Neuromuscular Disorders
Our neuromuscular team treats thousands of patients each year. We create research-based, personalized treatment plans designed to improve your quality of life and overall function.
Why choose us for neuromuscular disorder care?
BJC HealthCare works with WashU Medicine Physicians, BJC Medical Group, and providers across the region to deliver extraordinary care. Patients who visit our neuromuscular team have access to a full range of advanced services. We work with multiple specialists to ensure you receive comprehensive care to increase your quality of life with a neuromuscular disorder.
We offer:
- Access to specialists: Neuromuscular disorders can affect multiple aspects of your health. Our neuromuscular specialists work with neurorehabilitation therapists, pathologists, cardiologists, orthotics experts, and social workers. This team approach helps ensure you receive the full range of services you need.
- Recognized expertise: The Muscular Dystrophy Association (MDA) has designated Barnes-Jewish Hospital as an Amyotrophic Lateral Sclerosis (ALS)/MDA clinic. This designation highlights our holistic, comprehensive care that includes electrodiagnostic testing, nutrition services, wheelchair evaluations, and more.
- Extensive experience: The neurologists who perform electrodiagnostic studies are all board-certified and have expertise in both electrodiagnosis and neuromuscular diseases. They consult on nerve and muscle disorders, including whether you could benefit from electrodiagnostic testing.
- Subspecialty care: If necessary during your care, you'll have access to advanced subspeciality care. We treat a range of rare disorders, such as inclusion body myositis, amyloid disorders, and spinal muscular atrophy.
What is a neuromuscular disorder?
Types of muscular dystrophy and neuromuscular diseases
What are the types of muscular dystrophy?
Neuromuscular disorders are conditions that affect the nerve, muscle, or neuromuscular junction (where the nerve talks to the muscle). Muscular dystrophy is a group of inherited diseases that causes weakness. They also cause wasting away of muscle tissue. There are multiple types of muscular dystrophy. Each type leads to loss of strength, increasing disability, and possible deformity.
The most common muscular dystrophy is Duchenne muscular dystrophy. The next most common is Becker muscular dystrophy.
Listed below are nine major types of muscular dystrophy. Each type differs in the muscles affected, the age of onset, and its rate of progression. The following are named for the affected muscles:
| Type | Age at onset | Symptoms, rate of progression, and life expectancy |
|---|---|---|
| Becker | Teen to early adulthood | Symptoms are almost the same as Duchenne, but less severe. It progresses more slowly than Duchenne. Survival goes into middle age. Becker disease is almost always limited to males. This is the same as with Duchenne. |
| Congenital | Birth | Symptoms include general muscle weakness and possible joint deformities. The disease progresses slowly. It causes a shortened life span. |
| Duchenne | Ages 2 to 6 | Symptoms include general muscle weakness and wasting. It affects the pelvis, upper arms, and upper legs. Over time, it includes all voluntary muscles. Survival beyond the 20s is rare. It happens mostly in boys. Very rarely it can affect women, who have much milder symptoms and a better prognosis. |
| Distal | Ages 40 to 60 | Symptoms include weakness and wasting of muscles of the hands, forearms, and lower legs. Progression is slow. It rarely leads to total disability. |
| Emery-Dreifuss | Childhood to early teens | Symptoms include weakness and wasting of shoulder, upper arm, and shin muscles. Joint deformities are common. Progression is slow. Sudden death may occur from cardiac problems. |
| Facioscapulohumeral | Childhood to early adults | Symptoms include facial muscle weakness and weakness with some wasting of shoulders and upper arms. Progression is slow with periods of rapid deterioration. Life span may be many decades after onset. |
| Limb-Girdle | Late childhood to middle age | Symptoms include weakness and wasting, affecting shoulder girdle and pelvic girdle first. Progression is slow. Death is usually due to cardiopulmonary complications. |
| Myotonic | Ages 20 to 40 | Symptoms include weakness of all muscle groups and delayed relaxation of muscles after contraction. It affects the face, feet, hands, and neck first. Progression is slow, sometimes spanning 50 to 60 years. An infantile form causes more severe problems. |
| Oculopharyngeal | Ages 40 to 70 | Symptoms affect muscles of eyelids and throat causing weakening of throat muscles. Over time, this causes inability to swallow and severe weight loss from lack of food. Progression is slow. |
What are other neuromuscular diseases?
| Disease | Disease |
|---|---|
Motor neuron diseases:
Inflammatory myopathies:
Diseases of peripheral nerve:
Diseases of the neuromuscular junction:
| Metabolic diseases of the muscle:
Less common myopathies:
|
Search for a neuromuscular disorder specialist
Our highly trained team of specialists offers advanced treatments as well as compassionate, personalized care.
Testing for neuromuscular disorders
Electrodiagnostic testing can help establish a diagnosis for disorders affecting nerves and muscles. It also helps distinguish between problems that are caused by peripheral nerves (nerves outside the brain and spinal cord) from those that are caused by a disorder in the central nervous system (nerves within the brain and spinal cord).
Each year, thousands of patients receive electrodiagnostic testing with our neuromuscular experts. Available tests include:
- Autonomic testing
- Electromyography (EMG)
- Nerve and muscle ultrasound
- Nerve conduction studies
- Quantitative sensory testing (QST)
- Repetitive nerve stimulation
- Single-fiber EMG