Hereditary ataxia (G11) ICD-10-CM
The G11 code range covers hereditary ataxia with 13 ICD-10-CM diagnosis codes. 11 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Type 2 Excludes
A type 2 excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate.
Codes in the G11 Range 13 codes · 11 billable
- G11 Hereditary ataxiaNon-billable
- G11.0 Congenital nonprogressive ataxia
- G11.1 Early-onset cerebellar ataxiaNon-billable
- 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
- 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
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the G11 range.
Ataxia Telangiectasia
An autosomal recessive inherited disorder characterized by choreoathetosis beginning in childhood, progressive CEREBELLAR ATAXIA; TELANGIECTASIS of CONJUNCTIVA and SKIN; DYSARTHRIA; B- and T-cell immunodeficiency, and RADIOSENSITIVITY to IONIZING RADIATION. Affected individuals are prone to recurrent sinobronchopulmonary infections, lymphoreticular neoplasms, and other malignancies. Serum ALPHA-FETOPROTEINS are usually elevated. (Menkes, Textbook of Child Neurology, 5th ed, p688) The gene for this disorder (ATM) encodes a cell cycle checkpoint protein kinase and has been mapped to chromosome 11 (11q22-q23).
Congenital Non-Progressive Ataxia
A clinically and genetically heterogenous group of rare, predominantly non-progressive neurological disorders present at birth and characterized by hypotonia, developmental delays, and ataxia. Cognitive and motor impairments may improve over time.
Friedreich Ataxia
An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)
Hereditary Cerebellar Ataxia
Cerebellar ataxia that is transmitted from parent to child.
Spastic Paraplegia, Hereditary
A group of inherited diseases that share similar phenotypes but are genetically diverse. Different genetic loci for autosomal recessive, autosomal dominant, and x-linked forms of hereditary spastic paraplegia have been identified. Clinically, patients present with slowly progressive distal limb weakness and lower extremity spasticity. Peripheral sensory neurons may be affected in the later stages of the disease. (J Neurol Neurosurg Psychiatry 1998 Jan;64(1):61-6; Curr Opin Neurol 1997 Aug;10(4):313-8)
About the G11 Code Range
Hereditary ataxia sits among nervous system conditions grouped as systemic atrophies that primarily affect the central nervous system.
The subdivisions distinguish congenital nonprogressive ataxia, early-onset cerebellar ataxia, and late-onset cerebellar ataxia. Early-onset codes separate Friedreich ataxia from other and unspecified forms. Other subdivisions identify cerebellar ataxia with defective DNA repair, hereditary spastic paraplegia, and named conditions involving white matter. The category also has codes for other hereditary ataxias and hereditary ataxia that is not further specified.
Questions About This Page
How many billable codes are in the G11 range?
Of the 13 codes in this range, 11 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the G11 range classify?
The range classifies hereditary ataxia. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
