Muscular dystrophy - late onset

Many people think that muscular dystrophy is exclusively a childhood disorder. However, it can occur at any point in a person’s life span. There are four types of muscular dystrophy which can occur later in life:

• Limb-girdle muscular dystrophy
• Congenital muscular dystrophy
• Opthalmoplegic muscular dystrophy
• Distal muscular dystrophy.

Muscle degeneration leads to increasing weakness

In the more severe forms of the disease, affected people:

• Will need to use a wheelchair full time
• Are unable to carry out even the simplest of daily activities
• Develop recurrent infections that they cannot fight
• Usually die as a result of respiratory disease. Involvement of the heart muscle may also contribute.

Earlier onset usually leads to more severe disability

There is considerable variation in the severity of muscle degeneration among the various types of muscular dystrophy. Generally, the earlier the clinical signs appear, the more rapid the progression and the more muscles that are involved.

Limb-girdle muscular dystrophy

Limb-girdle muscular dystrophy usually occurs in the first to third decades of life and involves:

• The proximal (back of the body) muscles of the pelvis and shoulders
• Slow to fairly rapid progressive muscle deterioration
• A possible normal life span, if the muscle deterioration progresses slowly.

People with this form of muscular dystrophy have generally inherited the defective gene from both parents.

Congenital muscular dystrophy

In this form of muscular dystrophy:

• The most active phase of muscle degeneration occurs before the fetus is born.
• Effects are apparent at birth. Signs include muscle weakness, hypotonia (poor muscle tone) and joint contractures.
• Some improvement occurs in childhood and the disease shows little or no progression.
• Both parents probably carry the defective gene.

Ophthalmoplegic muscular dystrophy

This form of muscular dystrophy:

• Affects the extraocular (eye) muscles and muscles associated with swallowing
• Usually occurs in adulthood
• Leads to drooping eyelids.

Distal muscular dystrophy

This is the rarest of the muscular dystrophies, although its incidence is comparatively high in Sweden. It affects the small muscles of the extremities.

Treatment for muscular dystrophy

There is no cure for muscular dystrophy, but the use of orthopaedic devices and physiotherapy can:

• Keep affected people walking for longer
• Minimise crippling joint contractures
• Prevent or delay scoliosis (curvature of the spine)
• Ease discomfort
• Maximise independence in daily activities.

Where to get help

• Your doctor
• Muscular Dystrophy Association Tel. (03) 9320 9555

Things to remember

• Muscular dystrophy is not just a disease of childhood.
• There are several forms of muscular dystrophy.
• Generally, the earlier the onset of muscular dystrophy, the more severe the disability.

You might also be interested in:

• Infantile spinal muscular atrophy.
  
• Muscular dystrophy - Duchenne and Becker.
  
• Muscular dystrophy - the face and shoulders.
  
• Muscular dystrophy explained.
  
• Neuromuscular system.
  

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Last reviewed: June 2011

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Muscular dystrophy is the name given to a group of inherited muscle diseases that cause progressive degeneration and weakness of voluntary muscles. Muscular dystrophy can occur at any age. Limb-girdle muscular dystrophy, congenital muscular dystrophy, ophthalmoplegic muscular dystrophy and distal muscular dystrophy occur in later life.

Content on this website is provided for education and information purposes only. Information about a therapy, service, product or treatment does not imply endorsement and is not intended to replace advice from your qualified health professional. Content has been prepared for Victorian residence and wider Australian audiences, and was accurate at the time of publication. Readers should note that over time currency and completeness of the information may change. All users are urged to always seek advice from a qualified health care professional for diagnosis and answers to their medical questions.

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