2020 ICD-10-CM Diagnosis Code G11.1

Early-onset cerebellar ataxia

    2016 2017 2018 2019 2020 Billable/Specific Code
  • G11.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM G11.1 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of G11.1 - other international versions of ICD-10 G11.1 may differ.
Applicable To
  • Early-onset cerebellar ataxia with essential tremor
  • Early-onset cerebellar ataxia with myoclonus [Hunt's ataxia]
  • Early-onset cerebellar ataxia with retained tendon reflexes
  • Friedreich's ataxia (autosomal recessive)
  • X-linked recessive spinocerebellar ataxia
The following code(s) above G11.1 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to G11.1:
  • G00-G99
    2020 ICD-10-CM Range G00-G99

    Diseases of the nervous system

    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the nervous system
  • G11
    ICD-10-CM Diagnosis Code G11

    Hereditary ataxia

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 2 Excludes
    • cerebral palsy (G80.-)
    • hereditary and idiopathic neuropathy (G60.-)
    • metabolic disorders (E70-E88)
    Hereditary ataxia
Approximate Synonyms
  • Azorean disease
  • Cerebellar ataxia (loss of muscle coordination)
  • Cervical neuromuscular scoliosis due to friedreich's ataxia
  • Cervical scoliosis due to friedreichs ataxia
  • Cervicothoracic neuromuscular scoliosis due to friedreich's ataxia
  • Cervicothoracic scoliosis due to friedreichs ataxia
  • Early onset cerebellar ataxia
  • Friedreich's ataxia
  • Friedreichs ataxia
  • Josephs disease
  • Lumbar neuromuscular scoliosis due to friedreich's ataxia
  • Lumbar scoliosis due to friedreichs ataxia
  • Lumbosacral neuromuscular scoliosis due to friedreich's ataxia
  • Lumbosacral scoliosis due to friedreichs ataxia
  • Machado joseph disease
  • Neuromuscular scoliosis due to friedreich's ataxia
  • Neuromuscular scoliosis due to friedreichs ataxia
  • Occipitoatlantoaxial neuromuscular scoliosis due to friedreich's ataxia
  • Occipitoatlantoaxial scoliosis in friedreichs ataxia
  • Spinocerebellar ataxia
  • Thoracic neuromuscular scoliosis due to friedreich's ataxia
  • Thoracic scoliosis due to friedreichs ataxia
  • Thoracolumbar neuromuscular scoliosis due to friedreich's ataxia
  • Thoracolumbarscoliosis due to friedreichs ataxia
Clinical Information
  • A condition marked by progressive cerebellar ataxia combined with myoclonus usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal seizures, spasticity, and dyskinesias. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the cerebellum are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (from Joynt, Clinical Neurology, 1991, ch37, pp60-1)
ICD-10-CM G11.1 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 058 Multiple sclerosis and cerebellar ataxia with mcc
  • 059 Multiple sclerosis and cerebellar ataxia with cc
  • 060 Multiple sclerosis and cerebellar ataxia without cc/mcc

Convert G11.1 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to G11.1:
  • Ataxia, ataxy, ataxic R27.0
    ICD-10-CM Diagnosis Code R27.0

    Ataxia, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Type 1 Excludes
    • ataxia following cerebrovascular disease (I69. with final characters -93)
    • cerebellar (hereditary) G11.9
      ICD-10-CM Diagnosis Code G11.9

      Hereditary ataxia, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Hereditary cerebellar ataxia NOS
      • Hereditary cerebellar degeneration
      • Hereditary cerebellar disease
      • Hereditary cerebellar syndrome
      • early-onset G11.1
    • hereditary G11.9
      ICD-10-CM Diagnosis Code G11.9

      Hereditary ataxia, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Hereditary cerebellar ataxia NOS
      • Hereditary cerebellar degeneration
      • Hereditary cerebellar disease
      • Hereditary cerebellar syndrome
      • spinal G11.1 (Friedreich's)
    • spinal
      • hereditary G11.1 (Friedreich's)
    • Friedreich's G11.1 (heredofamilial) (cerebellar) (spinal)
    • Hunt's G11.1
    • spinocerebellar, X-linked recessive G11.1
  • Cardiomyopathy (familial) (idiopathic) I42.9
    ICD-10-CM Diagnosis Code I42.9

    Cardiomyopathy, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Cardiomyopathy (primary) (secondary) NOS
    • due to
      • Friedreich's ataxia G11.1
  • Disease, diseased - see also Syndrome
  • Dyssynergia
    • cerebellaris myoclonica G11.1 (Hunt's ataxia)
  • Friedreich's
    • ataxia G11.1
    • combined systemic disease G11.1
    • sclerosis G11.1 (cerebellum) (spinal cord)
  • Hunt's
    • disease or syndrome (herpetic geniculate ganglionitis) B02.21
      ICD-10-CM Diagnosis Code B02.21

      Postherpetic geniculate ganglionitis

        2016 2017 2018 2019 2020 Billable/Specific Code
      • dyssynergia cerebellaris myoclonica G11.1
  • Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic) I42.9
    - see also Cardiomyopathy
    ICD-10-CM Diagnosis Code I42.9

    Cardiomyopathy, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Cardiomyopathy (primary) (secondary) NOS
    • in (due to)
      • Friedreich's ataxia G11.1
  • Ramsay-Hunt disease or syndrome B02.21
    - see also Hunt's disease
    ICD-10-CM Diagnosis Code B02.21

    Postherpetic geniculate ganglionitis

      2016 2017 2018 2019 2020 Billable/Specific Code
    • meaning dyssynergia cerebellaris myoclonica G11.1
  • Sclerosis, sclerotic
    • hereditary
      • spinal G11.1 (Friedreich's ataxia)
    • spinal (cord) (progressive) G95.89
      ICD-10-CM Diagnosis Code G95.89

      Other specified diseases of spinal cord

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Cord bladder NOS
      • Drug-induced myelopathy
      • Radiation-induced myelopathy
      Type 1 Excludes
      • hereditary G11.1 (Friedreich's) (mixed form)
    • Friedreich's G11.1 (spinal cord)

ICD-10-CM Codes Adjacent To G11.1
G06 Intracranial and intraspinal abscess and granuloma
G06.0 Intracranial abscess and granuloma
G06.1 Intraspinal abscess and granuloma
G06.2 Extradural and subdural abscess, unspecified
G07 Intracranial and intraspinal abscess and granuloma in diseases classified elsewhere
G08 Intracranial and intraspinal phlebitis and thrombophlebitis
G09 Sequelae of inflammatory diseases of central nervous system
G10 Huntington's disease
G11 Hereditary ataxia
G11.0 Congenital nonprogressive ataxia
G11.1 Early-onset cerebellar ataxia
G11.2 Late-onset cerebellar ataxia
G11.3 Cerebellar ataxia with defective DNA repair
G11.4 Hereditary spastic paraplegia
G11.8 Other hereditary ataxias
G11.9 Hereditary ataxia, unspecified
G12 Spinal muscular atrophy and related syndromes
G12.0 Infantile spinal muscular atrophy, type I [Werdnig-Hoffman]
G12.1 Other inherited spinal muscular atrophy
G12.2 Motor neuron disease
G12.20 …… unspecified

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.