Ataxia describes a lack of muscle control or coordination of voluntary movements, such as walking or picking up objects. A sign of an underlying condition, ataxia can affect various movements and create difficulties with speech, eye movement and swallowing. Ataxia is a degenerative disease of the nervous system. Many symptoms of Ataxia can mimic those of being drunk slurred speech, stumbling, falling and in coordination. All are related to degeneration of the part of the brain, called the cerebellum, that is responsible for coordinating movement. Ataxia is a disease that affects people of all ages.
Many conditions can cause ataxia, including alcohol misuse, certain medication, stroke, tumor, cerebral palsy, brain degeneration and multiple sclerosis. Inherited defective genes also can cause the condition. The area of your brain that coordinates movement is called the cerebellum. It’s located at the base of your brain just above the brainstem.
Damage to or degeneration of the nerve cells in or around the cerebellum can result in ataxia. Genes you inherit from your parents may also cause ataxia.
Types of ataxia
There are many different types of ataxia, which can be divided into three broad categories:
- Acquired ataxia – Acquired ataxia occurs due to nerve damage from external factors, such as an injury, as opposed to inherited genes. Where symptoms develop as the result of trauma, a stroke, multiple sclerosis, a brain tumor, nutritional deficiencies, or other problems that damage the brain or nervous system.
- Cerebellar ataxia. This type of ataxia results from a dysfunction of the cerebellum, a region of the brain that plays a role in assimilating sensory perception, coordination, and motor control. Cerebellar ataxia can cause neurological symptoms such as: jerking or shaking of the body or limbs when trying to and move decreased muscle tone.
- Hereditary ataxia. Where symptoms develop slowly over many years and are caused by faulty genes that a person inherits from their parents, the most common type is Friedreich’s ataxia.
- Sensory Ataxa. Proprioception is a person’s sense of the relative position of neighboring parts of their body. It is a sense that indicates whether the body is moving with appropriate effort and gives feedback on the position of body parts relative to each other. If a person stands with their eyes closed and feet together, the instability will worsen. This is because a loss of proprioception increases their reliance on visual data.
- Idiopathic late-onset cerebellar ataxia (ILOCA) – where the brain is progressively damaged over time for reasons that are unclear.
- Vestibular ataxia. This type of ataxia affects the vestibular system, which plays a role in hearing. It can result from nerve damage in the ear.
Symptoms for Ataxia
Ataxia can develop over time or come on suddenly. A sign of a number of neurological disorders, ataxia can cause:
- Poor coordination.
- Unsteady walk and a tendency to stumble.
- Difficulty with fine motor tasks, such as eating, writing or buttoning a shirt.
- Tremors, shaking, or trembling in parts of the body.
- Nystagmus, an involuntary, rapid, rhythmic, repetitive eye movement that may be vertical, horizontal, or circular.
- Problems with balance.
- Change in speech.
- Involuntary back-and-forth eye movements (nystagmus).
- Difficulty swallowing.
- Walking difficulties that may lead to wheelchair use.
- Vision and hearing problems.
- A sideward curvature of the spine.
- Weakened heart muscle.
- High arching feet
Causes of Ataxia
Ataxia usually results from damage to a part of the brain called the cerebellum, but it can also be caused by damage to other parts of the nervous system. Ataxia may also result from certain immunological disorders and infections.
There are many types of ataxia. In this article, we discuss some of the more common types, their causes, and the available treatments.
Ataxia can result from:
- a genetic feature
- a health condition that leads to nerve damage, such as a stroke or MS
- a vitamin B-12 deficiency
- an immunological problem
This damage can be part of an underlying condition such as MS, or can be caused by a head injury lack of oxygen to the brain, or long-term, excessive alcohol consumption.
- Head trauma. Damage to your brain or spinal cord from a blow to your head, such as might occur in a car accident, can cause acute cerebellar ataxia, which comes on suddenly.
- Either a blockage or bleeding in the brain can cause ataxia. When the blood supply to a part of your brain is interrupted or severely reduced, depriving brain tissue of oxygen and nutrients, brain cells die.
- Cerebral palsy. This is a general term for a group of disorders caused by damage to a child’s brain during early development — before, during or shortly after birth — that affects the child’s ability to coordinate body movements.
- Autoimmune diseases. Multiple sclerosis, sarcoidosis, celiac disease and other autoimmune conditions can cause ataxia.
In order to make a diagnosis, your healthcare provider will first request your medical history. They’ll ask if you have a family history of inherited ataxia.
They may also ask about the medications you take and your level of alcohol consumption. They’ll then perform physical and neurological evaluations.
- Imaging tests. A CT or MRI scan can create detailed images of your brain. This can help your doctor to see any abnormalities or tumors.
- Blood tests. Blood tests can be used to help determine the cause of your ataxia, particularly if it’s due to an infection, vitamin deficiency, or hypothyroidism.
- Lumbar puncture (spinal tap). With a lumbar puncture, a sample of cerebrospinal fluid (CSF) is collected from between two vertebrae in the lower back. The sample is then sent to a lab for testing.
- Genetic testing. Genetic testing is available for many types of inherited ataxias. This type of testing uses a blood sample to see if you have genetic mutations associated with an inherited ataxia.
In most cases, there’s no cure for ataxia and supportive treatment to control the symptoms is necessary.
This may include:
- Coordination and balance issues: Adaptive devices such as walking sticks, crutches, walkers, and wheelchairs can help a person maintain independence. It may be necessary to make some home adaptations, such as to enable wheelchair access.
- Tremor, stiffness, muscle spasms, and weakness: A doctor may prescribe medication and physical or occupational therapy to help improve a person’s strength, maintain their mobility, and help them find new ways to carry out tasks.
- Curvature of the spine: A doctor may recommend orthopedic care.
- Speech and language therapy to help with speech and swallowing problems.
- Physiotherapy to help with movement problems
- Occupational therapy to help you cope with the day-to-day problems
- Medication to control muscle, bladder, heart and eye problems
- Immune problems: Treatment for ataxia telangiectasia may include gamma-globulin injections to boost the immune system.
In a few cases, it’s possible to improve ataxia or stop it getting worse by treating the underlying cause.
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