Acute Cerebellar Ataxia (ACA)
What is acute cerebellar ataxia?
Acute cerebellar ataxia (ACA) is a disorder that occurs when the cerebellum becomes inflamed or damaged. The cerebellum is the area of the brain responsible for controlling gait and muscle coordination.
The term ataxia refers to a lack of fine control of voluntary movements. Acute means the ataxia comes on quickly, on the order of minutes to a day or two. ACA is also known as cerebellitis.
People with ACA often have a loss of coordination and may have difficulty performing daily tasks. The condition most commonly affects children, particularly those between ages 2 and 7. However, it occasionally affects adults as well.
What causes acute cerebellar ataxia?
Viruses and other diseases that affect the nervous system can injure the cerebellum. These include:
- hepatitis A
- infections caused by the Epstein-Barr and Coxsackie viruses
- West Nile virus
ACA can take weeks to appear following a viral infection.
Other causes of ACA include:
- bleeding in the cerebellum
- exposure to mercury, lead, and other toxins
- bacterial infections, such as Lyme disease
- head trauma
- deficiencies of certain vitamins, such as B-12, B-1 (thiamine), and E
What are the symptoms of acute cerebellar ataxia?
The symptoms of ACA include:
- impaired coordination in the torso or arms and legs
- frequent stumbling
- an unsteady gait
- uncontrolled or repetitive eye movements
- trouble eating and performing other fine motor tasks
- slurred speech
- vocal changes
These symptoms are also associated with several other conditions that affect the nervous system. It’s important to see your doctor so they can make a proper diagnosis.
How is acute cerebellar ataxia diagnosed?
Your doctor will run several tests to determine whether you have ACA and to find the underlying cause of the disorder. These tests can include a routine physical exam and various neurological assessments. Your doctor may also test your:
If you weren’t infected with a virus recently, your doctor will also look for signs of other conditions and disorders that commonly lead to ACA.
There are a number of tests your doctor can use to evaluate your symptoms, including:
- Nerve conduction study . A nerve conduction study determines whether your nerves are working correctly.
- Electromyography (EMG). An electromyogram records and evaluates the electrical activity in your muscles.
- Spinal tap . A spinal tap allows your doctor to examine your cerebrospinal fluid (CSF), which surrounds the spinal cord and brain.
- Complete blood count (CBC). A complete blood count determines whether there are any decreases or increases in your number of blood cells. This can help your doctor assess your overall health.
- CT or MRI scan. Your doctor may also look for brain damage using these imaging tests. They provide detailed pictures of your brain, allowing your doctor to get a closer look and evaluate any damage in the brain more easily.
- Urinalysis and ultrasound . These are other tests that your doctor might perform.
How is acute cerebellar ataxia treated?
Treatment for ACA isn’t always necessary. When a virus causes ACA, a full recovery is usually expected without treatment. Viral ACA generally goes away in a few weeks without treatment.
However, treatment is usually required if a virus is not the cause of your ACA. The specific treatment will vary depending on the cause, and can last weeks, years, or even a lifetime. Here are some possible treatments:
- You may need surgery if your condition is the result of bleeding in the cerebellum.
- You may need antibiotics if you have an infection.
- Blood thinners can help if a stroke caused your ACA.
- You can take medications to treat inflammation of the cerebellum, such as steroids.
- If a toxin is the source of ACA, reduce or eliminate your exposure to the toxin.
- If ACA was brought on by a vitamin deficiency, you can supplement high doses of vitamin E, injections of vitamin B-12, or thiamine.
- In some instances, ACA can be brought on through gluten sensitivity. In this case, you need to adopt a strict gluten-free diet.
If you have ACA, you might need help with daily tasks. Special eating utensils and adaptive devices such as canes and speaking aids can help. Physical therapy, speech therapy, and occupational therapy may also help improve your symptoms.
Some people also find that making certain lifestyle changes can further relieve the symptoms. This can include changing your diet or taking nutritional supplements.
How does acute cerebellar ataxia affect adults?
The symptoms of ACA in adults are similar to that of children. As with children, treating adult ACA involves treating the underlying condition that caused it.
While many of the sources of ACA in children may also cause ACA in adults, there are some conditions that are more likely to cause ACA in adults.
Toxins, especially excessive consumption of alcohol, are one of the largest causes of ACA in adults. Additionally, medications such as antiepileptic drugs and chemotherapy are more often associated with ACA in adults.
Underlying conditions such as HIV, multiple sclerosis (MS), and autoimmune disorders may also be more likely to increase your risk of ACA as an adult. Nevertheless, in many cases, the cause of ACA in adults remains a mystery.
When diagnosing ACA in adults, doctors first try to distinguish ACA from other types of cerebellar ataxias that come on more slowly. While ACA strikes within minutes to hours, other forms of cerebellar ataxia may take days to years to develop.
The ataxias with a slower rate of progression may have different causes, such as genetic predispositions, and require different treatments.
As an adult, it’s more likely that you’ll receive brain imaging, such as an MRI, during the course of diagnosis. This imaging may show abnormalities that can cause ataxias with a slower progression.
What other conditions are similar to acute cerebellar ataxia?
ACA is characterized by rapid onset — minutes to hours. There are other forms of ataxia that have similar symptoms but differing causes:
Subacute ataxias develop over days or weeks. Sometimes subacute ataxias can seem to come on quickly, but in reality, they have been developing slowly over time.
Chronic progressive ataxias
Chronic progressive ataxias develop and last over months or years. They are often caused by hereditary conditions.
Chronic progressive ataxias can also be due to mitochondrial or neurodegenerative disorders. Other diseases may cause or mimic chronic ataxias as well, such as migraine headache with brainstem aura, a rare syndrome where ataxia accompanies migraine headache.
Congenital ataxias are present at birth and are often permanent, although some can be treated with surgery. These ataxias are caused by congenital structural abnormalities of the brain.
What complications are associated with acute cerebellar ataxia?
The symptoms of ACA might become permanent when the disorder is caused by a stroke, an infection, or bleeding into the cerebellum.
Joining a support group or meeting with a counselor can help you cope with your symptoms and any challenges you’re facing.
Is it possible to prevent acute cerebellar ataxia?
As an adult, you can reduce your risk of ACA by avoiding excessive alcohol consumption and other toxins. Reducing your risk of stroke by exercising, maintaining a healthy weight, and keeping blood pressure and cholesterol in check can also be helpful in preventing ACA.
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