Many baseball enthusiasts would have heard the name Lou Gehrig as he was diagnosed with ALS in 1930’s. Hence, it is also known as Lou Gehrig disease.
Basically what happens in our body is that the special neurons for actions (motor neurons) in the brain sends signals to our muscles to do various actions and then the muscles can perform those actions such as walk, run, chew, swallow, breath, pickup your smart phone.
So in ALS these neurons in brain & spinal cord are affected and so they can’t send these signals to the muscle. So gradually over a period of time if the muscles stop working they become useless (atrophic) and the strength or power of muscle decreases.
What really happens?
- As your muscles stops doing work it becomes weak and stiff
- Initially there may be trouble in buttoning a shirt or turning a key
- One may fall more than usual while walking
- As the disease progresses it becomes difficult to move arms, legs, body, and at one point even breathing
- Slurring of speech
- Muscle cramps twitching in your arms, shoulders & tongue
- Difficulty in maintaining good posture or holding up the head
It usually starts in hand, feet and then spread to other body parts.
ALS does not affect bowel & bladder control or your thinking ability. Also, sense of touch, smell, sight, taste & hearing are retained.
What cause ALS?
To a large extent no one knows the reason for ALS although, 5-10% of the cases could be inherited
No one test can provide definitive diagnosis of ALS. Thus, a battery of tests may be needed to come to diagnosis. It is primarily diagnosed based on the detailed history of the symptoms and signs.
Complete neurological examination by physician is warranted.
- MRI – It is used to rule out any tumors or space occupying lesions causing similar symptoms.
- Blood & urine tests – As a part of routine tests.
- Electromyogram – Basically this test measures the signals received by muscles for performing various actions.
- Nerve conduction study – This test shows the doctors whether there is defect in the nerve, so that the signals are hampered.
- Muscle biopsy – If the various tests points towards some muscle disease then a muscle biopsy may be advised.
ALS has no cure as of now, but the main aim towards treatment is that the patients should be able to retain the strength of muscle and avert the progression of the disease.
The main aim of the treatment is that patient should maintain chores independently as far as possible and thus improve the quality of life.
- Medication – Few medications such as Riluzole and Edaravove have limited utility.
- Other medication – various drugs are given to relive the symptoms of cramps, spasms, spasticity, pain, fatigue & depression
- Therapy sessions – Different kinds of therapies are prescribed to support life such as –
- Breathing therapy – Eventually with time it becomes difficult to breath and one may need a mechanical ventilator as well.
- Physical – Low impact exercises to maintain muscle strength, range motion so as to maintain independent life.
- Speech therapy – In later stages of ALS, speech may become slurred & hence a therapist may help adapt to speak more clearly
There are many clinical studies going on for new medications & treatment modalities for improving the outcome & quality of life for ALS patients.