ICD-10-CM 2027
ICD-10 code for Boder-Sedgwick syndrome
From the official ICD-10-CM alphabetic index entry “Boder-Sedgwick syndrome (ataxia-telangiectasia)”. Page updated September 29, 2026.
About coding Boder-Sedgwick syndrome
The ICD-10-CM code for Boder-Sedgwick syndrome is G11.3 (Cerebellar ataxia with defective DNA repair).
Within G11 (hereditary ataxia), G11.3 is specifically for cerebellar ataxia with defective DNA repair. Related codes cover congenital nonprogressive ataxia (G11.0), early-onset cerebellar ataxia (G11.1), Late-onset cerebellar ataxia (G11.2), hereditary spastic paraplegia (G11.4), Hypomyelination - hypogonadotropic hypogonadism - hypodontia (G11.5), leukodystrophy with vanishing white matter disease (G11.6), other hereditary ataxias (G11.8) and unspecified (G11.9).
The conditions in its Excludes2 note aren't part of G11.3, but may be coded alongside it if the patient has both: Cockayne's syndrome (Q87.19), other disorders of purine and pyrimidine metabolism (E79.-) and xeroderma pigmentosum (Q82.1).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
Common questions about Boder-Sedgwick syndrome ICD-10 codes
What is the ICD-10 code for Boder-Sedgwick syndrome?
- G11.3 — Cerebellar ataxia with defective DNA repair.
Is G11.3 billable?
- Yes. G11.3 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can G11.3 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict G11.3 as a principal diagnosis.
Is G11.3 a CC or MCC?
- G11.3 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.