Amyotrophic Lateral Sclerosis Case Study Discussion

Signs and Symptoms

Amyotrophic lateral sclerosis (ALS) is a complex disease that progressively affects the body’s muscles, bringing on symptoms such as weakness and atrophy. Patients like Mr. Hammen observe early symptoms like challenges in holding things, such as involuntary cramps or contractions in muscles known as fasciculations and muscle fatigue in the limbs, jaw, and the rest of the body. Other signs include difficulty swallowing, speech and language problems, and breathing issues in the latter stages. These symptoms are due to the loss of function of the upper and lower motor neurons, and the mixed motor neuron signs are the hallmarks of ALS. Amyotrophic Lateral Sclerosis Case Study Discussion

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Diagnosis and Confirmatory Tests

ALS diagnosis involves clinical examination, tests, and imaging. The first diagnosis indicates motor neuron damage and symptoms (Goutman et al., 2022). Electromyography confirms ALS, while NCS excludes other neuropathies. MRI helps rule out spinal cord tumors. Standard biochemical and hematological tests, including blood and urine tests, exclude other diagnoses. A genetic test is used in patients with familial ALS due to a family history. Other tests may also be conducted to exclude other possible diagnoses.

Prognosis

ALS is a fatal disease that affects motor neurons, and the progression of the disease is estimated to allow patients to live for 3 to 5 years after being diagnosed. Although the rate at which the disease progresses may not necessarily be fast, some patients may live longer, more so when they receive all the necessary supportive care. In Mr. Hammen’s case, there is nothing wrong with getting a diagnosis and treatment as early as possible to enable him to control the manifestations and possibly even halt the progression of the disease, thus improving the quality of life.

Treatment with Riluzole and Other Medications

Riluzole is the only drug proven beneficial in treating ALS by reducing glutamate release, a neurotransmitter linked to neuronal toxicity (Andrews et al., 2020)Amyotrophic Lateral Sclerosis Case Study Discussion. It slows the disease’s progression and increases a patient’s life expectancy. Edaravone, also known as Radicava, also slows physical deterioration. Symptomatic treatments include medications like Baclofen or Tizanidine, antidepressants, and Glycopyrrolate or Amitriptyline for emotional support.

Impact of ALS Through a Review of Systems

ALS affects various body systems, including musculoskeletal, neurological, respiratory, and cognitive (Mahoney et al., 2021). Musculoskeletal symptoms include weakness, atrophy, and spasticity, while neurological symptoms include hyperreflexia and spasticity. Respiratory issues include a deterioration in the diaphragm and intercostal muscle strength, leading to dyspnea and ventilatory assistance. Dysphagia increases the risk of aspiration and malnutrition, while dysarthria makes language comprehension challenging. Due to its rapid progression, ALS patients are more likely to suffer from depression and anxietyAmyotrophic Lateral Sclerosis Case Study Discussion.

Addressing Familial Concerns and Risk Factors

Mrs. Hammen’s concern about ALS being contagious is a misconception. ALS is not a contagious disease, with only a family history being a well-established risk factor. The remaining 90% occur randomly and do not have a pattern of inheritance. Genetic and genetic factors are believed to cause ALS. Age and gender may also be associated with CABG, with patients most affected between 40 and 70 years old.

Reflection

The case highlights the challenges in diagnosing and addressing ALS. The team identified specifics of ALS, including diagnostic tests, and developed an extensive care plan based on understanding ALS pathophysiology, diagnostic criteria, and treatments. ALS symptoms emphasize the need for a comprehensive approach, considering physical and psychological aspects and providing extensive support to patients and their families. Amyotrophic Lateral Sclerosis Case Study Discussion

References

Andrews, J. A., Jackson, C. E., Heiman-Patterson, T. D., Bettica, P., Brooks, B. R., & Pioro, E. P. (2020). Real-world evidence of riluzole effectiveness in treating amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 21(7-8), 509-518. https://www.tandfonline.com/doi/abs/10.1080/21678421.2020.1771734

Goutman, S. A., Hardiman, O., Al-Chalabi, A., Chió, A., Savelieff, M. G., Kiernan, M. C., & Feldman, E. L. (2022). Recent advances in the diagnosis and prognosis of amyotrophic lateral sclerosis. The Lancet Neurology, 21(5), 480-493. https://www.thelancet.com/article/S1474-4422(21)00465-8/abstract

Mahoney, C. J., Ahmed, R. M., Huynh, W., Tu, S., Rohrer, J. D., Bedlack, R. S., … & Kiernan, M. C. (2021). Pathophysiology and treatment of non-motor dysfunction in amyotrophic lateral sclerosis. CNS drugs, 35(5), 483-505. https://link.springer.com/article/10.1007/s40263-021-00820-1  Amyotrophic Lateral Sclerosis Case Study Discussion

Case Presentation

Jack Hammen is 64 years old. As he is walking to the exam room, he drops his water bottle stating with his arthritis he is always dropping something. Jack has enjoyed a relatively healthy life with no major illnesses. He is happily married with three children and his first grandchild is on the way. You ask what prompted his visit. Jack responds, “I was golfing and I noticed I was having difficulty griping my club as tightly as I needed to. I’ve noticed a pattern of general weakness—legs, arm, hands, even my jaw—sneaking up on me lately and I thought I should check it out.”

Physical Exam

Jack appears relaxed and alert. His height and weight are within a healthy range for his age. His vital signs are as follows: BP 127/85, HR 72, RR 17, O2 sat 97% on room air, temp 97.5. You ask Jack to swallow and you note he has some difficulty doing so. He states that is a common issue recently. He does exhibit a general muscle weakness—he can overcome a lowered resistance, but not full resistance in most cases.

Electromyography (EMG) demonstrates muscle twitching and decreased strength of muscle contraction. Laboratory studies show increased levels of Glutamate and creatinine kinase (CK)Amyotrophic Lateral Sclerosis Case Study Discussion.

Assignment

Please answer all the questions below.

1. Mr. Hammen is diagnosed with amyotrophic lateral sclerosis (ALS). What are the signs and symptoms of ALS? How is the disease typically diagnosed? What test (s) should be performed to confirm the diagnosis? What is the prognosis for Mr. Hammen?

S&S:
Diagnosis:
Tests/Examinations:
Prognosis:
2. Mr. Hammen is prescribed Rilutek (riluzole). What is the mechanism of action for this medication? Why it is prescribed for the ALS patient? What other medications may be prescribed? Provide rationale. Amyotrophic Lateral Sclerosis Case Study Discussion

3. Discuss the impact of ALS through a review of systems (ROS).

4. Mrs. Hammen expresses concern that she or one of their children may “catch” ALS or develop the disease in the future. What is your response to her? Is ALS contagious? What is the strongest risk factor for the disease?

5. Write a short reflection after completing this study. How confident were you in the plans of action? Did you use additional resources to assist with your responses Amyotrophic Lateral Sclerosis Case Study Discussion