Aspects of Amyotrophic Lateral Sclerosis

Introduction

Amyotrophic lateral sclerosis (ALS) clinical presentation generally comprises of adult-onset localized muscular weakness and atrophy that spreads with disease progression. ALS is distinguished by a combination of dysfunction in the upper and lower motor neurons, muscle cramps and weakness, hyperreflexia, and psychological symptoms (Rojas et al., 2020). There are numerous molecular mechanisms involved in the pathology of ALS, such as neurotoxic effects, mitochondrial disorders, modifications in synaptic transmission, oxidative stress, the buildup of misfolded proteins, and neuroinflammation. In addition to genetic factors; the pathophysiology directly impacts the motor neurons, the vertebral column, the cerebellum, and the brain; it has, moreover, been shown to affect the visual system (Rojas et al., 2020). ALS eventually impairs muscular control, affecting the ability to move, eat, and breathe. The purpose of the study is to research evidence-based physical therapy treatment for the ALS. Although there is no proven cure for this pathology, the specific medical management, with the PTA being an important part, can influence and improve the condition of such a patient, especially in the early stages.

Pathology, Etiology and Diagnosis of Amyotrophic Lateral Sclerosis

Progressive pure motor weakness that localizes focally is the most distinctive clinical feature of ALS. The disease begins with limb weakness in about two-thirds of patients, often preceded by cramps, and with bulbar weakness causing dysarthria and dysphagia. In rare instances, there is cognitive impairment, behavioral disturbance, or early respiratory failure as the initial manifestation of ALS. The characteristic combination of upper and lower motor neuron dysfunction is usually evident on neurological examination with the presence of weakness, atrophy and fasciculations together with hyperreflexia and increased tone in the same motor segment and not infrequently an extensor response to plantar stimulation. Sensory findings are minimal or absent, and the disease relentlessly spreads to other body parts and eventually to respiratory muscles leading to death from respiratory failure within 30 months on average.

The clinical diagnosis of ALS is based on history and physical examination that demonstrates increasing upper and lower motor neuron impairment. The diagnosis is usually supported by electrophysiological studies and neuroimaging and laboratory tests to exclude mimickers. ALS diagnosis is largely defined via the El Escorial Criteria by following factors: evidence of lower motor neuron degeneration either on examination or through specialized testing, evidence of upper motor neuron disease on examination, and the presence of either of the above in more than one region of the body (Richards et al., 2020). Furthermore, there must be no evidence of a viable alternative diagnosis and the disease must be progressive.

Treatment Interventions

Spasticity, a muscle stiffness affecting one or more whole limbs, is a symptom of ALS. While some drugs are being administered, it is critical to ensure that mobility does not suffer as a result of increased muscular weakness (Robinson & Estupian, 2019). The physical therapy treatment plays an essential role, with a focused exercise program that aims to help maintain joint range movement, prevent contractures, reduce stiffness and discomfort, and optimize function and quality of life are employed by therapists. The programs should, however, be appropriate to the patient’s level of function and tailored to their needs, abilities, and preferences. Other interventions of physical therapy helping to adapt to the physical and psychological challenges include counseling, provision of orthotics and appliances, and training in assistive technology (Hogden et al., 2017). The prognosis of such a treatment cannot guarantee a patient’s recovery, though, it ensures the relief on the physical and mental level.

Patients with ALS have a higher likelihood of developing depression; this is common especially after their diagnosis and perhaps also before their diagnosis. Depression and anxiety in ALS patients can be treated with both pharmaceutical and non-pharmacological therapies. Neither has been demonstrated to be superior to the other; therefore, a combined approach is recommended. Psychotherapy approaches such as cognitive behavioral therapy improve a patient’s ability to cope with their diagnosis and to adapt to the progressive decline in function. Cognitive-behavioral therapy, assisted by the PTAs, was shown to have promise for reducing anxiety and depression levels in patients with psychological disorders. Through a combined approach of pharmacological and non-pharmacological interventions, both the ALS patients and their families can be helped to maintain a level of emotional condition as they encounter the disease.

Progression of Physical Therapy Treatment

Patients in the early stages of the condition may benefit from preventative exercise regimens to avoid movement limitations. According to Martin (2020), moderate-load and moderate-resistance workouts increase function in a group of individuals with early-stage ALS when compared to a matched control group doing stretching; and he recommends the following parameters for exercise in the ALS population: avoid excessive unconventional exercise, mild resistance can develop muscular strength with a manual muscle testing grade of above 3, and excess activity is not a problem if the muscles have a manual muscle testing grade of 3 and above out of 5. As the condition advances, mobility declines, therefore the focus shifts to supporting weak muscles and modifying the home and workplace with support from both family members and colleagues. Some patients can be helped by a custom orthosis to support the neck and upper thoracic spine. In the course of the disease, it is appropriate to assess the patient’s need for pressure-relieving devices, such as a mattress or a wheelchair cushion.

Medical Management of Amyotrophic Lateral Sclerosis

There is no known cure for ALS, however, medication treatments administered to treat the disease classified as disease-modifying include riluzole and edaravone. While riluzole slows the progression of ALS by protecting motor neurons from toxic excitation, edaravone mitigates oxidative injury in neurons and neighboring glia that are at risk for degeneration in ALS. Therefore, the medication affects physical therapy treatment as it can cause such adverse effects as gait disturbance and muscular weakness, which requires the help of PTAs. Moreover, it was approved for use by those specializing in physical medicine and rehabilitation. Still, careful follow-up should be conducted after the initiation of the two medications.

Physical Therapy Assistants and Multidisciplinary Care

Physical therapist assistants (PTAs) coordinate ALS patients’ physical, occupational, behavioral, and speech therapies. Physical therapy aims to increase and maintain joint mobility, strength, endurance, and equilibrium. In addition, the physical therapists offer recommendations for adaptive equipment and other consultative services for ALS patients. Alongside their primary givers, ALS patients should have regular follow-ups with a multidisciplinary ALS team through a team-based treatment approach. PTAs observe the patients during the whole treatment process. The activation of cross-functional interaction across several care sectors by specialist ALS clinics has proven critical for the delivery of high-quality care in ALS (Hogden et al., 2017). Multidisciplinary care in ALS, therefore, encompasses the provision of care to ALS patients through a range of healthcare disciplines and support services (Andersen et al., 2021). Collaboration among community-based services, family physicians, physical therapists and their assistants, and hospice is essential, such that many ALS patients have had many engagements with various experts across multiple healthcare sectors by the final phase of the disease.

Conclusion

Professional multidisciplinary treatment is more than just a way to provide ALS patients with effective treatment therapy. Multiple components of treatment are included in this research to provide coordinated and interprofessional care that fulfils the expectations of ALS patients and their families. These include evidence-based treatment, service providers that take a patient-centered approach to care, effective inter-service communication, and a multidisciplinary team that is resourced and equipped to deliver expert care to patients. The role of PTA is vital as they serve as the bridge between a patient and a therapist and implement all the required treatments.

References

Andersen, P. M., Abrahams, S., & Borasio, G. D. (2021). EFNS Guidelines on the clinical management of amyotrophic lateral sclerosis (MALS): Revised report of an EFNS task force [With Systematic Review]. APTA. Web.

Robinson, M. T., & Estupian, D. (2019). Neuromuscular diseases. In Creutzfeldt, C. J., Kluger, B. M., & Holloway, R. G. (Eds.), Neuropalliative care: A guide to improving the lives of patients and families affected by neurologic disease (pp. 101-115). Springer.

Hogden, A., Foley, G., Henderson, R., James, N., & Aoun, S. (2017). Amyotrophic lateral sclerosis: Improving care with a multidisciplinary approach. Journal of Multidisciplinary Healthcare, Volume 10, 205–215. Web.

Martin, K. (2020). Neurologic interventions for physical therapy (4th ed.). Elsevier.

Richards, D., Morren, J. A., & Pioro, E. P. (2020). Time to diagnosis and factors affecting diagnostic delay in amyotrophic lateral sclerosis. Journal of the Neurological Sciences, 417, 117054. Web.

Rojas, P., Ramírez, A. I., Fernández-Albarral, J. A., López-Cuenca, I., Salobrar-García, E., Cadena, M., Elvira-Hurtado, L., Salazar, J. J., de Hoz, R., & Ramírez, J. M. (2020). Amyotrophic lateral sclerosis: A neurodegenerative motor neuron disease with ocular involvement. Frontiers in Neuroscience, 14. Web.

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NursingBird. (2024, February 5). Aspects of Amyotrophic Lateral Sclerosis. https://nursingbird.com/aspects-of-amyotrophic-lateral-sclerosis/

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NursingBird. (2024) 'Aspects of Amyotrophic Lateral Sclerosis'. 5 February.

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NursingBird. 2024. "Aspects of Amyotrophic Lateral Sclerosis." February 5, 2024. https://nursingbird.com/aspects-of-amyotrophic-lateral-sclerosis/.

1. NursingBird. "Aspects of Amyotrophic Lateral Sclerosis." February 5, 2024. https://nursingbird.com/aspects-of-amyotrophic-lateral-sclerosis/.


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NursingBird. "Aspects of Amyotrophic Lateral Sclerosis." February 5, 2024. https://nursingbird.com/aspects-of-amyotrophic-lateral-sclerosis/.