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Spinal Muscular Atrophy |
hereditary motor neuronopathy; Progressive Muscular Atrophy; SMA |
Spinal muscular atrophy (SMA) is a genetic, motor neuron disease caused by progressive degeneration of motor neurons in the spinal cord. The disorder causes weakness and wasting of the voluntary muscles. Weakness is often more severe in the legs than in the arms. The childhood SMAs are all autosomal recessive diseases. This means that they run in families and more than one case is likely to occur in siblings or cousins of the same generation. Parents usually have no symptoms, but still carry the gene. The gene for SMA has been identified and accurate diagnostic tests exist. There are many types of SMA; some of the more common types are described below.
... Spinal muscular atrophy affects the control of muscle movement and is caused by a loss of specialized nerve cells (motor neurons) in the spinal cord and the part of the brain that is connected to the spinal cord (brain stem). The loss of motor neurons leads to weakness and shrinkage (atrophy) of voluntary muscles used for activities such as crawling, walking, sitting up, and controlling head movement.
Spinal muscular atrophy is divided into subtypes based on the severity and age of onset of symptoms. Type I (also called Werdnig-Hoffman disease) is the most severe form of spinal muscular atrophy. Symptoms are evident at birth or within the first few months of life. Affected infants usually have difficulty breathing and swallowing, and they are never able to sit or stand. Children with type II spinal muscular atrophy usually develop muscle weakness between 6 and 12 months of age. Type II children can sit, although they cannot stand or walk unaided.
Type III spinal muscular atrophy (also called Kugelberg-Welander disease or juvenile type) is the least severe form, and patients are able to stand and walk unaided. Symptoms appear between early childhood (after age 18 months) and early adulthood. Type IV or adult-onset spinal muscular atrophy typically begins after age 30, and symptoms can range from very mild to very severe. ...
SMA is a TLA that may stand for: Supplementary motor area San Manuel Arizon ...
From the WEST scientific·clinical |
From the EAST traditional·alternative |
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Spinal Muscular Atrophy Other1-2 of 6 more...
Spinal Muscular Atrophy
... Spinal muscular atrophy (SMA) is a genetic, motor neuron disease that is the second most common neuromuscular disorder of childhood, second only to Duchenne’s muscular dystrophy. It is characterized b...
Source: Cleveland Clinic
Kennedy''s Disease Information Page ... Kennedy's disease is an inherited motor neuron disease that affects males. It is one of a group of disorders called spinal muscular atrophy (SMA). Onset of the disease is usually between the ages ...
Source: National Institute of Neurological Disorders and Stroke
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Spinal Muscular Atrophy Articles
- Genes and Disease: Spinal muscular atrophy
... Death of spinal motor neurons and subsequent muscle paralysis characterize Spinal Muscular Atrophy (SMA), a hereditary neuromuscular disorder that is the most common genetic cause of childhood fatalit... - Kennedy's Disease Information Page
... Kennedy's disease is an inherited motor neuron disease that affects males. It is one of a group of disorders called spinal muscular atrophy (SMA). Onset of the disease is usually between the ages ... - Spinal and bulbar muscular atrophy
... Spinal and bulbar muscular atrophy is a disorder of specialized nerve cells that control muscle movement (motor neurons). The condition, which mainly affects males, is characterized by muscle weakness... - Spinal muscular atrophy
... Spinal muscular atrophy affects the control of muscle movement and is caused by a loss of specialized nerve cells (motor neurons) in the spinal cord and the part of the brain that is connected to the ... - Spinal Muscular Atrophy Information Page
... Spinal muscular atrophy (SMA) is a genetic, motor neuron disease caused by progressive degeneration of motor neurons in the spinal cord. The disorder causes weakness and wasting of the voluntary muscl...
Resources
- About Kennedy's Disease: Genetic Counseling/Inheritance (Kennedy's Disease Association)
- About Kennedy's Disease: KD Symptoms (Kennedy's Disease Association)
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