ALS or Amyotrophic Lateral Sclerosis is a progressive, fatal, neurodegenerative disease, which is caused by the degeneration of motor neurons. Motor neurons travel from the brain to the spinal cord and from the spinal cord to the muscles of the body. ALS causes the motor neurons to degenerate and eventually die. Motor neurons form an important part of the neuromuscular system. The neuromuscular system is responsible for executing our every day functions, such as walking, lifting stuff etc. When the motor neurons die, the brain becomes incapable of sending impulses to the muscle fibers to control and initiate muscle movement. As the muscles no longer receive any messages from the motor neurons, they slowly start to atrophy. A patient in the last stages of this disease may become completely paralyzed. Although there are no treatments or cure for this disease which can either reverse or halt the process, there is an FDA approved drug, riluzole which slows down the progression of ALS. The disease can affect people of any age, but it is extremely rare in case of kids. At the onset of the disease, the symptoms are so subtle that they can be easily overlooked. In the following lines, we have provided a detailed information about the common signs and symptoms of ALS disease.
Signs & Symptoms Of ALS Disease
Early symptoms of this disease vary from individual to individual. Initial symptoms may include weakness and muscle atrophy. Which body part is affected first depends upon which motor neurons of the body are damaged first. In case of most people muscle weakness typically begins from arms and legs and generally spreads to the rest of the body. This is usually followed by twitching, cramping and stiffness of the affected muscles. In initial stages patients may experience problem while walking, running, lifting things or speaking. The patient will find it difficult to perform simple day to day tasks, requiring manual dexterity like tying shoelaces, buttoning a shirt or writing.
Typical speech problems include nasality, loss of volume, slurred or garbled speech. These problems may later aggravate with loss of mobility of the tongue and difficulty in swallowing. As the disease progresses, the patient faces increasing difficulty in moving, swallowing and speaking. In some cases the symptoms remain confined to one limb. This is known as monomelic amyotrophy. The patient eventually becomes restricted to bed or wheelchair. About 15-45% patients experience pseudobulbar effect, which results in exaggeration of motor expression of emotions manifesting in the form of involuntary uncontrollable periods of laughing or crying.
The intensity and sequence of symptoms of ALS may differ, but eventually all patients find it difficult to walk and stand on their own or use their hands and arms. The patients have difficulty in chewing and swallowing which increases the risk of choking. The patients can no longer eat properly, which causes difficulty in maintaining weight. Since the disease doesn’t affect the cognitive functions of the patients, they are usually aware of their loss of bodily functions which further leads to depression. A small percentage of patients with a family history of dementia develop frontotemporal dementia, which results in profound personality changes in the patient. Forced vital capacity and inspiratory pressure diminishes with weakening of the ribcage. Bi-level positive pressure ventilation becomes a necessity to support breathing first during nights and later at daytime as well. After the breathing muscles are affected, the patient requires permanent ventilator support for survival.