ALS symptoms are seen in Amyotrophic Lateral Sclerosis, also known as Motor Neurone Disease or Lou Gehrig’s disease.
It is a degenerative disorder characterized by progressively worsening weakness, muscle atrophy and spasticity, along with associated typical ALS symptoms like labored breathing, speaking and swallowing difficulty.
What causes ALS symptoms
The main cause of ALS symptoms is demyelination and degeneration of the upper and lower motor neurons, which causes loss of voluntary movement. This leads to weakening and atrophy of the skeletal muscles, progressively spreading to all the muscles. ALS symptoms tend to not include bowel or bladder muscles, nor the muscles responsible for eye movements. Similarly, ALS symptoms show sparing of mental function, although signs of dementia may be seen in a small fraction of people.
ALS Symptoms break down
The first of the ALS symptoms to become apparent is muscle weakness, leading to atrophy. This is accompanied by twitching and cramping of muscles, stiffness in one or both limbs depending on which motor neurons are involved. ‘Leg-onset” ALS symptoms are characterized by clumsiness in walking, a shuffling or dragging gait with the affected leg lagging behind, or a dropped foot. “Arm-onset” ALS symptoms manifest with difficulty in tasks requiring fine motor skills first, such as sewing or writing. Next the gross hand movements are affected, which makes the disease extent more apparent.
Another type are ‘bulbar-onset’ ALS symptoms , showing first dysphagia and dysarthria, that is, difficulty in swallowing and in forming words, which progressively gets worse over time. This is accompanied by spastic muscles and an abnormal reflex called Babinski’s Sign seen in the foot. Exaggerated reflexes and exaggerated emotions are also a feature resulting from motor neuron degeneration, more severely seen in women.
While ALS symptoms progress at a different rate from case to case, most patients end up not being able to walk as the disease process intensifies. A standard measure of the progression is the ALS Functioning Rating Scale, which assigns FRS points from 48 to 0, with 0 being Severely Disabled. Spread of disease is generally slower in Limb Onset ALS, and much more rapid in Bulbar Onset ALS.
In the later stages, the lack of control over muscles required for chewing and swallowing predisposes to choking and aspiration of food into lungs. Therefore the latter stage ALS symptoms include drastic weight loss and aspiration pneumonia, which will necessitate a feeding tube and hospitalization. Management of ALS symptoms in the final stages of disease requires hospitalization and respiratory support.