Muscular Dystrophy – Common Forms of this Rare Disease and their ICD-10 Codes

by | Last updated Dec 8, 2023 | Published on Feb 19, 2021 | Medical Coding

Muscular Dystrophy
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Muscular dystrophy is a group of disorders characterized by a progressive decline in muscle mass, resulting in loss of strength. These rare diseases are caused by genetic factors. While muscular dystrophy starts early in childhood for some people, others may experience symptoms only in the teen years or in adulthood. In ICD-10, there are codes to report muscular dystrophy with a high level of specificity, which makes it easier for a medical billing and coding company to help physicians to report diagnosis on claims and get reimbursed for their services.

Types of Muscular Dystrophy

Muscular dystrophies comprise more than 30 inherited genetic diseases. Each form differs based on factors such as the genes that cause it, the muscles it affects, the age when symptoms become evident, and the pace of disease progression.

The nine most common forms of muscular dystrophy are:

  • Duchenne muscular dystrophy is the most common type. The condition mainly affects boys, starting between ages 3 and 5.
  • Becker muscular dystrophy also affects boys and is similar to Duchenne, but milder. Symptoms appear between ages 11 and 25.
  • Myotonic muscular dystrophy can affect both men and women and is the most common form of the disease in adults. It is characterized by prolonged muscle contractions (myotonia) and inability to relax muscles after use. It usually starts when people are in their 20s.
  • Congenital muscular dystrophy begins at birth or shortly afterwards.
  • Limb-girdle muscular dystrophy usually starts in the teens or 20s. The condition is characterized by progressive muscle wasting, mainly of the hip and shoulder muscles.
  • Facioscapulohumeral muscular dystrophy is a rare disease that affects the muscles of the face, shoulders, and upper arms. It can affect teenagers as well as adults.
  • Distal muscular dystrophy affects the muscles of the arms, legs, hands, and feet. It usually appears in people ages 40 and 60.
  • Oculopharyngeal muscular dystrophy starts when a person is in the 40s or 50s. It affects the muscles of the face, neck, and shoulders, and causes droopy eyelids (ptosis) and difficulty swallowing (dysphagia).
  • Emery-Dreifuss muscular dystrophy is another condition that affects mainly boys, It usually starts around age 10 and leads to heart problems and muscle weakness.

The disease has no cure but there are several treatments that can help to minimize its symptoms and slow progression of the disease.

Diagnosing Muscular Dystrophy

To diagnose muscular dystrophy, the physician will recommend one or more of the following tests:

  • An enzyme and protein blood test to check for elevated levels of an enzyme called creatine kinase. High levels of this enzyme usually indicate muscle damage due to muscular dystrophy.
  • Electromyography (EMG) to measure the electrical activity of muscles and nerves.
  • Muscle biopsy to identify any cell changes in muscle tissue.
  • Genetic tests identify gene mutations linked to muscular dystrophy.
  • Heart-monitoring tests to check heart function, especially in people diagnosed with myotonic muscular dystrophy.
  • Lung-monitoring tests to check lung function

ICD-10 Coding

The ICD-10 Code for muscular dystrophy is G71.0. Code G71.0 covers the following forms of muscular dystrophy:

  • Distal muscular dystrophy
  • Duchenne muscular dystrophy
  • Emery-Dreifuss muscular dystrophy
  • Erb’s muscular dystrophy
  • Facioscapulohumeral muscular dystrophy
  • Hereditary progressive muscular dystrophy
  • Limb-girdle muscular dystrophy
  • Mitochondrial ocular myopathy
  • Muscular dystrophy, becker
  • Muscular dystrophy, distal
  • Muscular dystrophy, duchenne
  • Muscular dystrophy, emery dreifuss
  • Muscular dystrophy, erbs
  • Muscular dystrophy, facioscapulohumeral
  • Muscular dystrophy, hereditary progressive
  • Muscular dystrophy, limb girdle
  • Muscular dystrophy, neuropathic
  • Muscular dystrophy, ocular
  • Muscular dystrophy, oculopharyngeal
  • Neuropathic muscular dystrophy
  • Ocular muscular dystrophy
  • Oculopharyngeal muscular dystrophy
  • Restrictive lung disease due to muscular dystrophy
  • Restrictive lung mechanics due to muscular dystrophy

G71.0 Muscular dystrophy is non-specific and non-billable diagnosis code. The billable codes with higher level of specificity for a diagnosis of muscular dystrophy are:

G71.01 Duchenne or Becker muscular dystrophy
G71.02 Facioscapulohumeral muscular dystrophy
G71.09 Other specified muscular dystrophies
G71.1 Myotonic disorders
G71.11 Myotonic muscular dystrophy
G71.12 Myotonia congenita
G71.13 Myotonic chondrodystrophy
G71.14 Drug induced myotonia
G71.19 Other specified myotonic disorders


Treatments for muscular dystrophy can help manage and reduce the severity of symptoms:

  • Physical therapy: Initiating physical therapy and stretching exercises early can help maintain muscle strength and flexibility.
  • Respiratory therapy: If the condition affects respiratory strength, treatments to prevent or delay respiratory problems may be recommended shortly after the disease is diagnosed.
  • Speech therapy: Speech therapy can benefit patients with weakness in the facial and throat muscles to maximize muscle strength.
  • Occupational therapy: This can help help patients relearn lost motor skills, to manage life with weakened muscles, use assistive devices and perform daily personal tasks
  • Drug therapy: Medications that can help delay damage to muscles or minimize the symptoms of muscular dystrophy include: glucocorticoids such as prednisone or deflazacort; vamorolone; anticonvulsants; immunosuppressants; beta blockers, angiotensin-converting-enzyme (ACE) inhibitors, and gene-based therapy. Requests for gene therapies such as antisense oligonucleotides require prior authorization and are screened for medical necessity and appropriateness using the criteria.

With proper documentation of the disorder by the physician, medical billing and coding service providers can assign the correct ICD-10 codes on claims.

Julie Clements

Julie Clements, OSI’s Vice President of Operations, brings a diverse background in healthcare staffing and a robust six-year tenure as the Director of Sales and Marketing at a prestigious 4-star resort.

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