G11.3 Cerebellar ataxia with defective DNA repair

The ICD-10-CM code for Cerebellar ataxia with defective DNA repair is G11.3 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

6: Diseases of the nervous system (G00-G99)

Section

G10-G14: Systemic atrophies primarily affecting the central nervous system (G10-G14)

Category G11

13 codes (11 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to G11.3:

• Ataxia-telangiectasia (Louis-Bar)
• Boder-Sedgwick syndrome (ataxia-telangiectasia)
• Louis-Bar syndrome (ataxia-telangiectasia)
• Telangiectasia, telangiectasis (verrucous) › ataxic (cerebellar) (Louis-Bar)
• Syndrome › Boder-Sedgewick
• Syndrome › ataxia-telangiectasia
• Ataxia, ataxy, ataxic › telangiectasia (Louis-Bar)
• Syndrome › Louis-Barré

Inclusion Terms

• Ataxia telangiectasia [Louis-Bar]

U.S. Hospital Utilization

• An estimated 220 U.S. inpatient stays in 2023 included G11.3 among the documented diagnoses.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Dominant/nondominant side (TOC entry)

a. Dominant/nondominant side ................................................................................................. 45

Pain - Category G89 (TOC entry)

b. Pain - Category G89.............................................................................................................. 45

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)