2024 ICD-10-CM Diagnosis Code G71.11

Myotonic muscular dystrophy

ICD-10-CM Code:
ICD-10 Code for:
Myotonic muscular dystrophy
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Diseases of the nervous system
    • Diseases of myoneural junction and muscle
      • Primary disorders of muscles

G71.11 is a billable diagnosis code used to specify a medical diagnosis of myotonic muscular dystrophy. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Cardiomyopathy in myotonic dystrophy
  • Cardiomyopathy in myotonic dystrophy
  • Congenital myotonic dystrophy
  • Dilated cardiomyopathy due to muscular dystrophy
  • Dilated cardiomyopathy due to myotonic dystrophy
  • Dystrophia myotonica facies
  • Myotonic dystrophy
  • Proximal myotonic myopathy
  • Steinert myotonic dystrophy syndrome

Clinical Classification

Clinical Information

  • Myotonic Dystrophy

    neuromuscular disorder characterized by progressive muscular atrophy; myotonia, and various multisystem atrophies. mild intellectual disability may also occur. abnormal trinucleotide repeat expansion in the 3' untranslated regions of dmpk protein gene is associated with myotonic dystrophy 1. dna repeat expansion of zinc finger protein-9 gene intron is associated with myotonic dystrophy 2.
  • Myotonin-Protein Kinase

    serine/threonine protein kinase responsible for various skeletal muscle functions; heart conduction system activity; calcium homeostasis; calcium uptake by sarcoplasmic reticulum and synaptic plasticity. it is encoded by the dmpk gene and its abnormal expanded trinucleotide repeat of ctg in the 3'-utr is associated with myotonic dystrophy 1.
  • Congenital Myotonic Dystrophy

    myotonic dystrophy that is present at birth.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Dystrophia myotonica Steinert
  • Myotonia atrophica
  • Myotonic dystrophy
  • Proximal myotonic myopathy (PROMM)
  • Steinert disease

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Convert G71.11 to ICD-9-CM

  • ICD-9-CM Code: 359.21 - Myotonic musclr dystrphy

Patient Education

Muscular Dystrophy

What is muscular dystrophy (MD)?

Muscular dystrophy (MD) is a group of more than 30 genetic diseases. They cause weakness of the muscles. Over time, the weakness gets worse and can cause trouble walking and doing daily activities. Some types of MD can also affect other organs.

What are the types of muscular dystrophy (MD)?

There are many different types of MD. Some of the more common types include:

  • Duchenne muscular dystrophy, which is the most common childhood form. It is severe and affects boys more often than girls. The symptoms usually start between ages 3 and 6.
  • Becker muscular dystrophy, which is similar to Duchenne but is less severe and gets worse more slowly. It often starts in the teenage years.
  • Congenital muscular dystrophies, which are present at birth or before age 2. They can be mild or severe.
  • Facioscapulohumeral muscular dystrophy, which often starts in the teenage years. At first, it affects the muscles of the face, shoulders, and upper arms.

Each of the types of MD can be different in many ways, such as:

  • Who is more likely to get them
  • Which muscles they affect
  • When they appear, such as in infancy, childhood, middle age, or later
  • What the symptoms are
  • How serious the symptoms are
  • How quickly they get worse
  • Whether they run in families
  • Whether they affect other organs

Even within the same type of MD, people can have different symptoms.

What causes muscular dystrophy (MD)?

MD is genetic, meaning that it caused by a change in one or more genes. Gene changes are also called gene variants or mutations. The gene changes in MD affect proteins that strengthen and protect muscles.

There are different gene changes that cause each type of MD. And sometimes people who have the same type of MD can have different gene changes.

Muscular dystrophy can run in families, or you can be the first in your family to have a muscular dystrophy.

How is muscular dystrophy (MD) diagnosed?

To find out if you or your child has MD, your health care provider may use:

  • A medical and family history
  • A physical exam
  • Blood and urine tests, including genetic tests and tests for certain enzymes that may be released by damaged muscles
  • Muscle biopsies
  • Electromyography and nerve conduction studies to find out if muscles are responding the right way to nerve signals
  • Heart testing, such as an electrocardiogram (EKG), since some types of MD can cause heart problems
  • Exercise tests to measure muscle strength and breathing and detect any increased rates of certain chemicals following exercise
  • Imaging tests such as an MRI to look at muscle quality and bulk and measure fatty replacement of muscle tissue

What are the treatments for muscular dystrophy (MD)?

There is no cure for muscular dystrophy. Treatment can help with the symptoms and prevent complications. It usually includes a combination of therapies, such as:

  • Physical therapy to help keep muscles flexible and strong
  • Occupational therapy to relearn lost motor skills and learn ways to work around weakened muscles
  • Respiratory care, such as breathing exercises, oxygen therapy, and ventilators
  • Speech therapy to help with speech and swallowing problems
  • Assistive devices, such as wheelchairs, splints and braces, and walkers
  • Medicines to help delay damage to muscles or minimize the symptoms of MD
  • Surgery to treat some of the conditions associated with MD, such as heart problems, scoliosis, and cataracts

NIH: National Institute of Neurological Disorders and Stroke

[Learn More in MedlinePlus]

Myotonic dystrophy

Myotonic dystrophy is part of a group of inherited disorders called muscular dystrophies. It is the most common form of muscular dystrophy that begins in adulthood.

Myotonic dystrophy is characterized by progressive muscle wasting and weakness. People with this disorder often have prolonged muscle contractions (myotonia) and are not able to relax certain muscles after use. For example, a person may have difficulty releasing their grip on a doorknob or handle. Also, affected people may have slurred speech or temporary locking of their jaw.

Other signs and symptoms of myotonic dystrophy include clouding of the lens of the eye (cataracts) and abnormalities of the electrical signals that control the heartbeat (cardiac conduction defects). Some affected individuals develop a condition called diabetes mellitus, in which blood sugar (glucose) levels can become dangerously high. The features of myotonic dystrophy often develop during a person's twenties or thirties, although they can occur at any age. The severity of the condition varies widely among affected people, even among members of the same family.

There are two major types of myotonic dystrophy: type 1 and type 2. Their signs and symptoms overlap, although type 2 tends to be milder than type 1. The muscle weakness associated with type 1 particularly affects muscles farthest from the center of the body (distal muscles), such as those of the lower legs, hands, neck, and face. Muscle weakness in type 2 primarily involves muscles close to the center of the body (proximal muscles), such as the those of the neck, shoulders, elbows, and hips. The two types of myotonic dystrophy are caused by mutations in different genes.

There are two variations of myotonic dystrophy type 1: the mild and congenital types. Mild myotonic dystrophy is apparent in mid to late adulthood. Affected individuals typically have mild myotonia and cataracts. Congenital myotonic dystrophy is often apparent at birth. Characteristic features include weak muscle tone (hypotonia), an inward- and upward-turning foot (clubfoot), breathing problems, delayed development, and intellectual disability. Some of these health problems can be life-threatening.

[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.


[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.