Term Paper: Etiology of Amyotrophic Lateral Sclerosis

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Amytrophic Lateral Sclerosis

Cognitive Deficits In Amytrophic Sclerosis

Amyotrophic Lateral Sclerosis (ALS) refers to a progressive neurodegenerative condition characterized by progressive loss of the upper and the lower neurons in the spinal cord and the brain. It affects the motor neurons that control the movement of the body's voluntary muscles. Destruction of these muscles results in an increase in the muscle and body weakness and consequent loss of the control of the discretionary body movements. The loss of the voluntary muscles causes a state of muscle paralysis. The prognosis of ALS is extremely variable. On average, the affected might survive for a period of three to five years following its diagnosis. Although the ALS is considered as a rare disorder, the condition commonly causes the degeneration of the neurons in adult muscles with an incidence rate of one to two people in every 100,000 individuals. The lack of an effective cure and/or treatment for the condition or therapy to reverse the weakening and destruction of the motor neurons compounds to the challenges associated with the management of the conditions (Neary, Snowden, & Mann, 2000).

Structure of the research paper

This research paper analyzes different issues associated with ALS, a cognitive degenerative condition. The essay begins with the analysis of the neurological etiology of the ALS disorder, a condition that underlies the cognitive domains. Secondly, the paper relates the impaired cognitive process the typically seen occupational performance problems with the treatment strategies for the condition. The third section will review studies conducted to analyze the effectiveness of the treatment method. The success of this analysis depends on the use of different research articles, journals, and books to illuminate the light into the topic (ALS and neuron-degeneration).

Neurological etiology of the Amytrophic Lateral Sclerosis, a condition underlying the cognitive domain

Executive Functions

Empirical evidence shows that ALS causes cognitive dysfunction, a factor that contributes to the decreased quality of life of the patient. Initially, researchers considered ALS as a neuromuscular disorder. New researches show that, some patients show symptoms of cognitive dysfunction, a disorder that affects their cognitive functioning. These studies show a strong evidence of cognitive disabilities in patients with ALS with a continuum from mild cognitive disturbances to frontotemporal dementia also known as FTD. The incidence rate of FTD is rare as it occurs at a percentage of 3-5% of patients with ALS. In addition, the involvement of the cognitive domain makes it possible to use the DSM-IV to diagnose and monitor the progress of individuals affected with ALS. DSM-IV provides the most effective method of classifying ALS related conditions that predispose the victims to mental health related conditions. Among the conditions identified in patients with ALS using the DSM-IV, include depressive symptoms such as major and minor depression that signify involvement of the cognitive domain in an individual.

Significant evidence shows that the most predominant cognitive impairment type in patients suffering from ALS is largely related to their executive functioning. The deficits indicate dysfunction of the frontal lobe. Patients with frontal lobe dysfunction have shown problems related to logic, planning, and verbal fluency among other trivial tasks. The loss in the functioning is highly related with the atrophy of the temporal and frontal with the loss of neurons, ubiquitinated tau-negative and superficial linear spongiosis (Neary, Snowden, & Mann, 2000). Apart from this, patients who cannot carry out the functions because of the FTD and ALS have some of the anterior horn of the spinal cord.

Besides, PET scans on patients with ALS with dysfunction of the frontal lobe show a significant reduction in the level of cerebral glucose metabolism in the frontal and motor cortex. This shows their involvement in the degeneration of the functioning of the frontal lobe hence, the symptoms. Besides this, evidence shows reduced activation of the neurons of the anterior cingulated region, anterior thalamic nuclear complex, and the parahippocampal gyrus hence, reduced executive functioning (Kim et al., 2009).

Relating impaired cognitive processes to the typically seen/documented occupational performance problems.

It is apparently clear that, cognitive impairment affects the occupational performance of an individual in the society. In specific, it affects their ability to perform their activities of the daily living that contribute to their optimal performance. There is a strong relationship between a variety of bulbar symptoms in patients with ALS and cognitive involvement. Dysarthria, dysphagia, motor speech disorder, and swallowing difficulties are some of the bulbar symptoms associated with ALS. Research shows that, approximately 75% of patients with cognitive dysfunction present with bulbar related symptoms as compared to 21% of patients with the cognitive involvement. As such, this shows a strong relationship between bulbar involvement in ALS and psychological breakdown (Brettschneide et al. 2012). On the other hand, little evidence shows a significant effect on memory.

It is shown that patients with ALS presents with the loss of immediate memory. The deficits in the delayed recall vary significantly across the patients. The abnormality in memory lies in the ability of patients to encode information rather than its speed of forgetting the stored information. Empirical evidence reveals that, patients with ALS present with the loss of free picture recall and little involvement of the immediate recall. There is significant preservation of the visuoperceptual function in patients with ALS. However, involvement of the frontotemporal lobe results in defects such as difficulties in navigating their familiar environments like the home environment, identification of their home location in the map, copying hand postures, and location of objects. PET and MRI scans show significant involvement of the language networks in patients with ALS. Language deficits become evidenced by decreased verbal output, altered perseveration, stereotypic expressions, semantic paraphasia, and echolalia among other language-related deficits (Miller et al., 2009).

In addition, the involvement of the frontotemporal lobe affects the social cognition and processing of emotions in patients with ALS. Patients with ALS with involvement of the frontotemporal lobe have impaired recognition of emotions, in specific, anger, and disgust. As such, they have difficulties in identifying social violations. Moreover, the lack of their response to emotions is attributable to the decreased response in the extra striate visual areas and anterior insula to the emotional changes. As a result, they have little emotional arousal hence, the alteration in the emotional and social cues. Frontal system behavior and frontal behavior inventory scales show significant impairment of behavior of the affected individuals. Many patients with ALS present symptoms like irritability, inflexibility, apathetic, and restlessness among other behaviors related impaired functioning. The behavioral impairment is highly attributed to the involvement of the frontotemporal lobe that affects the cognitive functioning, including the behavior of the patients (Goldstein et al. 2011).

In addition, the patient's inability to execute different functions and activities of daily living also shows possible impaired cognitive functioning. These patients presents with difficulties in problem solving, and response to novelty. Apart from these, patients have difficulties in planning, verbal fluency,-word retrieval tasks, and logic. When tested, the patients show difficulties in maintaining word fluency, hence, signifying the effect of the cognitive impairment to the activities of their daily living (NOLAN et al. 2008). Cumulatively, this analysis shows that, ALS has significant influence on the cognitive functioning of the patients. Some of the effects occur because of the involvement of the frontotemporal lobe.

Treatment methods or strategies to impaired cognitive processes as evidence by ALS and FTD associated symptoms.

There is no effective treatment method (s) available for the management of ALS. However, a number of strategies for managing the condition exist. These treatment methods or strategies aim at optimizing the patient's quality of life. As such, the strategies place the health care provider in activities he/she might not be used to do or activities that require broad application of pharmacological and non-pharmacologic interventions. Thus, the interventions used in the management of the symptoms associated with ALS aim at improving the patient's psychological state, independence, social relationships, relationship with the environment, and personal beliefs. One of the pharmacologic managements of the ALS is the administration of the Riluzole, an effective glutamate antagonist. The drug reduces the decline of the strength of the muscles, improving the quality of life and survival rate of patients with ALS. Pharmacologic properties such as its high tolerance rate among the ALS patients and wide use across patients with a variety of conditions. Other drugs such as Minocycline show a significant effect on the outcomes of patients with ALS. Minocycline reduces the onset of the disease and prolongs the survival of patients with ALS. Ultimately, most of the patients with ALS present with a progressive failure of respiratory functioning. As such, it is important for the monitoring of Forced Vital Capacity (FVC) to assess the respiratory functioning (Neary, Snowden, & Mann, 2000).

The bulbar symptoms associated with cognitive impairment predispose the patients to malnutrition and dehydration. Dysphagia, tongue weaknesses, coughing, and choking are some of the symptoms associated with ALS that predispose the patient to malnutrition and dehydration. As such, this necessitates management strategies such as changing food consistency to manage dysphagia,… [END OF PREVIEW]

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