G11.3 Cerebellar ataxia with defective DNA repair

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applicable to

• Ataxia telangiectasia [Louis-Bar]

Excludes2 (not included here)

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

Applies from G11 Hereditary ataxia

Excludes2 (not included here)

• cerebral palsy (G80.-)
• hereditary and idiopathic neuropathy (G60.-)
• metabolic disorders (E70-E88)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 200 — Friedreich and Other Hereditary Ataxias; Huntington Disease
• CMS-HCC V22: HCC 72 — Spinal Cord Disorders/Injuries
• ESRD V24: HCC 72

MS-DRG index

Defines the logic of DRG(s) 058-060 in MDC 01.

Approximate ICD-9-CM (GEMs)

• 334.8 Other spinocerebellar diseases (approximate)

Code annotations containing back-references to G11.3

Back-references to G11.3

• Type 1 Excludes: D82 Immunodeficiency associated with other major defects ("ataxia telangiectasia [Louis-Bar] (G11.3)")
• Type 1 Excludes: E31 Polyglandular dysfunction ("ataxia telangiectasia [Louis-Bar] (G11.3)")
• Type 1 Excludes: Q85 Phakomatoses, not elsewhere classified ("ataxia telangiectasia [Louis-Bar] (G11.3)")

Back-references to G11

• Type 1 Excludes: R26 Abnormalities of gait and mobility ("hereditary ataxia (G11.-)")
• Type 1 Excludes: R27 Other lack of coordination ("hereditary ataxia (G11.-)")

Diagnosis Index entries for G11.3

• 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)

Nearby codes

• G11.1 Early-onset cerebellar ataxia
• G11.10 Early-onset cerebellar ataxia, 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 (this code)
• G11.4 Hereditary spastic paraplegia
• G11.5 Hypomyelination - hypogonadotropic hypogonadism - hypodontia
• G11.6 Leukodystrophy with vanishing white matter disease
• G11.8 Other hereditary ataxias
• G11.9 Hereditary ataxia, unspecified