Amyotrophic Lateral Sclerosis
Amyotrophic Lateral Sclerosis is a malady where there is a motor neuron loss along the anterior horns of the spinal cord and the motor nuclei of the lower brain stem. When these cells die, the muscle fibers that they are supplying undergo atrophic changes. This leads to neuronal degeneration in the upper and lower motor neuron systems. The specific cause of Amyotrophic Lateral Sclerosis is utterly unknown; however, it has been suspected that some autoimmune diseases and free radical damages can be the underlying cause of such.
Manifestations
The signs and symptoms presented depend on the location of the affected neuron. Generally, the following presentations are evident:
- Fatigue
- Progressive muscle weakness
- Cramps
- Twitching
- Incoordination
Anterior horns
- Progressive weakness
- Muscle atrophy (arms, trunk, legs)
- Spasticity
- Brisk or overreactive muscle reflexes
Cranial nerves
- Muscle weakness
- Difficulty talking
- Difficulty swallowing
- Difficulty breathing
- Soft palate and upper esophageal weakness
- Weakness on the posterior tongue
Bulbar muscles
- Progressive difficulty in speaking
- Difficulty in swallowing
- Articulation and speech effects
- Compromised respiratory function
Assessment and Diagnosis
The following tests and assessment tools are used to verify the presence of Amyotrophic Lateral Sclerosis:
- EMG studies of affected muscles indicate reduction in the number of functioning motor units
- MRI may show high signal intensity in the corticospinal tracts
Management
In managing Amyotrophic Lateral Sclerosis, the medical and nursing team focuses on the optimal function of the patient since there is no targeted therapy.
- There is no specific therapy for this disease.
- Diagnosing the disease at an early stage could improve the patient’s condition (planning for the strategic planning of preventive measures) and avoid complications.
- The focus of management is to maintain or improve function, well being and quality of life
- Medications used to treat this disease are the following, these are used as ordered by the physician:
- Riluzole (Rilutek), a glutamate antagonist slows the deterioration of motor neurons and has neuroprotective effects
- Baclofen, dantrolene sodium or diazepam for spasticity
- For patients experiencing problems with aspiration and swallowing may require enteral feeding (percutaneous endoscopic gastrostomy tube). This tube can be used even if the patient is under a ventilation support system.
- While some patients are treated on the hospitals, some cases on the other hand are treated at home or in the community.
- Mechanical ventilation (negative-pressure) for alveolar hypoventilation
- Life support measures are discussed thoroughly with the patient’s family. Patients are encouraged to comply an advance directive or a will to help the family n the decision making.
Image by: yalemedicalgroup.org
Byron Webb Romero, RN, MSN
Finished BSN at Lyceum of the Philippines University, and Master of Science in Nursing Major in Adult Health Nursing at the University of the East Ramon Magsaysay Memorial Medical Center. Currently working at Manila Doctors College of Nursing as a Team Leader for Level I and II, Lecturer for Professional Nursing Subjects, and also a Clinical Instructor.
