# Amyotrophic Lateral Sclerosis --- **Amyotrophic lateral sclerosis** or **ALS** a is a disease of unknown cause in which there is a loss of motor neurons (nerve cells controlling muscles) in the anterior horns of the spinal cord and the motor nuclei of the lower brainstem. It is often referred to as Lou Gehrig disease, after the famous baseball player who suffered from the disease. As motor neuron cells die, the muscle fibers that they supply undergo atrophic changes. Neuronal degeneration may occur in both the upper and lower motor neuron systems (see Chapter 60). The leading theory held by researchers is that overexcitation of nerve cells by the neurotransmitter glutamate results in cell injury and neuronal degeneration it is progressive A mixed upper & lower mo r neuron disease Degeneration of distal ter inals of neurons first; progresses to ard proximal Results in denervation & ev ntual death of motor units Muscles eventually shrink; uscle fibers atrophy (amyotrophy) Loss of nerve fibers in lateral columns of spinal cord causes firmness of CNS tissue (lateral sclerosis) ## Risk Factors/Cause ## Signs & Symptoms The signs and symptoms of amyotrophic lateral aclerosis Clinical manifestations depend on the location of the affected motor neurons, because specific neurons activate specific muscle fibers. The chief symptoms are fatigue, progressive muscle weakness, cramps, fasciculations (twitching), and lack of coordination. Loss of motor neurons in the anterior horns of the spinal cord results in progressive weakness and atrophy of the muscles of the arms, trunk, or legs. Spasticity usually is present, and the deep tendon stretch reflexes become brisk and overactive. Usually, the function of the anal and bladder sphincters remains intact, because the spinal nerves that control muscles of the rectum and urinary bladder are not affected. ## Complications ## Diagnostic Tests ## Treatment Treatment for amyotrophic lateral aclerosis consists of No cure exists for ALS (Vacca, 2020). The main focus of medical and nursing management is on interventions to maintain or improve function, well-being, and quality of life Two drugs are used to treat ALS, riluzole and edaravone (Vacca, 2020). The precise action of these drugs is not clear, but both are considered disease modifying treatments for ALS ## Nursing Considerations ___