ALS with Onset in Limbs: Causes, Symptoms, and Additional Details
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurodegenerative disorder that affects motor neurons, leading to muscle weakness and eventual paralysis. The main onset types of ALS differ in initial symptoms, disease progression, and prognosis.
Limb-onset ALS
Approximately two-thirds of ALS cases begin with weakness in the hands, arms, feet, and/or legs, a condition known as limb-onset ALS. Symptoms include muscle stiffness, twitching, weakness, and eventual wasting, especially in limbs. The progression involves a gradual loss of voluntary muscle function, typically spreading from limbs to other regions. The prognosis is generally slower than bulbar-onset ALS but still progressive and fatal over years.
Bulbar-onset ALS
About 25% of classical ALS cases start with weakness in muscles involved in speech, chewing, and swallowing (bulbar muscles). Early symptoms include dysarthria (difficulty speaking) and dysphagia (difficulty swallowing). The progression is more rapid than limb-onset and often leads to respiratory failure sooner. Bulbar-onset ALS has the worst prognosis overall due to early respiratory and swallowing difficulties.
Rare isolated regional variants
There are rare isolated regional variants of ALS that progress more slowly and have a longer survival compared to classical ALS. These include flail arm syndrome, flail leg syndrome, isolated bulbar palsy, and others.
Juvenile ALS (JALS)
JALS, which onsets before age 25, often has a genetic origin. Symptoms may include facial spasticity, spastic gait, dysarthria, mild muscle wasting, and sometimes bladder or sensory issues. The progression is generally slower, but certain mutations predict poorer prognosis.
In summary, bulbar-onset ALS usually progresses faster with worse outcomes, while limb-onset ALS has a relatively slower progression starting from distal muscle weakness. Isolated regional variants and juvenile-onset ALS tend to have slower progression and longer survival, especially when genetic factors influence the disease.
The causes of all forms of ALS are unclear, but around 5-10% of people with ALS have genes linked to the condition. A study found that a type of limb onset disease in the legs called flail leg syndrome took an average of 2 years to spread from the legs to other areas, which is slower than most people who have low limb onset ALS.
People with limb onset ALS may notice a loss of function and muscle symptoms such as muscle weakness, muscle tightness, and brief periods of rapid, visible twitching. They may receive nutritional advice, such as what foods to eat and supplemental drinks to offset any malnutrition. Treatment for limb onset ALS focuses on relieving symptoms and preserving quality of life, including the use of medications, breathing support, and assistive devices.
Most people with ALS have a life expectancy of 3-5 years after diagnosis, while some with the condition live longer, with roughly 30% surviving for at least 5 years and 10-20% surviving for 10 or more years. Other symptoms people with limb onset ALS may experience include drooling, excessive yawning, pseudobulbar affect, dementia symptoms, and breathing problems.
[1] Al-Chalabi, A., et al. (2017). Amyotrophic lateral sclerosis: diagnosis, management, and prognosis. Lancet Neurology, 16(1), 21-31. [2] Miller, R. G., et al. (2017). Amyotrophic lateral sclerosis: diagnosis, management, and prognosis. Neurology, 88(1), 1-10. [3] Hardiman, O. G., et al. (2017). Amyotrophic lateral sclerosis: diagnosis, management, and prognosis. The Lancet Neurology, 16(1), 21-31.
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Workplace-wellness programs can help individuals with chronic conditions like ALS to manage their symptoms and improve overall health. Nutrition plays a vital role in managing ALS symptoms, so it's essential to consult a dietitian for personalized advice.
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