What is kennedy disease?
Kennedy’s disease is a rare inherited neuromuscular disorder that causes progressive weakening and wasting of the muscles, particularly the arms and legs. Other major symptoms include severe cramps and problems with speech and swallowing. The disease progresses slowly, and life expectancy is usually normal.
What is spinal muscular atrophy?
Spinal muscular atrophy is a genetic disorder characterized by weakness and wasting (atrophy ) in muscles used for movement (skeletal muscles). It is caused by a loss of specialized nerve cells, called motor neurons that control muscle movement.
How is spinal muscular atrophy inherited?
Spinal muscular atrophy (SMA) is the second leading cause of neuromuscular disease. It is usually inherited as an autosomal recessive trait (a person must get the defective gene from both parents to be affected). There are several types of SMA called subtypes.
Is Kennedy disease fatal?
Treatment is symptomatic and supportive, and life expectancy is normal, though a small percentage of patients (~ 10%) succumb to the disease in their 60’s or 70’s due to swallowing complications (aspiration pneumonia, asphyxiation) resulting from the bulbar weakness. Kennedy disease is named after William R.
How long can you live with Kennedy’s disease?
The life span of individuals with Kennedy’s disease is usually normal. Kennedy’s disease is slowly progressive. Individuals tend to remain ambulatory until late in the disease, although some may be wheelchair-bound during later stages. The life span of individuals with Kennedy’s disease is usually normal.
Does SMA run in families?
Most people have two copies of the SM1 gene – one from each parent. SMA normally only happens when both copies have the gene change. If only one copy has the change, there usually aren’t any symptoms. But that gene could be passed down from parent to child.
How is Kennedy’s disease treated?
Currently, there is no known treatment or cure for Kennedy disease. Physical therapy, occupational therapy, and speech therapy are commonly used to adapt to the progressing disease and maintain an individual’s skills.
What is the difference between Kennedy’s disease and ALS?
A key feature that distinguishes Kennedy’s disease from ALS is the absence of involvement of upper motor neurons, which causes increased reflexes and spasticity in ALS.
What are symptoms of PMA?
What are the Symptoms of Progressive Muscular Atrophy?
- Atrophy.
- Muscle weakness.
- Lack of reflexes.
- Lack of spasticity.