general description
Cerebellum and brainstem
Cerebellum and brainstem
Persistent ataxia usually results from damage to the part of the brain that controls muscle coordination (the cerebellum).
Ataxia describes poor muscle control that results in awkward voluntary movements. It can cause difficulties with walking and balance, hand coordination, speaking and swallowing, and eye movement.
Ataxia usually results from damage to the part of the brain that controls muscle coordination (the cerebellum) or its connections. Many conditions can cause ataxia, including alcohol abuse, stroke, tumor, brain degeneration, multiple sclerosis, certain medications, and genetic disorders.
Treatment of ataxia depends on the cause. Adaptive devices, such as walkers or canes, can help maintain independence. Physical therapy, occupational therapy, speech therapy, and regular aerobic exercise can also help.
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Symptoms
Ataxia can develop over time or come on suddenly. Ataxia is a sign of several neurological disorders and can cause:
- poor coordination
- Unsteady or wide-footed walking
- poor balance
- Difficulty with fine motor tasks like eating, writing, or buttoning a shirt
- change in speech
- Involuntary movements of the eyes back and forth (nystagmus)
- Difficulty to swallow
When to see a doctor
Unless you have a condition that causes ataxia, such as multiple sclerosis, see your doctor as soon as possible if:
- lose balance
- Loss of muscle coordination in the hand, arm, or leg
- have difficulty walking
- blur your speech
- have difficulty swallowing
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Causes
Damage to the part of the brain that controls muscle coordination (the cerebellum) or its connections can cause ataxia. The cerebellum, located at the base of the brain, connects to the brain stem. The cerebellum helps control balance, eye movements, swallowing, and speech. There are three main groups of causes of ataxia: acquired diseases, degenerative diseases, and hereditary causes.
Acquired causes:
- Alcohol.Long-term heavy drinking can cause persistent ataxia. This may be improved by avoiding alcohol altogether.
- Medicines.Ataxia is a potential side effect of certain medications, especially barbiturates such as phenobarbital; sedatives such as benzodiazepines; antiepileptics such as phenytoin; and some types of chemotherapy.
- toxinsHeavy metal poisoning, such as lead or mercury, and solvent poisoning, such as paint thinner, can also cause ataxia.
- vitaminsInsufficient amounts of vitamin E, vitamin B-1, vitamin B-12, or thiamine can cause ataxia.deficiency or excessVitamin B-6 deficiency can also cause ataxia. It is important to identify these causes because ataxia caused by these deficits can often be reversed.
- thyroid problems.Hypothyroidism and hypoparathyroidism can cause ataxia.
- Attack.The abrupt onset of ataxia occurs with a stroke. This can be caused by blocked blood vessels or bleeding in the brain.
- Multiple sclerosis.This neurological disorder can cause ataxia.
- Autoimmune diseases.Sarcoidosis, celiac disease, some types of encephalomyelitis, and other autoimmune diseases can cause ataxia.
- infectionsAtaxia may be a rare complication of childhood chickenpox and other viral infections such asHIVand Lyme disease. It can appear during the healing stages of the infection and persist for days or weeks. Symptoms usually go away over time.
- COVID-19infection.Most of the time, ataxia results from a severeCOVID-19Affairs.
- Paraneoplastic syndromes.These are rare degenerative diseases caused by the immune system's response to a malignant tumor (cancer), most commonly lung, ovarian, or breast cancer or lymphoma. Ataxia can appear months or years before the cancer is diagnosed.
- Abnormalities in the brain.An infected area (abscess) in the brain can cause ataxia. A growth in the brain, either malignant (malignant) or benign (benign), can damage the cerebellum.
- Head damage.Severe brain damage can cause cerebellar ataxia weeks or months after the injury.
- Cerebral palsy.This is a general term for a group of disorders caused by damage to a child's brain early in development (before, during, or shortly after birth) that affects the child's ability to coordinate body movements.
Degenerative causes:
- Multisystem atrophy.This applies to adults around the age of 50. In addition to ataxia, other problems associated with the condition include urinary incontinence, low blood pressure or fainting when standing, andrapid eye movementsleep behavior disorder (yelling, kicking, or hitting while sleeping).
Hereditary causes:
Some types of ataxia and some conditions that cause ataxia are hereditary. If you have one of these conditions, you may have been born with a mutation in a certain gene that produces irregular proteins.
Abnormal proteins prevent nerve cells, mainly in the cerebellum and spinal cord, from functioning and cause their degeneration. As the disease progresses, coordination problems worsen.
You can inherit genetic ataxia of the dominant gene from one parent (autosomal dominant disorder) or the recessive gene from both parents (autosomal recessive disorder). In a recessive disorder, the parents are not affected and there may be affected siblings.
Different gene mutations cause different types of ataxia, most of which are progressive. Each type causes poor coordination, but each has specific signs and symptoms.
Autosomal dominant ataxias
Autosomal dominant pattern of inheritance
Autosomal dominant pattern of inheritance
In an autosomal dominant disorder, the altered gene is the dominant gene. It is found on one of the non-sex chromosomes called autosomes. It only takes one altered gene to be affected by this type of disorder. A person with an autosomal dominant disorder, in this example the father, has a 50% chance of having an affected child with an altered gene. A person has a 50% chance of having a healthy baby.
- Ataxia espinocerebelosa.Scientists have identified more than 40 autosomal dominant ataxia genes, and the number continues to grow. Cerebellar ataxia and cerebellar degeneration are common to all types, and other neurologic signs and symptoms may be present.
- Episodic ataxia (EA).There are eight recognized types of ataxia that are episodic rather than progressive:EE. UU.1 forEE. UU.7 plus late-onset episodic ataxia.EE. UU.1 yoEE. UU.2 are the most common.EE. UU.1 involves brief episodes of ataxia that can last seconds or minutes. The episodes are triggered by stress, surprise, or sudden movement, and are often associated with muscle spasms.EE. UU.2 includes longer episodes, typically lasting anywhere from 30 minutes to six hours, which are also triggered by stress. Dizziness (vertigo), fatigue, and muscle weakness may occur during episodes. In some cases, the symptoms resolve later in life. Episodic ataxia does not shorten life, and symptoms may respond to medication.
autosomal recessive ataxias
Autosomal recessive pattern of inheritance
Autosomal recessive pattern of inheritance
To have an autosomal recessive disorder, you inherit two altered genes, sometimes called mutations. You get one from each parent. Their health is rarely affected as they only have one altered gene. Two carriers have a 25% chance of giving birth to a healthy child with two unaltered genes. They have a 50% chance of giving birth to a healthy baby who is also a carrier. They have a 25% chance of having an affected child with two altered genes.
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ataksya Friedreicha.It is the most common hereditary ataxia. It involves damage to the cerebellum, spinal cord, and peripheral nerves. Peripheral nerves carry signals from the arms and legs to the brain and spinal cord. In most cases, the signs and symptoms appear well before the age of 25. The cerebellum usually looks normal on a brain scan.
The first sign is general difficulty walking. The condition usually progresses to the arms and trunk. Foot deformities, such as high arches and curvature of the spine (scoliosis), are common.
Other signs and symptoms that may develop include slurred speech (dysarthria); fatigue; involuntary eye movements (nystagmus); hearing loss; enlarged heart (cardiomyopathy) and heart failure and diabetes. Early treatment of heart problems can improve quality of life and survival.
- RFC1-related ataxia:This is the most common cause of tardive ataxia. The symptoms of ataxia are often accompanied by dizziness, numbness or tingling in the body, and sometimes an unexplained cough.
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Ataxia telangiectasia.This rare progressive childhood disease causes degeneration of the brain and immune system. This increases the risk of other diseases, including infections and cancers.
Telangiectasias are tiny red "spider veins" that can appear in the corners of your baby's eyes or on the ears and cheeks. The first signs are usually delayed motor development, poor balance, and slurred speech. Sinus and respiratory infections are common.
Children with ataxia telangiectasia are at high risk of developing cancer, especially leukemia or lymphoma.
- Congenital cerebellar ataxia.This type of ataxia results from damage to the cerebellum that is present at birth.
- Wilson's disease.People with this disease accumulate copper in the brain, liver, and other organs. This can cause ataxia and other neurological problems.
By Mayo Clinic staff
FAQs
What is the main cause of ataxia? ›
Ataxia is usually caused by damage to a part of the brain known as the cerebellum, but it can also be caused by damage to the spinal cord or other nerves. The spinal cord is a long bundle of nerves that runs down the spine and connects the brain to all other parts of the body.
What are the early signs of ataxia? ›- Balance and coordination are affected first.
- Poor coordination of hands, arms, and legs.
- Slurring of speech.
- Wide-based gait (manner of walking)
- Difficulty with writing and eating.
- Slow eye movements.
People with the condition tend to have a shorter life expectancy than normal. Many people live until at least their 30s, and some can live into their 60s or beyond.
What can be mistaken for ataxia? ›Even in families where hereditary ataxia has presented in multiple generations, patients may struggle for an accurate diagnosis because the disease can be mistaken for other movement disorders, such as multiple sclerosis or amyotrophic lateral sclerosis.
Does ataxia ever go away? ›In most cases, there's no cure for ataxia and supportive treatment to control the symptoms is necessary. This may include: speech and language therapy to help with speech and swallowing problems. physiotherapy to help with movement problems.
What is the best treatment for ataxia? ›episodic ataxia can often be controlled with a medication called acetazolamide and by avoiding triggers such as stress, alcohol and caffeine. acquired ataxia can sometimes be treated depending on the specific cause – for example, antibiotic or antiviral medication may help if it's caused by an infection.
What are 3 causes of ataxia? ›Ataxia usually results from damage to the part of the brain that controls muscle coordination (cerebellum) or its connections. Many conditions can cause ataxia, including alcohol misuse, stroke, tumor, brain degeneration, multiple sclerosis, certain medications and genetic disorders.
What foods should be avoided with ataxia? ›Ataxia patients may sometimes benefit by avoiding simple carbohydrates. What this means is to eliminate foods sweetened with high fructose corn syrup, sugar, and artificial sweetener; no or very small amounts of cookies, cakes, candies, pastries, white flour, and fruit juice.
How quickly does ataxia progress? ›Rapid progression of ataxia (within months) should prompt a search for underlying malignancy, including with serological testing for paraneoplastic antibodies. 4 A fluorodeoxyglucose positron emission tomography study may be indicated, even if the CT scan of thorax, abdomen and pelvis is normal.
Can you reverse ataxia? ›There is no specific treatment for ataxia. In some cases, treating the underlying cause may help improve the ataxia. In other cases, such as ataxia that results from chickenpox or other viral infections, it is likely to resolve on its own.
Is ataxia a form of dementia? ›
Dementia occurs only in some forms of spinocerebellar ataxia (SCA), such as SCA1,1 SCA2, SCA3,2 and SCA12,3 developing in the latest stages of the disease.
Is ataxia a form of Parkinson's? ›Ataxia in the sense of uncoordinated movement is a primary symptom of Parkinson's Disease, while Ataxia as shorthand for Spinocerebellar Ataxia refers to a set of genetic conditions with which Parkinson's Disease has some symptoms in common.
What drugs cause ataxia? ›Benzodiazepines – Most commonly described in children with epilepsy. Ataxia is often mild and reversible. Elderly patients are also susceptible to benzodiazepine ataxia. Others – Carbamazepine, oxcarbazepine, lacosamide, lamotrigine, rufinamide, zonisamide, ezogabine, gabapentin, felbamate, and phenobarbital.
Is ataxia a symptom of ALS? ›In the most severe cases, ataxia resembles ALS, making it difficult to swallow and eventually to breathe.
Can a CT scan show ataxia? ›Ataxia can also be caused by a stroke. The appropriate imaging of ataxia depends on the suspected cause. When ataxia occurs after head injury, CT scan of the brain without intravenous (IV) contrast is usually an appropriate initial imaging test.
What is the blood test for ataxia? ›Genetic testing involves taking a sample of blood and testing the DNA in it for any genetic mutation known to cause ataxia. Currently, tests can detect the mutations responsible for Friedreich's ataxia, ataxia-telangiectasia and most of the spinocerebellar ataxias.
What vitamin is good for ataxia? ›Description. Ataxia with vitamin E deficiency is a disorder that impairs the body's ability to use vitamin E obtained from the diet. Vitamin E is an antioxidant, which means that it protects cells in the body from the damaging effects of unstable molecules called free radicals.
What is the physical exam test for ataxia? ›Romberg's test is used to assess for loss of proprioceptive or vestibular function (known as sensory ataxia). The test does not assess cerebellar function and instead is used to quickly screen for evidence of sensory ataxia (i.e. non-cerebellar causes of balance issues).
Can a neurologist help with ataxia? ›Our ataxia team includes neurologists and experts in other specialties, including physical therapy, diet, genetic counseling and speech-language pathology, who all work together to help identify underlying conditions and manage symptoms.
Can you drive with ataxia? ›Most people with a cerebellar ataxia are able to safely drive. It is the duty of someone who develops a cerebellar disorder to notify the road licensing authority in their state, to ensure that their driver's license is valid and that they are covered by their insurance.
What is the new drug for ataxia? ›
The recently approved drug, SKYCLARYS, showed promise in the Phase 2 MOXIe trial for slowing the progression of Friedreich's ataxia. The placebo-controlled, randomized, double-blinded trial tested the use of SKYCLARYS over a 48-week period.
Can caffeine make ataxia worse? ›Episodes of ataxia and other symptoms can begin anytime from early childhood to adulthood. They can be triggered by environmental factors such as stress, caffeine, alcohol, certain medications, physical activity, and illness.
Is ataxia a stroke? ›A stroke that affects the cerebellum can cause problems with coordination and muscle control. This means that your nervous system may struggle to coordinate movement, which is a condition known as ataxia.
Does coffee affect ataxia? ›The several symptoms of ataxia may occur due to various underlying causes. However, certain factors like stress, caffeine, and specific medications also trigger the development of ataxia. Prolonged intake of caffeine may lead to loss of motor skills and problems with balance and coordination.
Does vitamin B12 deficiency cause ataxia? ›Conclusion: Ataxia cerebellar dementia and leukoencephalopathy can result from vitamin B12 deficiency.
Does exercise help ataxia? ›“The aerobic training group showed improvement in ataxia, which is the primary complaint of these patients.” With no available disease-modifying medications, balance training to improve motor skills and functional performance has been the mainstay of treatment.
Will I be in a wheelchair with cerebellar ataxia? ›Although not everyone with ataxia uses a wheelchair, many people find it makes life easier. Some people can walk short distances or stand for a short period; they may use a wheelchair for the rest of the time.
Can stress cause ataxia? ›Stress is the most common trigger among episodic neurologic disorders. In episodic ataxia type 2 (EA2), physical or emotional stress causes episodes of severe motor dysfunction that manifest as ataxia and dystonia.
Can you live a normal life with ataxia? ›It largely depends on the type of ataxia you have. Some types may stay stable or even improve with time. But most will get progressively worse over many years. Life expectancy is generally shorter than normal for people with hereditary ataxia, although some people can live well into their 50s, 60s or beyond.
What does gait ataxia look like? ›An unsteady, staggering gait is described as an ataxic gait because walking is uncoordinated and appears to be 'not ordered'. Many motor activities may be described as ataxic if they appear to others, or are perceived by patients, as uncoordinated.
What are the 3 types of ataxia? ›
The three types of ataxia, according to the location, are cerebellar, sensory, and vestibular.
Can ataxia be caused by Covid? ›Many neurological manifestations of COVID‐19 have been reported in the literature that range from mild non‐specific features such as headache, lethargy or confusion; isolated focal signs including hyposmia, focal seizures, eye‐movement abnormalities or ataxia; through to severe life‐threatening conditions including ...
Is ataxia a degenerative disease? ›Ataxia is a degenerative disease of the nervous system. Many symptoms of Ataxia mimic those of being drunk, such as slurred speech, stumbling, falling, and incoordination. These symptoms are caused by damage to the cerebellum, the part of the brain that is responsible for coordinating movement.
What virus causes ataxia? ›Viral infections that may cause this include chickenpox, Coxsackie disease, Epstein-Barr, echovirus, among others. Other causes of acute cerebellar ataxia include: Abscess of the cerebellum. Alcohol, medicines, insecticides, and illicit drugs.
Is ataxia due to neuropathy? ›As the name implies, people with ataxia neuropathy spectrum typically have problems with coordination and balance (ataxia) and disturbances in nerve function (neuropathy). The neuropathy can be classified as sensory, motor, or a combination of the two (mixed).
What are 4 symptoms of ALS? ›- Muscle twitches in the arm, leg, shoulder, or tongue.
- Muscle cramps.
- Tight and stiff muscles (spasticity)
- Muscle weakness affecting an arm, a leg, the neck, or diaphragm.
- Slurred and nasal speech.
- Difficulty chewing or swallowing.
Neuroradiological diagnosis of hereditary degenerative ataxias can be very challenging, given that usually brain MRI scans show, in most of these conditions, the presence of non-specific and sometimes overlapping imaging findings.
What vitamin deficiencies cause ataxia? ›Ataxia with vitamin E deficiency (AVED) is a rare cause of hereditary ataxia in developing countries with unknown prevalence. AVED is an autosomal-recessive disorder, which is characterized by ataxia, areflexia, and proprioceptive and vibratory sensory loss.
Does B12 help ataxia? ›Depending on severity of symptoms treatment can be parenteral or oral with cyanocobalamin or hydroxocobalamin. 1. Patients diagnosed with ataxia and Vitamin B12 deficiency should be treated with Vitamin B12.
How fast does ataxia progress? ›Rapid progression of ataxia (within months) should prompt a search for underlying malignancy, including with serological testing for paraneoplastic antibodies. 4 A fluorodeoxyglucose positron emission tomography study may be indicated, even if the CT scan of thorax, abdomen and pelvis is normal.
What is the new medicine for ataxia? ›
FDA has approved Skyclarys (omaveloxolone) as the first treatment for Friedreich's ataxia, a rare, inherited, degenerative disease that damages the nervous system, characterized by impaired coordination and walking.