2024 ICD-10-CM Diagnosis Code G71.0

Specific Coding Applicable to Muscular dystrophy

Non-specific codes like G71.0 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10-CM codes with a higher level of specificity when coding for muscular dystrophy:

Clinical Information

• Autosomal Recessive Limb-Girdle Muscular Dystrophy Type 2Y|LGMD2Y|MRRSDC|Muscular Dystrophy, Autosomal Recessive, with Rigid Spine and Distal Joint Contractures|TOR1AIP1-Related LGMD|TOR1AIP1-Related Limb-Girdle Muscular Dystrophy

  an autosomal recessive subtype of limb-girdle muscular dystrophy caused by mutation(s) in the tor1aip1 gene, encoding torsin-1a-interacting protein 1.

• Autosomal Recessive Limb-Girdle Muscular Dystrophy-4|Beta-Sarcoglycan-Related Limb-Girdle Muscular Dystrophy R4|LGMD2E|LGMDR4|Limb-Girdle Muscular Dystrophy Type 2E

  an autosomal recessive subtype of limb-girdle muscular dystrophy caused by mutation(s) in the sgcb gene, encoding beta-sarcoglycan.

• Becker's Muscular Dystrophy|Becker

  an x-linked inherited disorder characterized by slowly progressing weakness in the muscles of the legs and pelvis.

• Canine X-Linked Muscular Dystrophy

  x-linked muscular dystrophy occurring in a dog.

• CDISC Duchenne Muscular Dystrophy Therapeutic Area User Guide Version 1.0|Duchenne Muscular Dystrophy Therapeutic Area User Guide v1.0

  the 1.0 version of the cdisc duchenne muscular dystrophy therapeutic area user guide.

• CDISC SDTM Duchenne Muscular Dystrophy Findings About Test Code Terminology|DMFATSCD|Duchenne Muscular Dystrophy Findings About Test Code|SDTM-DMFATSCD

  terminology associated with the duchenne muscular dystrophy findings about test code codelist of the clinical data interchange standards consortium (cdisc) study data tabulation model (sdtm).

• CDISC SDTM Duchenne Muscular Dystrophy Findings About Test Name Terminology|DMFATS|Duchenne Muscular Dystrophy Findings About Test Name|SDTM-DMFATS

  terminology associated with the duchenne muscular dystrophy findings about test name codelist of the clinical data interchange standards consortium (cdisc) study data tabulation model (sdtm).

• Congenital Muscular Dystrophy-Dystroglycanopathy with Mental Retardation Type B2|MDDGB2

  an autosomal recessive inherited congenital muscular dystrophy caused by mutations in the pomt2 gene. it is characterized by mental retardation and mild structural brain abnormalities resulting from defective glycosylation of alpha-dystroglycan.

• Distal Muscular Dystrophy|Distal Myopathy

  a group of genetic degenerative muscle disorders affecting the muscles of the lower arms, hands, lower legs, and feet.

• DMD Gene|DMD|DMD|Dystrophin (Muscular Dystrophy, Duchenne And Becker Types) Gene

  this gene is involved in muscle development.

• DMD wt Allele|BMD|CMD3B|DXS142|DXS164|DXS206|DXS230|DXS239|DXS268|DXS270|DXS272|Dystrophin (Muscular Dystrophy, Duchenne And Becker Types) wt Allele

  human dmd wild-type allele is located in the vicinity of xp21.2 and is approximately 2225 kb in length. this allele, which encodes dystrophin protein, plays a role in muscle cell development. mutation of the gene is associated with cardiomyopathy dilated x-linked type 3b and with both the duchenne and becker types of muscular dystrophy.

• Duchenne Muscular Dystrophy|Duchenne

  an x-linked inherited disorder caused by mutations in the dmd gene found on the x chromosome. it is characterized by rapidly progressing muscle weakness and muscle atrophy initially involving the lower extremities and eventually affecting the whole body. it affects males whereas females can be carriers. the symptoms start before the age of six and may appear at infancy.

• DYSF Gene|DYSF|DYSF|Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) Gene

  this gene plays a role in muscle contraction and plasma membrane dynamics.

• DYSF wt Allele|Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive) wt Allele|FER1L1|FLJ00175|FLJ90168|LGMD2B

  human dysf wild-type allele is located in the vicinity of 2p13.3 and is approximately 233 kb in length. this allele, which encodes dysferlin protein, is involved in sarcolemmal repair and muscle contraction. mutation of the gene is associated with limb girdle muscular dystrophy type 2b, distal myopathy with anterior tibial onset, and miyoshi myopathy.

• EMD wt Allele|EDMD|Emerin wt Allele|Emery-Dreifuss Muscular Dystrophy Gene|LEM Domain Containing 5 Gene|LEMD5|STA

  human emd wild-type allele is located in the vicinity of xq28 and is approximately 2 kb in length. this allele, which encodes emerin protein, is involved in the association of actin filaments with the nuclear lamina and linking centrosome microtubules to the nuclear envelope. mutation of the gene is associated with x-linked emery-dreifuss muscular dystrophy 1.

• Emery-Dreifuss Muscular Dystrophy 1, X-Linked|EDMD1

  emery-dreifuss muscular dystrophy inherited in an x-linked recessive pattern and caused by mutations in the emd gene, encoding emerin.

• Emery-Dreifuss Muscular Dystrophy 2, Autosomal Dominant|EDMD2

  emery-dreifuss muscular dystrophy inherited in an autosomal dominant pattern and caused by mutations in the lmna gene.

• Emery-Dreifuss Muscular Dystrophy|EDMD

  an x-linked or autosomal dominant inherited muscular dystrophy. it is characterized by slowly progressive muscle weakness, atrial conduction defects, cardiomyopathy, and early contractures of the elbow, ankle and neck.

• Facioscapulohumeral Muscular Dystrophy

  an autosomal dominant disorder affecting the skeletal muscles of the face, scapula, and upper arm. patients present with muscle weakness in these anatomic areas. the muscle weakness eventually spreads to other skeletal muscles as well.

• Facioscapulohumeral Muscular Dystrophy 1|FSHD1

  an autosomal dominant form of facioscapulohumeral muscular dystrophy associated with contraction of the d4z4 macrosatellite repeat.

• Facioscapulohumeral Muscular Dystrophy 2|FSHD2

  a form of facioscapulohumeral muscular dystrophy with digenic inheritance, caused by a combination of heterozygous mutation in the smchd1 gene and the presence of a haplotype that is permissive for dux4.

• Limb-Girdle Muscular Dystrophy Type 1C|LGMD1C

  a sub-type of limb-girdle muscular dystrophy caused by mutation(s) in the cav3 gene, encoding caveolin-3.

• Limb-Girdle Muscular Dystrophy Type 2A|Autosomal Recessive Muscular Dystrophy Limb-Girdle 1|LGMD2A|LGMDR1

  an autosomal recessive condition caused by mutation(s) in the capn3 gene, encoding calpain-3. it is characterized by muscular dystrophy, primarily affecting the proximal muscles, resulting in difficulty walking.

• Limb-Girdle Muscular Dystrophy Type 2B|Autosomal Recessive Muscular Dystrophy Limb-Girdle 2|LGMD2B|LGMDR2

  an autosomal recessive condition caused by mutation(s) in the dysf gene, encoding dysferlin. it is characterized by progressive muscular dystrophy, primarily affecting the proximal muscles, resulting in difficulty walking.

• Limb-Girdle Muscular Dystrophy Type 2D|Autosomal Recessive Muscular Dystrophy Limb-Girdle 3|LGMD2D|LGMDR3

  an autosomal recessive condition caused by mutation(s) in the sgca gene, encoding alpha-sarcoglycan. it is characterized by progressive muscular dystrophy, primarily affecting the proximal muscles, resulting in difficulty walking.

• Limb-Girdle Muscular Dystrophy Type 2Z|Autosomal Recessive Muscular Dystrophy Limb-Girdle 21|LGMD2Z|LGMDR21

  an autosomal recessive condition caused by mutation(s) in the poglut1 gene, encoding protein o-glucosyltransferase 1. it is characterized by progressive muscular dystrophy, primarily affecting the proximal muscles, resulting in difficulty walking.

• Limb-Girdle Muscular Dystrophy|Limb girdle dystrophy

  a group of autosomal recessive and less frequently autosomal dominant muscular dystrophies affecting the muscles of the hips and shoulders.

• LMNA wt Allele|CDCD1|CDDC|CMD1A|CMT2B1|Cardiomyopathy, Dilated 1A (Autosomal Dominant)|EMD2|FPL|FPLD|FPLD2|HGPS|IDC|LDP1|LFP|LGMD1B|LMN1|LMNC|LMNL1|Lamin A/C wt Allele|Lamin A/C-Like 1 Gene|Lamin-A/C Gene|Limb Girdle Muscular Dystrophy 1B (Autosomal Dominant) Gene|MADA|Mandibuloacral Dysplasia Type A Gene|PRO1|Progeria 1 (Hutchinson-Gilford Type) Gene

  human lmna wild-type allele is located within 1q22 and is approximately 25 kb in length. this allele, which encodes prelamin-a/c protein, plays a role in nuclear stability and chromatin structure. mutations in the lmna gene are associated with charcot-marie-tooth disease, type 2b1, hutchinson-gilford progeria syndrome, emery-dreifuss muscular dystrophy, malouf syndrome, autosomal dominant familial partial lipodystrophy type 2, lethal restrictive dermopathy, autosomal dominant limb girdle muscular dystrophy 1b and autosomal dominant dilated cardiomyopathy 1a..

• Merosin-Deficient Congenital Muscular Dystrophy Type 1A|MDC1A

  an autosomal recessive inherited congenital muscular dystrophy caused by mutations in the lama2 gene. it is characterized by severe hypotonia, muscle weakness, elevated levels of serum creatinine kinase, and white matter abnormalities.

• Miyoshi Muscular Dystrophy 1|MMD1

  a rare, autosomal recessive inherited skeletal muscle disorder caused by mutation in the dysferlin gene. it affects young adults and is characterized by weakness and atrophy in the muscles of the upper and lower limbs.

• Muscular Dystrophy

  a group of inherited progressive muscle disorders characterized by muscle weakness and eventual death of the muscle tissues. examples include duchenne muscular dystrophy, becker's muscular dystrophy, emery-dreifuss muscular dystrophy, facioscapulohumeral muscular dystrophy, and limb-girdle muscular dystrophy.

• Muscular Dystrophy Congenital, LMNA-Related|MDCL

  an autosomal recessive muscular dystrophy caused by mutation(s) in the lmna gene, encoding prelamin-a/c. limb-girdle muscular dystrophy type 1b and emery-dreifuss muscular dystrophy-2 are allelic disorders with overlapping phenotypes.

• Muscular Dystrophy Secondary to Mitochondrial Disorder|Mitochondrial

  a muscular dystrophy that was caused by a primary mitochondrial disorder.

• Muscular Dystrophy Secondary to Oxidative Phosphorylation Disorder|Ox/Phos

  a muscular dystrophy that was caused by a primary disorder of oxidative phosphorylation.

• Muscular Dystrophy-Dystroglycanopathy (Congenital with Brain and Eye Anomalies) Type A, 1|MDDGA1

  an autosomal recessive muscular dystrophy caused by mutations in the pomt1 gene, encoding protein o-mannosyl-transferase 1. it is associated with characteristic brain and eye malformations, profound mental retardation, and early death.

• Muscular Dystrophy-Dystroglycanopathy (Congenital with Brain and Eye Anomalies) Type A, 2|MDDGA2

  an autosomal recessive muscular dystrophy caused by mutations in the pomt2 gene. it is associated with characteristic brain and eye malformations and profound mental retardation.

• Muscular Dystrophy-Dystroglycanopathy (Congenital with Brain and Eye Anomalies) Type A, 3|MDDGA3

  an autosomal recessive muscular dystrophy caused by mutations in the pomgnt1 gene. it is associated with characteristic brain and eye malformations, profound mental retardation, and death usually in the first years of life.

• Muscular Dystrophy-Dystroglycanopathy (Congenital with Brain and Eye Anomalies) Type A, 4|MDDGA4

  an autosomal recessive muscular dystrophy caused by mutations in the gene encoding fukutin (fktn). it is associated with characteristic brain and eye malformations, seizures, and mental retardation.

• Muscular Dystrophy-Dystroglycanopathy (Congenital with Brain and Eye Anomalies) Type A, 6|MDDGA6

  an autosomal recessive muscular dystrophy caused by mutations in the large gene. it is associated with characteristic brain and eye malformations, profound mental retardation, and death usually in the first years of life.

• Muscular Dystrophy-Dystroglycanopathy (Limb-Girdle) Type C, 1|LGMD2K|Limb-Girdle Muscular Dystrophy Type 2K|MDDGC1

  an autosomal recessive limb-girdle muscular dystrophy caused by mutations in the pomt1 gene, encoding protein o-mannosyl-transferase 1. it is characterized by mental retardation without structural brain abnormalities and limb-girdle muscular dystrophy.

• Muscular Dystrophy-Dystroglycanopathy (Limb-Girdle) Type C, 5|LGMD2I|Limb-Girdle Muscular Dystrophy Type 2I|MDDGC5

  an autosomal recessive inherited limb-girdle muscular dystrophy caused by mutations in the gene encoding fukutin-related protein (fkrp). it is characterized by variable age at onset, normal cognition, and no structural brain changes.

• No History of Other Muscular Dystrophy or Glycogen Storage Disorder|No

  an indication that the patient does not have a history of a muscular dystrophy or storage disorder other than those listed.

• Oculopharyngeal Muscular Dystrophy|OPMD

  an autosomal dominant disorder caused by mutations in the pabpn1 gene, encoding polyadenylate-binding protein 2. the condition is characterized by progressive ptosis, dysphagia and weakness of the muscles of the face, neck, and extraocular muscles.

• Rigid Spine Muscular Dystrophy 1|RSMD1|RSS|Rigid Spine Syndrome

  an inherited muscular dystrophy caused by mutations in the sepn1 gene. it is characterized by severe limitation in flexion of the dorsolumbar and cervical spine, due to contracture of the spinal extensors. it leads to loss of movement of the spine and the thoracic cage.

• Ullrich Congenital Muscular Dystrophy|Scleroatonic Ullrich Disease|UCMD

  a rare, autosomal recessive inherited disorder caused by mutations in the col6a1, col6a2, and col6a3 genes. signs and symptoms usually appear at birth or early infancy. affected individuals have severe muscle weakness, multiple contractures, and hypermobility in their distal joints.

• Unknown History of Other Muscular Dystrophy or Glycogen Storage Disorder|Unknown

  an indication that it is unknown whether the patient has a history of a muscular dystrophy or storage disorder other than those listed.

• Walker-Warburg Syndrome|Muscular Dystrophy-Dystroglycanopathy (Congenital with Brain and Eye Anomalies) Type A|WWS|Walker-Warburg Muscular Dystrophy

  a rare autosomal recessive inherited muscular dystrophy. it presents with generalized hypotonia, muscle weakness, mental retardation, developmental delays, and brain and eye abnormalities.

Convert G71.0 to ICD-9-CM

• ICD-9-CM Code: 359.1 - Hered prog musc 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]

Oculopharyngeal muscular dystrophy

Oculopharyngeal muscular dystrophy is a genetic condition characterized by muscle weakness that begins in adulthood, typically after age 40. The term "oculopharyngeal" refers to the eyes (oculo-) and a part of the throat called the pharynx (-pharyngeal). Affected individuals usually first experience weakness of the muscles in both eyelids that causes droopy eyelids (ptosis). Ptosis can worsen over time, causing the eyelid to impair vision, and in some cases, limit eye movement. Along with ptosis, affected individuals develop weakness of the throat muscles that causes difficulty swallowing (dysphagia). Dysphagia begins with dry food, but over time, liquids can also become difficult to swallow. Dysphagia can cause saliva to accumulate and a wet-sounding voice. Many people with oculopharyngeal muscular dystrophy also have weakness and wasting (atrophy) of the tongue. These problems with food intake may cause malnutrition, choking, or a bacterial lung infection called aspiration pneumonia.

Individuals with oculopharyngeal muscular dystrophy frequently have weakness in the muscles near the center of the body (proximal muscles), particularly muscles in the shoulders, upper legs, and hips (limb-girdle muscles). The weakness slowly gets worse, and people may need the aid of a cane or a walker. Rarely, affected individuals need wheelchair assistance.

Rarely, individuals have a severe form of oculopharyngeal muscular dystrophy with muscle weakness that begins before age 45, and have trouble walking independently by age 60. These individuals often also have disturbances in nerve function (neuropathy), a gradual loss of intellectual functioning (cognitive decline), and psychiatric symptoms such as depression or strongly held false beliefs (delusions).

[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.