants had a comparable baseline knowledge of ventilatory methods.Four participants were atypical of classical ALS in that they had been diagnosed as having the disease for between nine and 23 years. A significant portion of the interviews with those participants already using mechanical ventilation was retrospective and based on recall. Not all participants answered all questions, and not all questions were applicable to all participants. Results may not be replicable with a different or larger sample. Conclusions Both past and present researches have failed on their quest for the cure or treatment of ALS.While this is apparently true, there are nevertheless several treatments that are found to be promising and responsive to patients. Individually, a specific approach may not be effective enough for treat ing ALS but a collaboration and combination of all known and practiced treatments is proved to be helpful. By now, this is the least that we can do for treating ALS while attempts are made in finding a cure for this certain incurable disease. References Caroscio J. (Ed. ). (1986). Amyotraphic lateral sclerosis A guide to patient care. New York Thieme.Gallagher J. P. (1989). Pathologic laughter and crying in amyotrophic lateral sclerosis A search for their origin. Acia Neurologica Scandinavica. 80, 114-117. Gallassi R. , Montagna P. , Ciardulli C. , Lorusso S. , Mussuto V. , & Stracciari A. (1985). Cognitive harm in motor neuron disease. Acta Neurologica Scandinavica. 71, 480-484. Gould, B. S. (1980). Psychiatric aspects. In D. W. Mulder (Ed. ), The diagnosis and treatment of amyotrophic lateral sclerosis (pp. 157-168). Boston, MA Houghton Mifflin. Hillel, A. D. , Miller, R. M., Yorkston, K. , McDonald, E. , Norris, E H. , & Konikow, N. (1989). Amyotrophic Lateral Sclerosis Severit y Scale. Neuroepidemiology. 8, 142-150. Houpt J. L. , Gould B. S. , & Norris F. H. (1977). 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