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    v1.0.28
    OpenPayerBilling CodesSystemic atrophies primarily affecting the central nervous systemICD-10-CM g11-hereditary-ataxia
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    ICD-10-CM Code For Hereditary Ataxia: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group G11 includes inherited ataxias and related hereditary cerebellar syndromes such as spinocerebellar ataxias and other familial ataxic disorders that primarily affect coordination and balance. These ICD-10-CM diagnosis codes are used to document the presence, progression, and neurologic manifestations of hereditary ataxia, capturing disease severity, cerebellar involvement, and associated clinical complexity. Accurate coding within the ICD-10-CM G11 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM G11: Hereditary Ataxia Overview

    Hereditary ataxias are a group of inherited neurodegenerative disorders characterized by progressive incoordination of gait, limb movements, and often associated with dysarthria and ocular motor abnormalities. These conditions primarily affect the cerebellum and its connections within the central nervous system. Accurate coding matters because it supports precise clinical documentation, influences claim adjudication, and helps ensure appropriate reimbursement for neurologic care.

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    When to Use

    Use a code from this group when a patient has a documented hereditary ataxia diagnosis based on clinical evaluation, genetic testing results, or family history indicating a familial ataxic syndrome. Select the most specific code available to reflect disease subtype, presence of systemic neurologic involvement, and documented clinical features to support accurate billing and recordkeeping.

    ICD-10-CM Codes

    11 rows

    ICD-10-CM CodeDescription
    G11.0Congenital nonprogressive ataxia
    G11.10Early-onset cerebellar ataxia, unspecified
    G11.11Friedreich ataxia
    G11.19Other early-onset cerebellar ataxia
    G11.2Late-onset cerebellar ataxia
    G11.3Cerebellar ataxia with defective DNA repair
    G11.4Hereditary spastic paraplegia
    G11.5Hypomyelination - hypogonadotropic hypogonadism - hypodontia
    G11.6Leukodystrophy with vanishing white matter disease
    G11.8Other hereditary ataxias
    G11.9Hereditary ataxia, unspecified

    DRG Codes

    3 rows

    DRG CodeDescription
    058Multiple Sclerosis and Cerebellar Ataxia with MCC
    059Multiple Sclerosis and Cerebellar Ataxia with CC
    060Multiple Sclerosis and Cerebellar Ataxia without CC/MCC

    Typical Clinical Scenarios

    • A middle-aged adult with progressive unsteady gait, dysarthria, and limb coordination difficulties over several years presents to neurology for evaluation of slowly worsening cerebellar signs and a family history of similar symptoms. Workup including neurologic exam and genetic testing confirms autosomal dominant cerebellar degeneration consistent with hereditary spinocerebellar ataxia, and a code from this group is assigned to capture the hereditary ataxia diagnosis. Typical codes: G11.1, G11.11.
    • A child is brought to the emergency department after an acute worsening of balance and tremor following a febrile illness; previously diagnosed with Friedreich ataxia and progressive cardiomyopathy, the child now has new gait instability requiring admission for observation and supportive care. The hereditary ataxia code is used as the underlying chronic neurologic condition contributing to the acute decompensation. Typical codes: G11.2, G11.4.
    • An adult with long-standing hereditary cerebellar ataxia is seen in clinic for evaluation of new dysphagia and recurrent aspiration pneumonia; the visit documents the ataxia as a chronic systemic neurodegenerative disorder contributing to the complication and care planning. The hereditary ataxia diagnosis is coded to reflect the underlying neurologic disease that is a comorbidity complicating management. Typical codes: G11.3, G11.9.

    Related Codes

    G10-HUNTINGTON-S-DISEASE

    ICD-10-CM Code For Huntington's Disease: Group Overview

    G12-SPINAL-MUSCULAR-ATROPHY-AND-RELATED-SYNDROMES

    ICD-10-CM Code For Spinal Muscular Atrophy And Related Syndromes: Group Overview

    G13-SYSTEMIC-ATROPHIES-CENTRAL-NERVOUS-SYSTEM

    ICD-10-CM Code For Systemic Atrophies Primarily Affecting Central Nervous System: Group Overview

    G14-POSTPOLIO-SYNDROME

    ICD-10-CM Code For Postpolio Syndrome: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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