ICD-10-CM Code G11.9

Hereditary ataxia, unspecified

Version 2021 Billable Code

Valid for Submission

G11.9 is a billable code used to specify a medical diagnosis of hereditary ataxia, unspecified. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code G11.9 might also be used to specify conditions or terms like ataxia due to mitochondrial mutations, ataxia with deafness and intellectual disability syndrome, ataxia with tapetoretinal degeneration syndrome, autosomal dominant cerebellar ataxia, deafness and narcolepsy syndrome, autosomal recessive ataxia due to ubiquinone deficiency, autosomal recessive cerebellar ataxia due to stub1 deficiency, etc

Short Description:Hereditary ataxia, unspecified
Long Description:Hereditary ataxia, unspecified

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code G11.9:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Hereditary cerebellar ataxia NOS
  • Hereditary cerebellar degeneration
  • Hereditary cerebellar disease
  • Hereditary cerebellar syndrome

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code G11.9 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Ataxia due to mitochondrial mutations
  • Ataxia with deafness and intellectual disability syndrome
  • Ataxia with tapetoretinal degeneration syndrome
  • Autosomal dominant cerebellar ataxia, deafness and narcolepsy syndrome
  • Autosomal recessive ataxia due to ubiquinone deficiency
  • Autosomal recessive cerebellar ataxia due to STUB1 deficiency
  • Autosomal recessive cerebellar ataxia with oculomotor apraxia type 1
  • Autosomal recessive cerebellar ataxia with oculomotor apraxia type 2
  • Autosomal recessive cerebellar ataxia, epilepsy, intellectual disability syndrome due to RUBCN deficiency
  • Autosomal recessive cerebellar ataxia, epilepsy, intellectual disability syndrome due to TUD deficiency
  • Boucher Neuhäuser syndrome
  • Cerebellar ataxia
  • Cerebellar ataxia and ectodermal dysplasia
  • Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome
  • Cerebral ataxia
  • Choreoathetosis
  • Congenital cataract with ataxia and deafness syndrome
  • Cutaneous syndrome with ichthyosis
  • Early-onset progressive neurodegeneration, blindness, ataxia, spasticity syndrome
  • Episodic ataxia
  • Episodic ataxia type 7
  • Fragile X associated tremor ataxia syndrome
  • Gemignani syndrome
  • Hereditary ataxia
  • Hereditary cerebellar atrophy
  • Hereditary cerebellar degeneration
  • Ichthyosis, cerebellar degeneration and hepatosplenomegaly
  • Infantile cerebellar and retinal degeneration
  • Myoclonus, cerebellar ataxia, deafness syndrome
  • Narcolepsy
  • Oculomotor apraxia
  • Oculomotor apraxia
  • Paroxysmal choreoathetosis
  • Paroxysmal dystonia
  • Paroxysmal dystonic choreoathetosis with episodic ataxia and spasticity
  • Posthemiplegic ataxia
  • Primary cerebellar degeneration
  • Primary progressive cerebellar degeneration
  • Progressive cerebellar ataxia
  • Retinal pigment epithelial dystrophy
  • Retinitis pigmentosa-deafness syndrome
  • Retinitis pigmentosa-deafness-ataxia syndrome
  • Saldino-Mainzer dysplasia
  • Seizure, sensorineural deafness, ataxia, intellectual disability, electrolyte imbalance syndrome
  • Spinocerebellar disease
  • X-linked progressive cerebellar ataxia

Clinical Information

  • 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;33516:1169 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;33516:1169 75
  • SPINOCEREBELLAR DEGENERATIONS-. a heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. sporadic and inherited subtypes occur. inheritance patterns include autosomal dominant autosomal recessive and x linked.

Convert G11.9 to ICD-9

  • 334.9 - Spinocerebellar dis NOS (Approximate Flag)

Code Classification

  • Diseases of the nervous system (G00–G99)
    • Systemic atrophies primarily affecting the central nervous system (G10-G14)
      • Hereditary ataxia (G11)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

Cerebellar Disorders

When you play the piano or hit a tennis ball you are activating the cerebellum. The cerebellum is the area of the brain that controls coordination and balance. Problems with the cerebellum include

  • Cancer
  • Genetic disorders
  • Ataxias - failure of muscle control in the arms and legs that result in movement disorders
  • Degeneration - disorders caused by brain cells decreasing in size or wasting away

Treatment of cerebellar disorders depends on the cause. In some cases, there is no cure but treatment may help with symptoms.

NIH: National Institute of Neurological Disorders and Stroke

  • Acute cerebellar ataxia (Medical Encyclopedia)
  • Olivopontocerebellar atrophy (Medical Encyclopedia)

[Learn More]

Movement Disorders

Movement disorders are neurologic conditions that cause problems with movement, such as

  • Increased movement that can be voluntary (intentional) or involuntary (unintended)
  • Decreased or slow voluntary movement

There are many different movement disorders. Some of the more common types include

  • Ataxia, the loss of muscle coordination
  • Dystonia, in which involuntary contractions of your muscles cause twisting and repetitive movements. The movements can be painful.
  • Huntington's disease, an inherited disease that causes nerve cells in certain parts of the brain to waste away. This includes the nerve cells that help to control voluntary movement.
  • Parkinson's disease, which is disorder that slowly gets worse over time. It causes tremors, slowness of movement, and trouble walking.
  • Tourette syndrome, a condition which causes people to make sudden twitches, movements, or sounds (tics)
  • Tremor and essential tremor, which cause involuntary trembling or shaking movements. The movements may be in one or more parts of your body.

Causes of movement disorders include

  • Genetics
  • Infections
  • Medicines
  • Damage to the brain, spinal cord, or peripheral nerves
  • Metabolic disorders
  • Stroke and vascular diseases
  • Toxins

Treatment varies by disorder. Medicines can cure some disorders. Others get better when an underlying disease is treated. Often, however, there is no cure. In that case, the goal of treatment is to improve symptoms and relieve pain.

  • Angelman syndrome (Medical Encyclopedia)
  • Chronic motor tic disorder (Medical Encyclopedia)
  • Facial tics (Medical Encyclopedia)
  • Movement - uncontrollable (Medical Encyclopedia)
  • Movement - uncontrolled or slow (Medical Encyclopedia)
  • Movement - uncoordinated (Medical Encyclopedia)
  • Movement - unpredictable or jerky (Medical Encyclopedia)
  • Neurodegeneration with brain iron accumulation (NBIA) (Medical Encyclopedia)
  • Tardive dyskinesia (Medical Encyclopedia)

[Learn More]