G11.9 - Hereditary ataxia, unspecified

Approximate Synonyms

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

• Narcolepsy-. a condition characterized by recurrent episodes of daytime somnolence and lapses in consciousness (microsomnias) that may be associated with automatic behaviors and amnesia. cataplexy; sleep paralysis, and hypnagogic hallucinations frequently accompany narcolepsy. the pathophysiology of this disorder includes sleep-onset rapid eye movement (rem) sleep, which normally follows stage iii or iv sleep. (from neurology 1998 feb;50(2 suppl 1):s2-s7)
• Cerebellar Ataxia-. incoordination of voluntary movements that occur as a manifestation of cerebellar diseases. characteristic features include a tendency for limb movements to overshoot or undershoot a target (dysmetria), a tremor that occurs during attempted movements (intention tremor), impaired force and rhythm of diadochokinesis (rapidly alternating movements), and gait ataxia. (from adams et al., principles of neurology, 6th ed, p90)
• Myoclonic Cerebellar Dyssynergia-. 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)
• Narcolepsy-. a sleep disorder characterized by a tendency for excessive sleepiness during the day which occurs even after adequate sleep in the nighttime. the persons who suffer from this condition experience fatigue and may fall asleep at inappropriate times during the day.

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Patient Education

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

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.

Code History

• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)