Hereditary Ataxia: Causes, Symptoms, and Types

Medically reviewed by Federica Polidoro, M.D.

Written by Carrie Madormo, RN, MPH

Posted on August 3, 2026

Get smarter about Ataxia in less than 3 minutes for free.

Get It

Key Takeaways

Hereditary ataxia is a group of inherited disorders caused by gene changes that affect balance, coordination, and movement, and there are several types that are passed down through families in different ways.

View all takeaways

If you’ve been diagnosed with a type of hereditary ataxia — a group of inherited disorders that affect balance and coordination — you may worry about passing it on to your children. Hereditary ataxias may be caused by gene changes that run in families. Some types slowly get worse over time, while others cause episodes that come and go.

There are several types of hereditary ataxias, and they are inherited in different ways. In this article, we’ll cover the main types of hereditary ataxia, their causes, and their individual symptoms. We’ll also discuss when to see a genetic counselor.

Types and Causes of Inherited Ataxias

Hereditary ataxias are rare disorders. They’re caused by gene mutations that may be passed from parent to child or may arise for the first time in an affected person.

Many different gene changes can cause hereditary ataxia. Changes in the same gene may cause different conditions, and changes in different genes may cause similar symptoms.

Genes are made up of DNA — the molecule that carries all genetic information. Genes provide instructions for how the body grows, develops, and functions. They may control proteins or other genes.

Most people receive two copies of each gene — one from each parent. When the DNA inside a cell changes, it causes a gene mutation. Certain gene mutations cause hereditary ataxia.

Autosomal Dominant Ataxias

Autosomal dominant ataxias can occur when a person has one disease-causing change in a related gene. The gene change may be inherited from a parent or may arise for the first time in that person.

A person with a disease-causing gene change linked to an autosomal dominant ataxia has a 50 percent chance of passing that change to each child. Depending on the condition, a child who inherits the change may not always develop symptoms. Many different genes are involved in autosomal dominant ataxias.

Many autosomal dominant ataxias are called spinocerebellar ataxias, or SCAs. They are often given numbers or gene-based names, and the list continues to change as researchers identify new genetic causes.

How Common Are They?

An estimated 1 to 5 out of every 100,000 people have an autosomal dominant ataxia. Among people of European descent, about 3 out of every 100,000 people are estimated to have one of these disorders.

Episodic ataxias are a rare group of autosomal dominant ataxias. They affect 1 out of every 100,000 people. Dominant spastic ataxias are a varied group of inherited disorders that have been found in people from many populations.

Autosomal Recessive Ataxias

Autosomal recessive ataxias also run in families. When both parents carry a disease-causing change in the same gene, each child has a 25 percent chance of having the disorder. A child who inherits only one changed copy is usually called a carrier.

Common autosomal recessive ataxias include:

Friedreich ataxia — Caused by changes in the FXN gene
RFC1-related ataxia, including cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) — Caused by changes in the RFC1 gene
Ataxia with oculomotor apraxia type 1 (AOA1) — Caused by changes in the APTX gene
Ataxia with oculomotor apraxia type 2 (AOA2) — Caused by changes in the SETX gene
Ataxia-telangiectasia — Caused by changes in the ATM gene
Spastic paraplegia 7 — Caused by changes in the SPG7 gene

How Common Are They?

About 3 to 6 out of every 100,000 people are estimated to have autosomal recessive ataxia. Friedreich ataxia affects about 1 out of every 29,000 to 50,000 people and is most common among people of European ancestry.

X-Linked Ataxia

X-linked ataxias are passed down through sex chromosomes. Most males have one X and one Y chromosome, while most females have two X chromosomes. However, not everyone fits into this pattern.

X-linked ataxias are more common in males because they typically have just one X chromosome.

Fragile X-associated tremor/ataxia syndrome (FXTAS) is an inherited condition linked to an FMR1 premutation. Not everyone with this gene change develops FXTAS, and the condition affects males more often and usually more severely than females.

Mitochondrial Ataxias

Mitochondria are small structures inside cells that help make energy. Some mitochondrial ataxias are caused by changes in mitochondrial DNA. These changes are usually passed from a mother to their children.

Other mitochondrial ataxias are caused by changes in genes found in the cell’s nucleus. These conditions may follow autosomal dominant, autosomal recessive, or X-linked inheritance.

Symptoms of Inherited Ataxias

Inherited ataxias cause problems with movement. Symptoms vary by the type and stage of ataxia and may include problems with:

Walking
Balance
Speech
Swallowing
Coordination
Fine motor tasks
Back-and-forth movements

People with hereditary ataxia often develop an unsteady gait that can lead to falls. Dizziness or vertigo may also occur in some types, especially episodic or vestibular ataxias. You may notice a tremor or difficulty using your hands and fingers.

Speech problems may present as slurring. It’s also possible to experience choking when eating.

The pattern depends on the type of ataxia. Progressive ataxias usually get worse over time, while episodic ataxias cause attacks that come and go.

Autosomal Dominant Ataxia Symptoms

Spinocerebellar ataxias (SCAs) cause common ataxia symptoms like trouble with walking and coordination. Symptoms often begin in adulthood.

In some SCAs caused by repeat expansions (when a short DNA sequence is repeated too many times), symptoms may begin at an earlier age in younger generations. This is called anticipation, but it does not happen in every type or every family.

SCAs may also cause:

Difficulty with concentration and memory
Vision changes
Abnormal eye movements
Numbness, tingling, or burning in the arms and legs
Uncontrolled body movements

Dominant Spastic Ataxias

Dominant spastic ataxias cause spasticity (muscle stiffness) in the legs in addition to ataxia symptoms.

Episodic Ataxias

Episodic ataxias are often autosomal dominant disorders that cause attacks of movement problems and dizziness. Depending on the type, symptoms may begin in childhood, adolescence, or adulthood.

Autosomal Recessive Ataxia Symptoms

Autosomal recessive ataxias can begin at many different ages, from early childhood to later adulthood. Their severity and rate of progression vary depending on the condition and the person.

Friedreich Ataxia and RFC1-Related Ataxia

One of the most common types of autosomal recessive ataxia — Friedreich ataxia — usually progresses slowly and often starts causing symptoms before age 25. RFC1-related ataxia is also a common cause of recessive ataxia, especially when symptoms begin later in adulthood.

Friedreich ataxia may cause the following symptoms:

Loss of feeling or sensation
Heart problems, such as cardiomyopathy
Diabetes
Curving of the spine

Ataxia With Oculomotor Apraxia

Ataxia with oculomotor apraxia is a group of autosomal recessive ataxias that cause problems with eye movements and movement. AOA1 usually presents in childhood, and AOA2 usually starts during adolescence or young adulthood.

Ataxia Telangiectasia

Ataxia telangiectasia is a recessive form that causes enlarged blood vessels in the eyes and immune problems, in addition to movement problems. It may also raise the risk of certain cancers. It usually begins causing symptoms in the first 10 years of life.

X-Linked Ataxia Symptoms

FXTAS is a late-onset disorder. The likelihood of developing symptoms increases with age and is generally higher in males than in females. It may cause a tremor and may affect thinking (cognition) as well.

When To Talk With a Genetic Counselor

Consider meeting with a genetic counselor if hereditary ataxia runs in your family. Genetic counselors are healthcare providers who help people with genetic conditions understand their diagnosis and likelihood of passing the disease on to their children.

If hereditary ataxia runs in your family, a genetic counselor can help you decide whether testing is right for you. When possible, testing may begin with a relative who has symptoms. If you’re related to someone with hereditary ataxia, testing may help show whether you carry a disease-causing gene change.

For recessive and some X-linked conditions, you may be called a carrier. For dominant conditions, the gene change may mean that you could also develop symptoms.

Genetic testing may identify the exact cause of hereditary ataxia, but it does not always provide a clear answer. Some results are negative or uncertain, and certain gene changes, including repeat expansions, require specialized tests that standard genetic panels may not detect.