Amyotrophic Lateral Sclerosis

There are a heterogeneous group of degenerative diseases that involve destruction of the large motor neurons of the brain. Amyotrophic Lateral Sclerosis (ALS) 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 United Kingdom, ALS is often known as motor neuron disease. ALS is a progressive 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 mechanisms leading to the death of neurons that occurs in ALS and Parkinson disease. ALS, the most common adult-onset 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 death usually results from loss of respiratory function.

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The disease affects all racial, socioeconomic, and ethnic 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 lower motor neurons leading to muscle weakness, atrophy, and losts of function. In most cases there is no loss of sensation, and intellect and sphincter control are preserved. The damage is typically bilateral, and although usually rapid in progression, the disease may on occasion proceed slowly, or stabilize after a period of progression.

The reported incidence is about 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 between 55 and 60 and the disease is more common in males than females (2:1; Hudson, 1981). Death is usually preceded by progressive respiratory failure. The cause of ALS is unknown. In about 5% to 10% of cases the disease is familial; in most of these cases the mode of inheritance is autosomal dominant. The disease also occurs amongst the Chamorros on the island of Guam at a much higher rate than elsewhere, suggesting an environmental toxin can cause the disease (Rowland, 1987).

However, the majority of cases arise sporadically. Cognitive Deficits Clinicians working with ALS patients generally conclude that the disease does not cause dementia. 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 scales has produced mixed results. Poloni, Capitani, Mazzini, and Ceroni (1986) found 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, Mussuto, & Stracciari, 1985; Iwaski, Kinoshita, Ikeda, Takamiya, & Shiojima, 1990) intellectual deterioration in ALS patients was found 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 treatment. ALS is not generally regarded as a dementing disorder. Psychosocial Consequences

The individual with ALS faces major problems in communication because of dysarthria, a reduction in mobility, pain from muscle atrophy, and the knowledge that the disease will progress to incapacity and death. Motor dysfunctions occur in the absence of cognitive decline and so patients retain the capacity for awareness 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 stress model outlined in the next 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 everyday life, he develops feelings of helplessness.... Helplessness and failure, real or anticipated, lead to decrease of self-esteem, sense of worth, dignity and confidence.

Anger becomes mixed with fear 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 also directed against other persons, and at natural processes which appear to be increasingly harsh and threatening as the individual becomes progressively impaired and weaker. (p. 268) Although anecdotal reports of depressive and emotional reactions are common in the literature, few studies have examined emotionality in ALS systematically.

Houpt, B. S. Gould, and Norris (1977) found that the incidence of depression in ALS was comparable 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 have 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 these are not a consequence of ALS (Houpt et al. , 1977; Peters, Wedell, & Mulder, 1977). Families and caregivers are likely also to feel distressed by the onset and progress of the disease. The physical deficits reduce the patient's mobility and communication, leading to greater dependence on the family for emotional and functional support. The demands involved in caring for the ALS sufferer at home can result in the caregiver feeling alone, housebound, and unappreciated. Financial concerns and preexisting family conflict may accentuate these problems (Ringel, 1987).

For spousal caregivers there are many changes in role to be contemplated. Finally it is important to recognize that many people with ALS adapt to their illness in a creative, positive, and stoical manner. In an insightful commentary, B. S. Gould (1980) described how many ALS patients project 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 acknowledged.

The ALS patients in our series frequently maintained a strategy 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 ordinary conscious functioning, 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 enter 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 drug. For this reason, it is still 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 really 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 outer nerve cell sheath. Sublingual B12, antioxidants, CoQ10 and other dietary supplements are also thought to slow the disease’s progression. Ventilation is Still on Top From the patients’ perspectives, there are certain issues on the use of mechanical ventilation for the treatment of ALS.

These may include the examination of the process of and factors involved in decision 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’ impact on family, the decision as related to level of disability, and the ventilation availability through a nasal mask. Research format will include several general questions along with a demographic information questionnaire.

These general questions will focus on obtaining information about the process of the participant in their consideration of the use of mechanical ventilation, their current decision on the use of mechanical ventilation, the influence of others, and their decision’s 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 Hillel 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 research 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 arrangement for regular maintenance; 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 wearing a plastic mask apparatus over the nose, held in place by a strap 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 completion 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 author's familiarity with the participants may have influenced the selection process and the responses of the participants. Patients who had no means 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 treating 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

1. Caroscio J. (Ed. ). (1986). Amyotraphic lateral sclerosis: A guide to patient care. New York: Thieme.
2. Gallagher J. P. (1989). Pathologic laughter and crying in amyotrophic lateral sclerosis: A search for their origin.
3. Acia Neurologica Scandinavica. 80, 114-117. Gallassi R. , Montagna P. , Ciardulli C. , Lorusso S. , Mussuto V. , & Stracciari A. (1985).
4. Cognitive impairment in motor neuron disease. Acta Neurologica Scandinavica. 71, 480-484. Gould, B. S. (1980).
5. Psychiatric aspects. In D. W. Mulder (Ed. ), The diagnosis and treatment of amyotrophic lateral sclerosis (pp. 157-168).
6. Boston, MA: Houghton Mifflin. Hillel, A. D. , Miller, R. M.
7. , Yorkston, K. , McDonald, E. , Norris, E H. , & Konikow, N. (1989).
8. Amyotrophic Lateral Sclerosis Severity Scale. Neuroepidemiology. 8, 142-150. Houpt J. L. , Gould B. S. , & Norris F. H. (1977).
9. "Psychological characteristics of patients with amyotrophic lateral sclerosis". Psychosomatic Medicine. 39, 299-303. Hudson A. J. (1981).
10. "Amyotrophic lateral sclerosis and its association with dementia, Parkinsonism, and other neurological disorders: A review". Brain. 104, 217-247. Iwasaki Y. , Kinoshita M. , Ikeda K. , Takamiya K. , & Shiojima Y. (1990).
11. "Cognitive impairment in amyotrophic lateral sclerosis and its relation to motor disabilities". Acta Neurologica Scandinavica. 81, 141-143. Luloff P. B. (1986). "Reactions of patients, family, and staff in dealing with amyotrophic lateral sclerosis".
12. In J. Caroscio (Ed. ), Amyotrophic lateral sclerosis: A guide to patient care (pp. 266-271). New York: Thieme Publishers. Peters P. K. , Wedell M. S. , & Mulder P. W. (1977). "Is there a characteristic personality profile in amyotrophic lateral sclerosis? " Archives of Neurology. 35, 321-322. Poloni M. , Capitani E.

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