Limb-girdle muscular dystrophy
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| Limb-girdle muscular dystrophy Classification and external resources | |
| ICD-9 | 359.1 |
|---|---|
| DiseasesDB | 32189 |
| eMedicine | neuro/189 |
| MeSH | D049288 |
Limb-girdle muscular dystrophy or Erb's muscular dystrophy is a type of muscular dystrophy that includes Duchenne muscular dystrophy, Becker's muscular dystrophy, and a large number of rarer disorders.
The term "limb-girdle" is used to describe these disorders because the muscles most severely affected are generally those of the hips and shoulders -- the limb girdle muscles.
Common symptoms of limb-girdle muscular dystrophy are muscle weakness, myoglobinuria, pain, myotonia, cardiomyopathy, elevated serum CK, and rippling muscles.
The muscle weakness is generally symmetric, proximal, and slowly progressive.
Generally pain is not present with LGMD, and mental function is not affected.
LGMD can begin in childhood, adolescence, young adulthood or even later. The age of onset is usually between 10 and 30. Both genders are affected equally. When limb-girdle muscular dystrophy begins in childhood the progression appears to be faster and the disease more disabling. When the disorder begins in adolescence or adulthood the disease is generally not as severe and progresses more slowly.
The distal muscles are affected late in LGMD, if at all. Over time (usually many years), the person with LGMD loses muscle bulk and strength. Eventually, he may need a power wheelchair or scooter, especially for long distances.
While LGMD isn't a fatal disease, it may eventually weaken the heart and lung muscles, leading to illness or death due to secondary disorders.
LGMD is typically an inherited disorder, though it may be inherited as a dominant, recessive, or X-linked genetic defect. The result of the defect is that the muscles cannot properly form the proteins needed for normal muscle function. Several different proteins can be affected, and the specific protein that is absent or defective identifies the specific type of muscular dystrophy.
Treatment for LGMD is primarily supportive. Exercise and physical therapy are advised to maintain as much muscle strength and joint flexibility as possible. Caliphers may be used to maintain mobility and quality of life. Careful attention to lung and heart health is also required.
List of limb-girdle muscular dystrophies
- LGMD1A
- LGMD1B
- LGMD1C
- LGMD1D
- LGMD1E
- LGMD1F
- LGMD2A
- LGMD2B
- LGMD2C
- LGMD2D
- LGMD2E
- LGMD2F
- LGMD2G
- LGMD2H
- LGMD2I
- LGMD2J
External links
Muscular Dystrophy | |
|---|---|
| The Nine Primary Muscular Dystrophies | Congenital • dystrophin (Becker's, Duchenne) • Distal • Emery-Dreifuss • Facioscapulohumeral • Limb-girdle muscular dystrophy • Myotonic • Oculopharyngeal |
| Related topics | |
| National/International Organizations | Muscular Dystrophy Association (USA) . Muscular Dystrophy Canada |
| US government Institutes and Legislation | NINDS • NIAMS • NICHD • MD CARE Act • Genetic Information Nondiscrimination Act • Americans with Disabilities Act of 1990 |
| National/International Events | Jerry Lewis MDA Telethon (USA) |
| Recent or Ongoing Clinical Trials | Stamulumab (MYO-029) |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
