Ataxia

Table of Contents

    What Is Ataxia?

    The word ataxia originally comes from the Greek language and means “without order.” It is used to describe someone that has trouble with balance and appears clumsy and disorganized when performing voluntary movements such as walking or picking something up. Ataxia is actually not a disease but rather a sign of an underlying condition.

    Even the most basic movement requires the coordination of multiple, different parts of the nervous system, including the central nervous system (primarily the brain and the spinal cord) and the peripheral nervous system. Damage to or an abnormality in one of these areas may result in ataxia. The most common area of the brain involved in ataxia is the cerebellum.

    Sometimes ataxia results from genetics as there are a wide variety of inherited diseases where the main symptom is ataxia. This form of ataxia is called hereditary ataxia.

    Facts About Ataxia

    • Ataxia is not a disease but rather a description of neurological signs and symptoms.
    • Ataxia that is confined to one side of the body is known as hemiataxia.
    • The cerebellum is the area of the brain that is most commonly responsible for ataxia.
    • Treating ataxia depends on its underlying cause.

    What Are the Symptoms of Ataxia?

    Ataxia patients may experience:

    • Difficulty with balance when standing
    • Difficulty with walking which may include:
      • Unsteadiness, which can lead to falls
      • Walking with your feet spread widely apart
      • Swaying or falling to one side or the other
      • The inability to walk in a straight line
    • Uncoordinated, clumsy hand movements
    • Slurred or difficulty with speech
    • Problems with eye movements that may cause double or blurred vision
    • Trouble swallowing
    • Dizziness

    The 3 Types of Ataxia

    Cerebellar Ataxia

    Cerebellar ataxia refers to ataxia caused by dysfunction of the cerebellum. Cerebellar ataxia is the most common form of ataxia and initially presents as poor balance in patients. As cerebellar ataxia progresses, the patient’s ability to walk is increasingly affected. In advanced cases of cerebellar ataxia, patients are often able to stand or walk only with great assistance.

    Sensory Ataxia

    Sensory ataxia refers to ataxia caused by the loss of proprioception which is the loss of sensitivity to the positions of joints and body parts. Sensory ataxia patients will often walk with an unsteady stomping gait characterized by heavier-than-normal heel strikes.

    Vestibular Ataxia

    Vestibular ataxia is caused by a dysfunction of the patient’s vestibular system which is a part of the sensory nervous system that is responsible for balance, coordination, and spatial orientation.

    NSC’s Approach to Treating Ataxia

    Successfully treating ataxia is dependent on the cause. If the patient’s ataxia is due to exposure to drugs or toxins, treating the ataxia may be as simple as stopping the exposure to the offending drug or toxin. If the ataxia is caused by an underlying condition such as a vitamin deficiency, autoimmune disease, metabolic disease, or even a tumor, treating the underlying condition may help alleviate the ataxia symptoms.

    For ataxia cases where there is no specific treatment available, a combination of speech, physical, and occupational therapy may help patients cope with their symptoms and improve their quality of life.

    You Are More Than Your Symptoms

    Movement disorders are incredibly complex diseases affecting multiple systems in the body. At Neurology Solutions, we believe that these challenging diseases require a comprehensive approach that treats the whole patient— not a list of symptoms. 

    What It’s Like to Live With Ataxia

    People with ataxia often have trouble walking, a condition that’s also known as “unsteady gait” (or even “ataxic gait.”) This can lead to some unwanted and potentially dangerous events such as losing one’s balance, stumbling, or falling which can result in serious injury.

    What Are the Causes of Ataxia?

    Ataxia is not a disease — it’s a description of neurological signs and symptoms that are caused by an underlying condition. The causes of ataxia are varied and include:

    • Head trauma resulting in brain injury, specifically in the cerebellum
    • Cerebral palsy (or damage to a child’s brain during development)
    • Brain abnormalities, infections, or growths
    • Stroke
    • Toxic reactions, including to medications such as barbiturates, sedatives, antiepileptic drugs, and some forms of chemotherapy. Other drugs that can cause ataxia include phenytoin, carbamazepine, lithium, amiodarone, and some antibiotics. This category can also include alcohol and drug intoxication as well as poisoning from heavy metals or solvents.
    • Autoimmune diseases (including multiple sclerosis)
    • Infections
    • Vitamin deficiencies
    • Thyroid problems (hypothyroidism)
    • Inherited disorders

    How Is Ataxia Diagnosed?

    To diagnose ataxia, a doctor must conduct an extensive clinical history, including a thorough review of the patient’s family history. In addition, the examining physician must perform a complete neurological examination and may order diagnostic tests that may include the following:

    • A Brain Scan utilizing one of  a variety of imaging modalities, such as a CT scan or an MRI
    • Complete Labs, including blood, urine, and spinal fluid samples
    • Nerve Conduction Studies and Electromyography (ENG/EMG)
    • An Eye Examination
    • Blood tests for genetic or hereditary ataxias

    What Is Hereditary Ataxia?

    Hereditary Ataxia (HA) does not refer to a single disease, but rather includes a wide range of inherited diseases where the main symptom of the disease is ataxia. HA is caused by changes in certain genes and in most cases affects more than one family member. However, sometimes there is no family history of HA even in cases where a patient is diagnosed with HA.

    Four Types of Hereditary Ataxia

    Autosomal Dominant Inheritance

    In these cases, a patient only needs to inherit one abnormal gene from either parent. Likewise, a person with the abnormal gene has a 50% chance of passing that gene to a child. This category of HA’s includes spinocerebellar ataxias (SCA), which usually starts in early to late adulthood.

    Autosomal Recessive Inheritance

    These complex, disabling diseases usually start before age 20 with the most common type being Friedreich’s Ataxia. There is a genetic blood test that can confirm a diagnosis.

    X-Lined Ataxia

    This type of HA includes Fragile X-associated Tremor-Ataxia syndrome (FXTAS).

    Mitochondrial Ataxia

    This category of HA includes myoclonic epilepsy ragged red fire syndrome (MERRF), neuropathy, ataxia, and retinitis pigmentosa (NARP), as well as Kearns-Sayre syndrome.

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