2023 ICD-10-CM Diagnosis Code G11.3

Cerebellar ataxia with defective DNA repair

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
  • G11.3 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2023 edition of ICD-10-CM G11.3 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of G11.3 - other international versions of ICD-10 G11.3 may differ.
Applicable To
  • Ataxia telangiectasia [Louis-Bar]
Type 2 Excludes
Type 2 Excludes Help
A type 2 excludes note represents "not included here". A type 2 excludes note indicates that the condition excluded is not part of the condition it is excluded from but a patient may have both conditions at the same time. When a type 2 excludes note appears under a code it is acceptable to use both the code (G11.3) and the excluded code together.
  • Cockayne's syndrome (
    ICD-10-CM Diagnosis Code Q87.19

    Other congenital malformation syndromes predominantly associated with short stature

      2020 - New Code 2021 2022 2023 Billable/Specific Code POA Exempt
    Applicable To
    • Aarskog syndrome
    • Cockayne syndrome
    • De Lange syndrome
    • Dubowitz syndrome
    • Noonan syndrome
    • Robinow-Silverman-Smith syndrome
    • Russell-Silver syndrome
    • Seckel syndrome
    Q87.19
    )
  • other disorders of purine and pyrimidine metabolism (
    ICD-10-CM Diagnosis Code E79
    • E79 Disorders of purine and pyrimidine metabolism...
      • E79.0 Hyperuricemia without signs of inflammatory a...
      • E79.1 Lesch-Nyhan syndrome
      • E79.2 Myoadenylate deaminase deficiency
      • E79.8 Other disorders of purine and pyrimidine meta...
      • E79.9 Disorder of purine and pyrimidine metabolism,...
    E79.-
    )
  • xeroderma pigmentosum (
    ICD-10-CM Diagnosis Code Q82.1

    Xeroderma pigmentosum

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code POA Exempt
    Q82.1
    )
The following code(s) above G11.3 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to G11.3:
  • G00-G99
    2023 ICD-10-CM Range G00-G99

    Diseases of the nervous system

    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the nervous system
  • G11
    ICD-10-CM Diagnosis Code G11

    Hereditary ataxia

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 2 Excludes
    • cerebral palsy (G80.-)
    • hereditary and idiopathic neuropathy (G60.-)
    • metabolic disorders (E70-E88)
    Hereditary ataxia
Approximate Synonyms
  • Ataxia telangiectasia syndrome
ICD-10-CM G11.3 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 058 Multiple sclerosis and cerebellar ataxia with mcc
  • 059 Multiple sclerosis and cerebellar ataxia with cc
  • 060 Multiple sclerosis and cerebellar ataxia without cc/mcc

Convert G11.3 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
Code annotations containing back-references to G11.3:
  • Type 1 Excludes: D82
    , E31
    , Q85
    ICD-10-CM Diagnosis Code D82

    Immunodeficiency associated with other major defects

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    ICD-10-CM Diagnosis Code E31

    Polyglandular dysfunction

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • dystrophia myotonica [Steinert] (G71.11)
    • pseudohypoparathyroidism (E20.1)
    ICD-10-CM Diagnosis Code Q85

    Phakomatoses, not elsewhere classified

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • ataxia telangiectasia [Louis-Bar] (G11.3)
    • familial dysautonomia [Riley-Day] (G90.1)

Diagnosis Index entries containing back-references to G11.3:
  • Ataxia, ataxy, ataxic R27.0
    ICD-10-CM Diagnosis Code R27.0

    Ataxia, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    Type 1 Excludes
    • ataxia following cerebrovascular disease (I69. with final characters -93)
    • cerebellar (hereditary) G11.9
      ICD-10-CM Diagnosis Code G11.9

      Hereditary ataxia, unspecified

        2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
      Applicable To
      • Hereditary cerebellar ataxia NOS
      • Hereditary cerebellar degeneration
      • Hereditary cerebellar disease
      • Hereditary cerebellar syndrome
      • with defective DNA repair G11.3
    • telangiectasia G11.3 (Louis-Bar)
  • Ataxia-telangiectasia G11.3 (Louis-Bar)
  • Boder-Sedgwick syndrome G11.3 (ataxia-telangiectasia)
  • Louis-Bar syndrome G11.3 (ataxia-telangiectasia)
  • Syndrome - see also Disease
    • ataxia-telangiectasia G11.3
    • Boder-Sedgewick G11.3
    • Louis-Barré G11.3
  • Telangiectasia, telangiectasis (verrucous) I78.1
    ICD-10-CM Diagnosis Code I78.1

    Nevus, non-neoplastic

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    Applicable To
    • Araneus nevus
    • Senile nevus
    • Spider nevus
    • Stellar nevus
    Type 1 Excludes
    Type 2 Excludes
    • ataxic G11.3 (cerebellar) (Louis-Bar)

ICD-10-CM Codes Adjacent To G11.3
G08 Intracranial and intraspinal phlebitis and thrombophlebitis
G09 Sequelae of inflammatory diseases of central nervous system
G10 Huntington's disease
G11 Hereditary ataxia
G11.0 Congenital nonprogressive ataxia
G11.1 Early-onset cerebellar ataxia
G11.10 …… unspecified
G11.11 Friedreich ataxia
G11.19 Other early-onset cerebellar ataxia
G11.2 Late-onset cerebellar ataxia
G11.3 Cerebellar ataxia with defective DNA repair
G11.4 Hereditary spastic paraplegia
G11.8 Other hereditary ataxias
G11.9 Hereditary ataxia, unspecified
G12 Spinal muscular atrophy and related syndromes
G12.0 Infantile spinal muscular atrophy, type I [Werdnig-Hoffman]
G12.1 Other inherited spinal muscular atrophy
G12.2 Motor neuron disease
G12.20 …… unspecified
G12.21 Amyotrophic lateral sclerosis
G12.22 Progressive bulbar palsy

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.