ICD-10-CM Code G71.2

Congenital myopathies

Version 2020 Billable Code

Valid for Submission

G71.2 is a billable code used to specify a medical diagnosis of congenital myopathies. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code G71.2 might also be used to specify conditions or terms like actin accumulation myopathy, akinesia, autosomal dominant centronuclear myopathy, autosomal recessive centronuclear myopathy, benign congenital myopathy, benign samaritan congenital myopathy, etc

Short Description:Congenital myopathies
Long Description:Congenital myopathies

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code G71.2:

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.
  • Central core disease
  • Fiber-type disproportion
  • Minicore disease
  • Multicore disease
  • Myotubular (centronuclear) myopathy
  • Nemaline myopathy

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • arthrogryposis multiplex congenita Q74.3

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code G71.2 are found in the index:


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

  • Actin accumulation myopathy
  • Akinesia
  • Autosomal dominant centronuclear myopathy
  • Autosomal recessive centronuclear myopathy
  • Benign congenital myopathy
  • Benign Samaritan congenital myopathy
  • Bethlem myopathy
  • Cap myopathy
  • Central core disease
  • Congenital anomaly of skeletal muscle
  • Congenital articular rigidity with myopathy
  • Congenital hereditary muscular dystrophy
  • Congenital lethal myopathy Compton North type
  • Congenital muscular dystrophy Paradas type
  • Congenital myopathy with abnormal subcellular organelles
  • Congenital myopathy with fiber type disproportion
  • Congenital myopathy with internal nuclei and atypical cores
  • Congenital myopathy with myasthenic-like onset
  • Congenital myopathy with uniform fiber type
  • Congenital nonprogressive myopathy with Moebius and Robin sequences
  • Cylindrical spirals myopathy
  • Desmin related myopathy with Mallory body-like inclusions
  • Desmin-related myofibrillar myopathy
  • Duane anomaly, myopathy, scoliosis syndrome
  • Duane's syndrome
  • Duane's syndrome, type 3
  • Inclusion body myopathy with early-onset Paget disease and frontotemporal dementia
  • Infantilism
  • Intellectual disability, cataract, calcified pinna, myopathy syndrome
  • Intellectual disability, myopathy, short stature, endocrine defect syndrome
  • Minimal change myopathy
  • Multi-core congenital myopathy
  • Muscle filaminopathy
  • Myofibrillar myopathy
  • Myofibrillar myopathy
  • Myopathy with abnormality of histochemical fiber type
  • Myopathy with cytoplasmic inclusions
  • Myopathy with hexagonally cross-linked tubular arrays
  • Myopathy with tubular aggregates
  • Myopathy with type I hypotrophy
  • Myosclerosis
  • Myotubular myopathy
  • Myotubular myopathy with type I atrophy
  • Nemaline myopathy
  • Nemaline myopathy, early onset type
  • Nemaline myopathy, late onset type
  • Pinnal calcification
  • Proximal myopathy
  • Reducing-body myopathy
  • Sarcotubular myopathy
  • Severe x-linked myotubular myopathy
  • Zebra body myopathy

Convert G71.2 to ICD-9

  • 359.0 - Cong hered musc dystrphy (Approximate Flag)

Code Classification

  • Diseases of the nervous system (G00–G99)
    • Diseases of myoneural junction and muscle (G70-G73)
      • Primary disorders of muscles (G71)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients

Muscle Disorders

Your muscles help you move and help your body work. Different types of muscles have different jobs. There are many problems that can affect muscles. Muscle disorders can cause weakness, pain or even paralysis.

Causes of muscle disorders include

  • Injury or overuse, such as sprains or strains, cramps or tendinitis
  • A genetic disorder, such as muscular dystrophy
  • Some cancers
  • Inflammation, such as myositis
  • Diseases of nerves that affect muscles
  • Infections
  • Certain medicines

Sometimes the cause is not known.

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Centronuclear myopathy Centronuclear myopathy is a condition characterized by muscle weakness (myopathy) and wasting (atrophy) in the skeletal muscles, which are the muscles used for movement. The severity of centronuclear myopathy varies among affected individuals, even among members of the same family.People with centronuclear myopathy begin experiencing muscle weakness at any time from birth to early adulthood. The muscle weakness slowly worsens over time and can lead to delayed development of motor skills, such as crawling or walking; muscle pain during exercise; and difficulty walking. Some affected individuals may need wheelchair assistance as the muscles atrophy and weakness becomes more severe. In rare instances, the muscle weakness improves over time.Some people with centronuclear myopathy experience mild to severe breathing problems related to the weakness of muscles needed for breathing. People with centronuclear myopathy may have droopy eyelids (ptosis) and weakness in other facial muscles, including the muscles that control eye movement. People with this condition may also have foot abnormalities, a high arch in the roof of the mouth (high-arched palate), and abnormal side-to-side curvature of the spine (scoliosis). Rarely, individuals with centronuclear myopathy have a weakened heart muscle (cardiomyopathy), disturbances in nerve function (neuropathy), or intellectual disability.A key feature of centronuclear myopathy is the displacement of the nucleus in muscle cells, which can be viewed under a microscope. Normally the nucleus is found at the edges of the rod-shaped muscle cells, but in people with centronuclear myopathy the nucleus is located in the center of these cells. How the change in location of the nucleus affects muscle cell function is unknown.
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Nemaline myopathy Nemaline myopathy is a disorder that primarily affects skeletal muscles, which are muscles that the body uses for movement. People with nemaline myopathy have muscle weakness (myopathy) throughout the body, but it is typically most severe in the muscles of the face; neck; trunk; and other muscles close to the center of the body (proximal muscles), such as those of the upper arms and legs. This weakness can worsen over time. Affected individuals may have feeding and swallowing difficulties, foot deformities, abnormal curvature of the spine (scoliosis), and joint deformities (contractures). Most people with nemaline myopathy are able to walk, although some affected children may begin walking later than usual. As the condition progresses, some people may require wheelchair assistance. In severe cases, the muscles used for breathing are affected and life-threatening breathing difficulties can occur.Nemaline myopathy is divided into six types. In order of decreasing severity, the types are: severe congenital, Amish, intermediate congenital, typical congenital, childhood-onset, and adult-onset. The types are distinguished by the age when symptoms first appear and the severity of symptoms; however, there is overlap among the various types. The severe congenital type is the most life-threatening. Most individuals with this type do not survive past early childhood due to respiratory failure. The Amish type solely affects the Old Order Amish population of Pennsylvania and is typically fatal in early childhood. The most common type of nemaline myopathy is the typical congenital type, which is characterized by muscle weakness and feeding problems beginning in infancy. Most of these individuals do not have severe breathing problems and can walk unassisted. People with the childhood-onset type usually develop muscle weakness in adolescence. The adult-onset type is the mildest of all the various types. People with this type usually develop muscle weakness between ages 20 and 50.
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X-linked myotubular myopathy X-linked myotubular myopathy is a condition that primarily affects muscles used for movement (skeletal muscles) and occurs almost exclusively in males. People with this condition have muscle weakness (myopathy) and decreased muscle tone (hypotonia) that are usually evident at birth.The muscle problems in X-linked myotubular myopathy impair the development of motor skills such as sitting, standing, and walking. Affected infants may also have difficulties with feeding due to muscle weakness. Individuals with this condition often do not have the muscle strength to breathe on their own and must be supported with a machine to help them breathe (mechanical ventilation). Some affected individuals need breathing assistance only periodically, typically during sleep, while others require it continuously. People with X-linked myotubular myopathy may also have weakness in the muscles that control eye movement (ophthalmoplegia), weakness in other muscles of the face, and absent reflexes (areflexia).In X-linked myotubular myopathy, muscle weakness often disrupts normal bone development and can lead to fragile bones, an abnormal curvature of the spine (scoliosis), and joint deformities (contractures) of the hips and knees. People with X-linked myotubular myopathy may have a large head with a narrow and elongated face and a high, arched roof of the mouth (palate). They may also have liver disease, recurrent ear and respiratory infections, or seizures.Because of their severe breathing problems, individuals with X-linked myotubular myopathy usually survive only into early childhood; however, some people with this condition have lived into adulthood.X-linked myotubular myopathy is a member of a group of disorders called centronuclear myopathy. In centronuclear myopathy, the nucleus is found at the center of many rod-shaped muscle cells instead of at either end, where it is normally located.
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