ALS is otherwise also known by its full name amyotrophic lateral sclerosis. It is a type of progressive neurodegenerative disease that affects upper and lower motor neurons in the brain and the spinal cord. It comes from the Greek language where “A” means no. “Myo” refers to muscle, and “Trophic” means nourishment – “No muscle nourishment.” The motor neurons are responsible for initiating voluntary muscle movements. They also act as a bridge between the brain and the muscles for crucial communication. So, as the name suggests, when a muscle does not have any nourishment, it “atrophies” or wastes away. As the motor neurons degenerate, it leads to a hardened area in the brain or scarring which is also known as “sclerosis”.
Physical Presentations of ALS:
ALS involves the voluntary movement of muscles. So muscle weakness, muscle cramps, inability to perform daily activities, are the main features. With tongue involvement, a person will also feel difficulty in chewing or swallowing or even slurred speech. ALS appear very gradually with weakness in one arm or legs. With the progression of the disease and involvement of other body areas, it becomes apparent of “something wrong” inside one’s body. Cognition is usually intact or minimally affected by ALS patients.
How to Diagnose ALS:
Unfortunately, there is no one test to diagnose the test. It is usually a combination of clinical symptoms, physician and neurologist’ assessment, along with a few series of medical tests that diagnosis the disease and confirms it. Few common medical tests that are usually done are EMG (electromyography), NCV ( Nerve conduction velocity test), X rays, MRI, blood test, urine tests, muscle and nerve biopsy, spinal tap, etc.
How to treat ALS:
Medications like Rilutek (Riluzole) and Radicava ( edaravone) are usually prescribed to treat ALS. Medicines can also help with muscle tightness, cramps, pain, depression, etc.
Proper nutritional support is crucial in patients with ALS. In a person with impaired swallowing or chewing, a physician or a dietitian may consider other nutritional options like PEG tube.
A person with impaired respiratory muscles may need respiratory support during the night or throughout the day. This may start with needing only add oxygen to CPAP to full ventilatory support. Respiratory therapy work on managing secretions in persons with respiratory muscles involvement.
Rehabilitation Approach :
Rehabilitation is a key to maintain muscle function, manage muscle stiffness and tone, as well as to limit other clinical symptoms. It can be in the form of physical, occupational and speech therapy.
Since a majority of ALS patients have intact cognition, educating and teaching new compensatory skills are important rehabilitation approaches.
This includes but not limited to educating about the disease progression, using different adaptive types of equipment for activities like walking, feeding, dressing etc, education regarding proper and safer positioning in bed or in a chair, using wheelchairs or motorized scooter for independence. Family and caregiver education, as well as training, is also crucial with ALS to help and support a patient in the right way.
In addition, maintaining and improving the range of motion is necessary. AAROM, AROM, or strengthening exercises are good options to improve range of motion. Physical therapy can target pain management and functional independence. A therapist can provide different braces like AFO or KAFO etc that can aid in functional mobility.
Physical and Occupational therapy can help identify the proper means of transport. They can measure and train a patient for appropriate wheelchair or motorized scooter. With appropriate positioning and independence in propulsion, therapists can aid in independent mobility as well as preventing skin breakdowns.
Speech therapy usually aims at improving swallowing function, strategies to clearing out the secretions and improving breathing techniques. Patient with respiratory muscles involvement, speech therapy, and respiratory therapy work together to provide respiratory support and improve respiratory function.
Prognosis :
ALS is a progressive disease. The prognosis of a disease depends on the age of onset, race, the progression of the disease and areas affected. Most people with ALS die from respiratory failure, usually within 3 to 5 years from when the symptoms first appear. However, about 10 percent of people with ALS survive for 10 or more years.