Cerebellar Ataxia Syndrome

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What is cerebellar ataxia syndrome?

The term cerebellar ataxia syndrome is a description of a set of symptoms rather than a specific diagnosis or disease. Ataxia refers to an unsteadiness of gait or lack of muscle coordination. Cerebellar refers to the part of the brain called the cerebellum. The cerebellum is located inside the back and base of the skull, just above the top of the spinal cord. It processes input from other areas of the brain, the spinal cord, and sensory receptors. It is responsible for coordination and balance.

In cerebellar ataxia syndrome, people may have an unsteady gait or lack of coordination because of problems with the cerebellum. Cerebellar ataxia can be classified as acute (happening suddenly and rapidly) or chronic (happening slowly over time). It can also be recurrent (happening on and off over time) or progressive (getting worse over time).

Symptoms of cerebellar ataxia syndrome can appear without any obvious cause, but there are many diseases, disorders and conditions that can cause it. They include infectious diseases, inherited or genetic conditions, certain medications, tumors, trauma, and vascular conditions. Because of the range of possible causes of cerebellar ataxia syndrome, a correct diagnosis of the underlying disease, disorder or condition is important. Contact your healthcare provider for a physical exam if you have an unsteady gait, difficulty walking or talking, or problems with coordination.

The exact incidence of cerebellar ataxia syndrome in the United States is not known. However, it can happen at any age, in both males and females. Children younger than three are most likely to experience acute cerebellar ataxia, usually after a viral infection, such as chickenpox. Adults are more likely to experience chronic cerebellar ataxia that is associated with another neurological condition, such as multiple sclerosis or tumors.

Treatment of cerebellar ataxia syndrome depends on the underlying disease, disorder or condition. Acute cerebellar ataxia caused by viral infections will usually go away without treatment after several weeks. Treating other underlying diseases, disorders or conditions can help improve symptoms. Otherwise, treatment is supportive and is aimed at relieving symptoms and assisting with daily activities.

In some cases, the sudden appearance of the symptoms of cerebellar ataxia syndrome may indicate a serious and potentially life-threatening condition. Seek immediate medical care (call 911) for sudden symptoms, such as difficulty speaking or walking, uncoordinated body movements, and severe headache or continued dizziness.

Seek prompt medical care if you have mild symptoms that are persistent, recur, or cause you concern.

What are the symptoms of cerebellar ataxia syndrome?

Ataxia is a term that means incoordination, unsteadiness or wobbliness. Cerebellar ataxia is ataxia that is caused by problems with the cerebellum. The cerebellum is the area of your brain responsible for coordination and balance.

Common symptoms of cerebellar ataxia syndrome include:

  • Clumsiness in daily activities

  • Difficulty speaking or clumsy speech (dysarthria)

  • Difficulty walking or unsteady gait

  • Dizziness and headache

  • Nausea and vomiting

  • Uncoordinated body movements

  • Uncoordinated eye movements (nystagmus)

Symptoms that might indicate a serious or life-threatening condition

The sudden appearance of the symptoms of cerebellar ataxia syndrome may be a sign of a serious and even life-threatening condition, including stroke, poisoning, encephalitis, or brain abscess. Seek immediate medical care (call 911) if you, or someone you are with, have a sudden onset of any of the above symptoms of cerebellar ataxia syndrome.

What causes cerebellar ataxia syndrome?

Cerebellar ataxia syndrome is caused by damage to or problems with the cerebellum. The cerebellum is the part of your brain that is responsible for coordination and balance. A wide variety of diseases, disorders and conditions can lead to problems with the cerebellum and cause ataxia.

Cerebellar ataxia syndrome can be categorized based on the cause of the problem.

Autoimmune and inflammatory causes of cerebellar ataxia syndrome

Autoimmune and inflammatory causes may include:

  • Behcet’s disease (disease that causes damage to blood vessels, especially the veins)

  • Celiac disease (severe sensitivity to gluten from wheat and other grains that causes intestinal damage)

  • Multiple sclerosis (disease that affects the brain and spinal cord causing weakness, coordination and balance difficulties, and other problems)

  • Paraneoplastic degeneration (rare immune system disorder that attacks the cerebellum)

Congenital and developmental causes of cerebellar ataxia syndrome

Congenital causes are disorders that are present at or before birth. They may include:

  • Agenesis (failure of the cerebellum to fully develop)

  • Chiari malformation (herniation of the cerebellum through the opening in the base of the skull)

  • Dandy-Walker cyst (congenital malformation of the cerebellum)

  • Joubert syndrome (absence or underdevelopment of the cerebellum)

Genetic or inherited causes of cerebellar ataxia syndrome

There are many rare genetic disorders that can cause cerebellar ataxia syndrome including:

  • Ataxia telangiectasia (rare disorder that causes degeneration of the cerebellum)

  • Friedreich’s ataxia (rare disease that causes degeneration of the cerebellum, spinal cord, and peripheral nerves)

  • Kearns-Sayre syndrome (rare neuromuscular disorder caused by DNA abnormalities)

  • Mitochondrial encephalopathy with ragged red fibers (MERRF, a metabolic disorder)

  • Mitochondrial encephalopathy, lactic acidosis, stroke-like episodes (MELAS syndrome, a metabolic disorder)

  • Multiple systems atrophy (rare, progressive neurological disorder)

  • Neuropathy, ataxia, retinitis pigmentosa (NARP, a metabolic disorder)

  • Spastic ataxia of Charlevoix-Saguenay (gene mutation)

Infectious causes of cerebellar ataxia syndrome

Infectious causes of cerebellar ataxia syndrome may include:

Metabolic causes of cerebellar ataxia syndrome

Metabolic causes of cerebellar ataxia syndrome may include:

  • Hypoparathyroidism (underactive parathyroid gland)

  • Thyroid diseases

  • Vitamin B12 deficiency

  • Vitamin E deficiency

Pharmacologic causes of cerebellar ataxia syndrome

Medications that may cause cerebellar ataxia syndrome include:

  • Amiodarone (Cordarone)

  • Carbamazepine (Tegretol)

  • Certain cancer chemotherapies

  • Cyclosporine (Gengraf, Neoral, Sandimmune)

  • Lithium (Eskalith, Lithobid)

  • Phenytoin (Dilantin)

Toxic causes of cerebellar ataxia syndrome

Toxins and poisons that may cause cerebellar ataxia syndrome include:

  • Alcohol

  • Carbon monoxide

  • Heavy metals, such as mercury, lead and thallium

  • Heroin

  • Organic solvents, such as toluene and benzene

  • Phencyclidine (PCP)

Other causes of cerebellar ataxia syndrome

Other causes of cerebellar ataxia syndrome may include:

  • Brain tumors

  • Stroke and other vascular conditions

  • Trauma

What are the risk factors for cerebellar ataxia syndrome?

A number of factors are thought to increase your chances of developing cerebellar ataxia syndrome. Common risk factors include:

  • Exposure to certain drugs or toxins

  • Having a family member with a genetic form of cerebellar ataxia

  • Head trauma

  • Recent vaccination

  • Viral infections

How is cerebellar ataxia syndrome treated?

Treatment of cerebellar ataxia syndrome varies depending on the cause, severity, and other factors. Cerebellar ataxia caused by viral infections will often go away on its own within a few months. Treating the underlying cause, if one is identified, can help improve problems with balance and coordination. Otherwise, treatment is supportive and is aimed at managing your symptoms and assisting with activities of daily life.

Treatment of cerebellar ataxia syndrome may include:

  • Devices to assist with walking and other activities

  • Medications to improve balance, tremor and nystagmus (rapid, uncontrolled eye movements)

  • Nutritional counseling and therapy to correct vitamin deficiencies and improve your overall health and wellness

  • Occupational therapy to improve your functionality

  • Physical therapy to strengthen your muscles and reduce tension

  • Speech therapy to improve speaking and communication

  • Surgery to treat certain underlying causes of cerebellar ataxia such as a brain tumor

  • Swallowing therapy to strengthen your swallowing muscles

What are the potential complications of cerebellar ataxia syndrome?

Complications of the underlying causes of cerebellar ataxia syndrome and the prognosis vary based on the disease, disorder or condition. You can best treat cerebellar ataxia syndrome and its underlying cause, lower your risk of complications, or delay the development of complications by following the treatment plan you and your healthcare professional design specifically for you.

Complications of cerebellar ataxia syndrome include:

  • Difficulties with work and school

  • Difficulty performing everyday activities, such as walking, communicating and self-care

  • Disability

  • Falls and injuries

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2021 Jan 16
  1. Acute cerebellar ataxia. Medline Plus, a service of the National Library of Medicine National Institutes of Health. http://www.nlm.nih.gov/medlineplus/ency/article/001397.htm.
  2. Acute cerebellar ataxia. New York University Langone Medical Center. http://www.med.nyu.edu/content?ChunkIID=191914.
  3. NINDS ataxias and cerebellar or spinocerebellar degeneration information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/ataxia/ataxia.htm
  4. Cerebellar disorders. The Merck Manual.  http://www.merckmanuals.com/professional/neurologic_disorders/movement_and_cerebellar_disorders/cere....
  5. Cerebellum – function. Medline Plus, a service of the National Library of Medicine National Institutes of Health. http://www.nlm.nih.gov/medlineplus/ency/imagepages/18008.htm.
  6. Schmahmann JD. Disorders of the cerebellum: ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome. J Neuropsychiatry Clin Neurosci. 2004;16:367-378.
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