Limb Girdle Muscular Dystrophy

Limb girdle muscular dystrophy (LGMD) causes weakness in shoulder and pelvic girdle. Weakness of legs is experienced before that of arms. The disease worsens with time. In some patients progression is more rapid as compared to others. Limb girdle muscular dystrophy can affect muscles of face also.

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Muscular dystrophy is a condition in which there is progressive weakening and wasting of muscles. There are different types of muscular dystrophy depending on the group of muscles involved.

Age Of Onset limb girdle muscular Dystrophy
Age of onset is usually between 10 and 30 years. Both genders are affected equally.

What Are The Causes Of Limb Girdle Muscular Dystrophy
There are genetic mutations in associated with limb girdle muscular dystrophy. The genes contain blueprints for proteins that are needed by the muscles to function properly. When a mutation in a LGMD gene occurs, muscles are not able to work properly and weakness occurs.

Sign And symptoms of Limb Girdle Muscular Dystrophy
• Difficulty in standing from a sitting position without the using hands.
• Difficulty climbing stairs.
• Difficulty in bending over.
• Ability to walk and run deteriorates slowly.

Complications Limb Girdle Muscular Dystrophy
• Contractures of joints.
• Abnormal rhythms of heart.
• Difficulty in performing daily activities due to shoulder weakness.
• Progressive weakness.

Treatment Of Limb Girdle Muscular Dystrophy
• Exercise and physical therapy are advised.
• Genetic counseling.
• Corticosteroids.
• Orthopedic surgery.

Diagnosis Of limb girdle Muscular Dystrophy
• DNA testing.
• ECG
• EMG
• Blood creatine kinase level
• Muscle biopsy.

Differential Diagnosis of limb girdle muscular dystrophy
• Congenital myopathies.
• Endocrine myopathies.
• Metabolic myopathies.
• Congenital muscular dystrophy.
• Spinal muscular atrophy.
• Emery-Dreifuss muscular dystrophy.
• Facioscapulohumeral dystrophy.
• Inclusion body myositis.
• Dermatomyositis.
• Dystrophinopathies.

Prevention of limb girdle muscular dystrophy
• Genetic counseling helps in guiding the families about risks and help in family planning.
• Appropriate treatment prevents some of the complications.
• Physical therapy helps in delaying or preventing joint contractures and thereby improves quality of life.
• Affected people are advised for DNA testing to identify family gene mutation. If mutation is found, testing for carriers, prenatal DNA testing and preimplantation genetic diagnosis is possible.

Prognosis of limb girdle muscular dystrophy
• Weakness worsens slowly.
• Loss of movement or patient becomes dependent on wheelchair within 20-30 years.
• If heart muscles get affected then risk of palpitation and fainting increases and sudden death also occurs.

Limb Girdle Muscular Dystrophy

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