The National Institute of Neurological Diseases and Stroke (NINDS) calls motor neuron diseases (MNDs) progressive, degenerative disorders that affect nerves in the upper or lower parts of the body. (Read about "Nervous System") Some are inherited, according to NINDS. Common MNDs include amyotrophic lateral sclerosis (ALS), which is better known as Lou Gehrig's disease, progressive muscular atrophy and post-polio syndrome. (Read about "Post-Polio Syndrome") Generally, MNDs strike in middle age. Symptoms may include difficulty swallowing, limb weakness, slurred speech, impaired gait, facial weakness and muscle cramps. (Read about cramps in "Feet, Ankles and Legs") Respiration may be affected in the later stages of these diseases. The cause(s) of most MNDs are not known, but environmental, toxic, viral or genetic factors are all suspects. Follow the alphabetical list below for more information on some common MNDs.
Adult Spinal Muscular Atrophy (SMA) - The spinal muscular atrophies (SMAs) are a group of genetics diseases that affect the voluntary muscles of the body. The Muscular Dystrophy Association (MDA) says they do not affect the involuntary muscles, like the ones that control our bladders and bowels. (Read about "The Urinary System") Almost always, the muscles that help us breathe are affected. Because of that, treatments often involve dealing with respiratory problems. (Read about "Respiratory System") SMAs differ mainly in the time of onset and how fast they progress, according to MDA. At this point, there are no cures for any of the SMAs. Adult SMA tends to show up in middle age, according to NINDS, and progresses rapidly. Symptoms include progressive limb weakness and atrophy of the muscles, difficulty speaking and swallowing, and respiratory problems. Many times, NINDS says, SMA is a form of amyotrophic lateral sclerosis. Genetic counseling is considered important for someone diagnosed with SMA, according to NINDS.
Amyotrophic Lateral Sclerosis (ALS) also known as Lou Gehrig's Disease - ALS is a progressive muscle wasting disease that strikes about 5,000 people each year in the United States, according to NINDS. It is commonly known as Lou Gehrig's disease. The New York Yankee first baseman died from ALS in 1941. NINDS says that some 20,000 people are living with the disease. ALS occurs when the nerve cells that control voluntary muscles are lost. As the nerve cells die off, the muscles weaken and waste away. Symptoms vary depending on which muscles weaken first. Eventually paralysis occurs. ALS usually shows itself in middle age, according to NINDS. Men are more likely to get ALS than women. About 10 percent of the time ALS appears to be inherited, according to MDA. The rest of the time the cause cannot be pinpointed. The disease tends to progress rapidly but many people survive for years with ALS. Recently new drugs have been approved that appear to help ALS patients.
Infantile Progressive Spinal Muscular Atrophy (SMA1) also known as SMA Type 1 or Werdnig-Hoffman - SMA Type 1 is also called Werdnig-Hoffmann disease. It shows up in the first few months of life and is considered the most severe, according to MDA. Symptoms, according to NINDS, may include a floppiness of the child, lack of movement in the arms and legs, swallowing, feeding and breathing problems. Affected children are not able to sit or stand. The disease progresses rapidly.
Intermediate Spinal Muscular Atrophy (SMA2) also known as SMA Type 2 - SMA Type 2 begins later than Type 1, anywhere from 3 months to 18 months. The symptoms are much the same as Type 1. The child may be able to sit up but will not be able to stand, according to MDA. The progression of the disease varies. The severity is dependent on the amount of respiratory problems. (Read about "Respiratory System")
Juvenile Spinal Muscular Atrophy (SMA3) also known as SMA Type 3 or Kugelberg-Welander - SMA Type 3 is also called Kugelberg-Welander after the doctors who first described it. It usually first appears between 5 and 15 years of age, according to MDA, but can appear as early as two. MDA calls it the milder form of the childhood SMAs. Symptoms mostly involve difficulties with leg motions and may include tremors in the fingers, according to NINDS. MDA says the greatest danger, if it occurs, is from respiratory problems. (Read about "Respiratory System")
Spinal Bulbar Muscular Atrophy (SBMA) also known as Kennedy's Disease or X-linked SBMA - SBMA is also called Kennedy's disease. SBMA is usually adult onset in nature but NINDS says it can occur as early as 15 years of age. Symptoms include weakness in the limbs and the face resulting in difficulty in lifting heavy objects, climbing stairs or just standing up. NINDS adds there may also be weakness of the facial and tongue muscles, resulting in difficulty swallowing and speech impairment. The disease also impacts the production of male hormones and can result in such things as enlarged breasts, infertility (Read about "Infertility") and possible shrinkage of the testes, according to MDA. The disease progresses slowly. SBMA usually only affects men, according to MDA. The gene that is responsible for the disease is passed on by the mother.
According to NINDS, there is currently no cure or standard treatment for the MNDs. Symptomatic and supportive treatment can help patients be more comfortable while maintaining their quality of life.
Adult Spinal Muscular Atrophy (SMA) - The spinal muscular atrophies (SMAs) are a group of genetics diseases that affect the voluntary muscles of the body. The Muscular Dystrophy Association (MDA) says they do not affect the involuntary muscles, like the ones that control our bladders and bowels. (Read about "The Urinary System") Almost always, the muscles that help us breathe are affected. Because of that, treatments often involve dealing with respiratory problems. (Read about "Respiratory System") SMAs differ mainly in the time of onset and how fast they progress, according to MDA. At this point, there are no cures for any of the SMAs. Adult SMA tends to show up in middle age, according to NINDS, and progresses rapidly. Symptoms include progressive limb weakness and atrophy of the muscles, difficulty speaking and swallowing, and respiratory problems. Many times, NINDS says, SMA is a form of amyotrophic lateral sclerosis. Genetic counseling is considered important for someone diagnosed with SMA, according to NINDS.
Amyotrophic Lateral Sclerosis (ALS) also known as Lou Gehrig's Disease - ALS is a progressive muscle wasting disease that strikes about 5,000 people each year in the United States, according to NINDS. It is commonly known as Lou Gehrig's disease. The New York Yankee first baseman died from ALS in 1941. NINDS says that some 20,000 people are living with the disease. ALS occurs when the nerve cells that control voluntary muscles are lost. As the nerve cells die off, the muscles weaken and waste away. Symptoms vary depending on which muscles weaken first. Eventually paralysis occurs. ALS usually shows itself in middle age, according to NINDS. Men are more likely to get ALS than women. About 10 percent of the time ALS appears to be inherited, according to MDA. The rest of the time the cause cannot be pinpointed. The disease tends to progress rapidly but many people survive for years with ALS. Recently new drugs have been approved that appear to help ALS patients.
Infantile Progressive Spinal Muscular Atrophy (SMA1) also known as SMA Type 1 or Werdnig-Hoffman - SMA Type 1 is also called Werdnig-Hoffmann disease. It shows up in the first few months of life and is considered the most severe, according to MDA. Symptoms, according to NINDS, may include a floppiness of the child, lack of movement in the arms and legs, swallowing, feeding and breathing problems. Affected children are not able to sit or stand. The disease progresses rapidly.
Intermediate Spinal Muscular Atrophy (SMA2) also known as SMA Type 2 - SMA Type 2 begins later than Type 1, anywhere from 3 months to 18 months. The symptoms are much the same as Type 1. The child may be able to sit up but will not be able to stand, according to MDA. The progression of the disease varies. The severity is dependent on the amount of respiratory problems. (Read about "Respiratory System")
Juvenile Spinal Muscular Atrophy (SMA3) also known as SMA Type 3 or Kugelberg-Welander - SMA Type 3 is also called Kugelberg-Welander after the doctors who first described it. It usually first appears between 5 and 15 years of age, according to MDA, but can appear as early as two. MDA calls it the milder form of the childhood SMAs. Symptoms mostly involve difficulties with leg motions and may include tremors in the fingers, according to NINDS. MDA says the greatest danger, if it occurs, is from respiratory problems. (Read about "Respiratory System")
Spinal Bulbar Muscular Atrophy (SBMA) also known as Kennedy's Disease or X-linked SBMA - SBMA is also called Kennedy's disease. SBMA is usually adult onset in nature but NINDS says it can occur as early as 15 years of age. Symptoms include weakness in the limbs and the face resulting in difficulty in lifting heavy objects, climbing stairs or just standing up. NINDS adds there may also be weakness of the facial and tongue muscles, resulting in difficulty swallowing and speech impairment. The disease also impacts the production of male hormones and can result in such things as enlarged breasts, infertility (Read about "Infertility") and possible shrinkage of the testes, according to MDA. The disease progresses slowly. SBMA usually only affects men, according to MDA. The gene that is responsible for the disease is passed on by the mother.
According to NINDS, there is currently no cure or standard treatment for the MNDs. Symptomatic and supportive treatment can help patients be more comfortable while maintaining their quality of life.