Version 2024

2024 ICD-10-CM Diagnosis Code G71.03

Limb girdle muscular dystrophies

ICD-10-CM Code:
G71.03
ICD-10 Code for:
Limb girdle muscular dystrophies
Is Billable?
Not Valid for Submission
Code Navigator:

Code Classification

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

G71.03 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of limb girdle muscular dystrophies. The code is not specific and is NOT valid for the year 2024 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding Applicable to Limb girdle muscular dystrophies

Non-specific codes like G71.03 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 limb girdle muscular dystrophies:

  • Use G71.031 for Autosomal dominant limb girdle muscular dystrophy - BILLABLE CODE

  • Use G71.032 for Autosomal recessive limb girdle muscular dystrophy due to calpain-3 dysfunction - BILLABLE CODE

  • Use G71.033 for Limb girdle muscular dystrophy due to dysferlin dysfunction - BILLABLE CODE

  • G71.034 for Limb girdle muscular dystrophy due to sarcoglycan dysfunction - NON-BILLABLE CODE

  • Use G71.0340 for unspecified - BILLABLE CODE

  • Use G71.0341 for Limb girdle muscular dystrophy due to alpha sarcoglycan dysfunction - BILLABLE CODE

  • Use G71.0342 for Limb girdle muscular dystrophy due to beta sarcoglycan dysfunction - BILLABLE CODE

  • Use G71.0349 for Limb girdle muscular dystrophy due to other sarcoglycan dysfunction - BILLABLE CODE

  • Use G71.035 for Limb girdle muscular dystrophy due to anoctamin-5 dysfunction - BILLABLE CODE

  • Use G71.038 for Other limb girdle muscular dystrophy - BILLABLE CODE

  • Use G71.039 for Limb girdle muscular dystrophy, unspecified - BILLABLE CODE

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.

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - Code Added, effective from 10/1/2022 through 9/30/2023