2026 ICD-10-CM Diagnosis Code G11.3Cerebellar ataxia with defective DNA repair

ICD-10-CM Codes›G00–G99›G10-G14›G11

ICD-10-CM G11.3
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

G11.3 is a billable ICD-10-CM diagnosis code for cerebellar ataxia with defective DNA repair. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as ataxia telangiectasia variant. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative).

Code Identity

ICD-10-CM Code
G11.3
Billable Status
Yes — Valid for Submission
Code Describes
Cerebellar ataxia with defective DNA repair
Short Description
Cerebellar ataxia with defective DNA repair
Same as the full description in the CMS dataset.
Parent Code
G11
Hereditary ataxia

Code Classification

ChapterG00–G99Diseases of the nervous system
SectionG10-G14Systemic atrophies primarily affecting the central nervous system
CategoryG11Hereditary ataxia
This CodeG11.3Cerebellar ataxia with defective DNA repair

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Ataxia telangiectasia variant
  • Ataxia-telangiectasia syndrome
  • Chorea due to ataxia telangiectasia syndrome
  • Chorea due to immunological disorder
  • Dystonia due to ataxia telangiectasia syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Ataxia telangiectasia Louis-Bar

Type 2 Excludes

  • Cockayne's syndrome Q87.19
  • other disorders of purine and pyrimidine metabolism E79
  • xeroderma pigmentosum Q82.1

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Ataxia, ataxy, ataxic
      • cerebellar (hereditary)
        • with defective DNA repair
    • Ataxia, ataxy, ataxic
      • telangiectasia (Louis-Bar)
    • Ataxia-telangiectasia(Louis-Bar)
    • Boder-Sedgwick syndrome(ataxia-telangiectasia)
    • Louis-Bar syndrome(ataxia-telangiectasia)
    • Syndrome
      • ataxia-telangiectasia
    • Syndrome
      • Boder-Sedgewick
    • Syndrome
      • Louis-Barré
    • Telangiectasia, telangiectasis(verrucous)
      • ataxic (cerebellar) (Louis-Bar)

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR NVS006
Other nervous system disorders (often hereditary or degenerative)
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Cerebellar Disorders

When you play the piano or hit a tennis ball you are activating the cerebellum. The cerebellum is the area of the brain that controls coordination and balance. Problems with the cerebellum include:

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert G11.3 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
334.8 Spinocerebellar dis NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About G11.3Overview

Is G11.3 (Hereditary ataxia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report cerebellar ataxia with defective DNA repair on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of G11.3?

Under the General Equivalence Mappings, cerebellar ataxia with defective DNA repair converts to ICD-9-CM 334.8 (spinocerebellar dis NEC). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[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.