Cerebellar Ataxia
Cerebellar Ataxia
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Author: Kristin Barañano, MD, PhD
Johns Hopkins Medicine

Reviewed: November 2022


Ataxia is a condition where a person lacks muscle coordination. It’s not because of weakness. There are many possible causes.  

Ataxia can develop suddenly. It can also develop slowly over time. This timing is very important. It helps determine the underlying cause. 

The outcome depends on the cause. The cause also determines whether there are treatment options.


Disorder Overview


The term “ataxia” means “lack of coordination.” It’s not due to muscle weakness. 

Nervous system problems can cause ataxia. For example, it can result from problems in the inner ear. Specifically, the vestibular apparatus is affected. This structure senses movements of the head. 

Problems with sensory nerves can also cause ataxia. In this case, the brain does not receive important information. This information would tell it the position of arms and legs.

Finally, ataxia can be caused by problems in the cerebellum. This structure is in the back of the brain. It’s found near the base of the skull. The cerebellum processes information from:  

  • The motor command centers of the brain 
  • The eyes 
  • The inner ear 
  • The sensory nerves

This information allows it to fine-tune motor movements. That way, they are accurate and appropriate.

The cerebellum is very sensitive to: 

  • Certain medications  
  • Alcohol

It can be damaged by: 

The immune system can also attack it.

Many genetic disorders affect cerebellar function. Sometimes these worsen over time. 

The cerebellum also plays an important role in:  

  • Cognition  
  • Emotions 


Signs of cerebellar ataxia include: 

  • A wide-based walk (gait ataxia) 
  • Jiggling eye movements (nystagmus) 
  • Slurred speech (dysarthria) 
  • Trouble swallowing (dysphagia) 
  • Wobbling of the head and trunk (titubation) 
  • Shakiness when reaching (dysmetria) 

Ataxia can have other signs and symptoms. It depends on the cause. These symptoms include: 

  • Muscle tightness (spasticity) 
  • Seizures (epilepsy) 
  • Involuntary jerks (myoclonus) 
  • Dizziness (vertigo) 
  • Tremors 
  • Developmental delays and learning problems 
  • Low core tone (hypotonia) 
  • Attention issues (ADHD) 
  • Fatigue 


Cerebellar ataxia can have many causes. It helps to divide them based on how quickly they start.

Acute cerebellar ataxia (ACA) 

With ACA, symptoms occur suddenly. They are very noticeable. 

In children, ACA is commonly caused by eating or drinking a substance. Many medications and alcohol can have this effect.  

Infections can cause inflammation in the cerebellum. They can be viral or bacterial. This can lead to ataxia as well.  

Ataxia can also happen very rarely after an immunization. The immune system responds to the vaccine appropriately, but cross-reacts with the cerebellum. This type of ataxia happens much more rarely than ataxia caused by the disease the vaccine prevents. 

Autoimmune processes can attack the cerebellum. They can cause ataxia too. (This is similar to multiple sclerosis.) 


Subacute cerebellar ataxia 

With this type of ataxia, symptoms develop slowly. They occur over:  

  • Minutes 
  • Hours 
  • Days 
  • Weeks 

With infections, symptoms can develop over hours or days. Strokes can also occur. This can happen when: 

  • A clot blocks blood flow to the cerebellum. That flow contains oxygen. (This is called an ischemic stroke.) 
  • There is bleeding into the brain. (This is called a hemorrhagic stroke.)  

Tumors in or near the cerebellum may grow slowly. They can gradually cause symptoms. One example is medulloblastoma.  

Rarely, a tumor elsewhere in the body can trigger antibodies. An example of this is neuroblastoma. These antibodies can cross-react with the cerebellum. This can cause ataxia. In this situation, there may be other symptoms. These include:  

  • Chaotic eye movements (opsoclonus) 
  • Involuntary jerks (myoclonus) 

This combination of symptoms is called opsoclonus-myoclonus-ataxia syndrome (OMAS) 


Chronic and progressive cerebellar ataxia 

Chronic cerebellar ataxia is long-lasting. Progressive cerebellar ataxia worsens over time.  

Some children have developmental brain differences. These differences affect the cerebellum. They can cause ataxia. Some conditions like this are:  

Children born prematurely can have a brain injury. Specifically, their motor system can be injured. This can cause cerebral palsy (CP). One type of CP is ataxic CP.  

Some children have congenital ataxia. As babies, they may have:  

As they grow, two things become clear. The first is that their cerebellum does not work properly. The second is that they have signs of cerebellar ataxia. 

Several genetic disorders can cause congenital ataxia. They can cause progressive ataxia too. Sometimes they mostly cause problems in the cerebellum. Other times, the issues are widespread.  

With other genetic disorders, the body cannot break down a certain compound. It then builds up in the nervous system. This causes problems. Examples of these disorders include:  

  • Hexosaminidase deficiency (Tay-Sachs and Sandhoff disease) 
  • Problems with cholesterol metabolism (Niemann-Pick type C) 
  • Copper metabolism (Wilson disease)  
  • White matter disorders (leukodystrophies like metachromatic leukodystrophy)  

Every person has two copies of each gene. An error in one copy can sometimes cause a problem. (This error is also called a spelling change or mutation.) These disorders are “autosomal dominant.”  

These diseases are often called “spinocerebellar ataxias.” They are also known as SCAs. The main symptom is cerebellar ataxia. These go by numbers. (For example, SCA1, SCA2, SCA3, SCA6, SCA7, and so forth.)  

Many of the SCAs are caused by a specific genetic change. It is called a triplet repeat expansion. This tends to cause progressive symptoms. These conditions can be life-limiting.  

Other SCAs are caused by more conventional genetic errors. They have more stable symptoms. One condition like this is SCA29. 

For some disorders, both copies of the gene need to have a change. Otherwise, symptoms do not occur. These are “autosomal recessive” disorders.  

This means that each parent is a carrier of the disorder. However, they do not have symptoms. This is because they have one working copy of the gene. It prevents symptoms from developing. 

When progressive ataxia starts before the age of twenty-five, the most common diagnosis is Friedreich ataxia (FA). FA is an autosomal recessive disorder. It is caused by triplet repeat expansions in the FRDA gene.  

Other better-known ataxias like this are:  

  • Ataxia-telangiectasia (A-T) 
  • AOA1 and AOA2 (ataxia with oculomotor apraxia type 1 and type 2) 
  • ARSACS (autosomal recessive spastic ataxia of Charlevoix-Saguenay) 


Episodic cerebellar ataxia 

Sometimes, children can have attacks of ataxia. They can last for: 

  • Minutes 
  • Hours 
  • Days 

They may not have any neurological issues between attacks.  

One condition is called vestibular migraine. With this, children may have ataxia without a headache 

With some genetic conditions, the body may have trouble creating energy. Cells need this energy. These conditions are called mitochondrial disorders. They can cause episodes of ataxia.  

Finally, there is a group of genetic disorders called “episodic ataxias.” With these, there are changes in key genes. These genes affect the function of the cerebellum. They cause:  

  • Dizziness 
  • Nausea 
  • Ataxia  
  • Headache (sometimes)
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When ataxia occurs suddenly, the blood is typically tested. The urine may be too. Tests look for signs of infection. They also look for abnormal levels of certain compounds. These include ammonia.  

Children are usually screened for possible ingestions of:  

  • Medications 
  • Alcohol  
  • Drugs 

The next step is usually magnetic resonance imaging (MRI). Images of the brain are taken. Sometimes the spine is imaged too. This helps rule out: 

A lumbar puncture can be necessary. This is also called a spinal tap. It tests the cerebrospinal fluid (CSF). The doctor looks for signs of: 

  • Infection  
  • Inflammation 

Sometimes, a child has ataxia that occurs suddenly. However, it does not get better quickly. Symptoms remain after days or weeks.  

In this case, the next steps include:  

  • Additional blood testing to look for: 
    • Vitamin deficiencies 
    • Metabolic disorders
  • Checking for certain antibodies in:  
      • The blood
      • The spinal fluid/CSF (sometimes) 
  • Looking for signs of a tumor in the rest of the body 

For disorders that are chronic or progress slowly, genetic testing is typically needed for diagnosis. 

Disorder directory


Treatment for many kinds of ACA is supportive. This includes those that develop after:  

  • An ingestion 
  • Viral infection 

Some disorders that cause ataxia have specific therapies. These include: 

Acute disseminated encephalomyelitis (ADEM).

Acute disseminated encephalomyelitis (ADEM). Corticosteroids are often helpful.

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Guillain-Barre syndrome (GBS).

Guillain-Barre syndrome (GBS). Immunoglobulin (IVIg) can help. It is given through a vein. It provides healthy antibodies. A plasma exchange can help too. It removes certain antibodies from the blood.

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Paraneoplastic syndromes.

These are related to antibodies caused by a cancer. The malignancy should be treated. Medications can keep the immune system in check.

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Migraines. It can help to avoid triggers. These include: 

  • Lack of sleep 
  • Certain foods 

Certain medications can stop migraines from forming too.   

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Episodic ataxias.

A specific medication can be used. It is called acetazolamide.

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Inherited ataxias.

Certain medications may be helpful. Supplements may be too. It depends on the specific cause.

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Most cases of ACA are due to: 

  • Eating or drinking a substance 
  • Viral infection 

People usually recover well from these.  

Diagnosing a specific genetic ataxia is helpful. It can predict if symptoms will be stable. It can also predict if they will worsen.  

A specific diagnosis is important for: 

  • Clinical trials of new medications  
  • Potential gene therapies in the future 


National Ataxia Foundation

The National Ataxia Foundation’s vision of a world without Ataxia will be accomplished through its primary programs of funding Ataxia research, providing vital programs and services for Ataxia families, and partnering with pharmaceutical companies in the search for treatments and a cure. NAF offers many educational resources for Ataxia, including a comprehensive library of brochures, fact sheets, and books. NAF oversees the Parents of Kids with Ataxia private Facebook group, which currently has over 800 members.

Childhood Stroke 1

Child Neurology Foundation (CNF) solicits resources from the community to be included on this webpage through an application process. CNF reserves the right to remove entities at any time if information is deemed inappropriate or inconsistent with the mission, vision, and values of CNF. 


ClinicalTrials.gov for Cerebellar Ataxia (birth to 17 years).

These are clinical trials that are recruiting or will be recruiting. Updates are made daily, so you are encouraged to check back frequently.   

ClinicalTrials.gov is a database of privately and publicly funded clinical studies conducted around the world. This is a resource provided by the U.S. National Library of Medicine (NLM), which is an institute within the National Institutes of Health (NIH). Listing a study does not mean it has been evaluated by the U.S. Federal Government. Please read the NLM disclaimer for details.    

Before participating in a study, you are encouraged to talk to your health care provider and learn about the risks and potential benefits.

Family Stories 

The National Ataxia Foundation (NAF) shares stories of adults and children living with Ataxia on their Member Stories page.

The information in the CNF Child Neurology Disorder Directory is not intended to provide diagnosis, treatment, or medical advice and should not be considered a substitute for advice from a healthcare professional. Content provided is for informational purposes only.  CNF is not responsible for actions taken based on the information included on this webpage. Please consult with a physician or other healthcare professional regarding any medical or health related diagnosis or treatment options. 


Brandsma R, Verschuuren-Bemelmans CC, Amrom D, Barisic N, Baxter P, Bertini E, Blumkin L, Brankovic-Sreckovic V, Brouwer OF, Bürk K, Catsman-Berrevoets CE, Craiu D, de Coo IFM, Gburek J, Kennedy C, de Koning TJ, Kremer HPH, Kumar R, Macaya A, Micalizzi A, Mirabelli-Badenier M, Nemeth A, Nuovo S, Poll-The B, Lerman-Sagie T, Steinlin M, Synofzik M, Tijssen MAJ, Vasco G, Willemsen MAAP, Zanni G, Valente EM, Boltshauser E, Sival DA. A clinical diagnostic algorithm for early onset cerebellar ataxia. Eur J Paediatr Neurol. 2019 Sep;23(5):692-706. https://doi.org/10.1016/j.ejpn.2019.08.004. Epub 2019 Aug 10. PMID: 31481303. 

Caffarelli M, Kimia AA, Torres AR. Acute Ataxia in Children: A Review of the Differential Diagnosis and Evaluation in the Emergency Department. Pediatr Neurol. 2016 Dec;65:14-30. https://doi.org/10.1016/j.pediatrneurol.2016.08.025. Epub 2016 Sep 8. PMID: 27789117. 

National Ataxia Foundation (NAF) [Internet]. Minneapolis, MN. Available from: https://www.ataxia.org. 

Neuromuscular Disease Center of Washington University. ATAXIAS: General [Internet]. St. Louis: Washington University; 2015. Available from: https://neuromuscular.wustl.edu/ataxia/aindex.html. 

Paulson HL, Shakkottai VG, Clark HB, Orr HT. Polyglutamine spinocerebellar ataxias – from genes to potential treatments. Nat Rev Neurosci. 2017 Oct;18(10):613-626. https://doi.org/10.1038/nrn.2017.92. Epub 2017 Aug 17. PMID: 28855740; PMCID: PMC6420820. 

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