Ataxia

Anatomy or system affected: Brain, musculoskeletal system, nervous system

Definition: A lack of coordination while performing voluntary movements that may appear as clumsiness, inaccuracy, or instability.

Causes: Head trauma, neurodegenerative disease, stroke, brain hemorrhage, brain tumor, congenital abnormality, postviral infections, exposure to certain drugs or toxins, cardiac or respiratory arrest, hypothyroidism, some vitamin deficiencies, certain cancers, multiple sclerosis, syphilis

Symptoms: Lack of coordination while performing voluntary movements (clumsiness, inaccuracy, instability)

Duration: Acute, chronic, or progressive, depending on cause

Treatments: Medications or surgery, depending on underlying condition; physical and occupational therapy

Causes and Symptoms

Ataxia most often results from disorders of the cerebellum (a large structure at the lower back of the brain, just above where the spinal cord enters the skull at the bottom) or its connections. Disorders resembling ataxia can also be seen following parietal or frontal lobe lesions of the brain. Ataxia is a symptom, not a diagnosis. It may affect any part of the body.

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Some medical conditions can cause ataxia to appear suddenly, such as head trauma, stroke, brain hemorrhage, brain tumor, congenital abnormality, postviral infections, exposure to certain drugs or toxins (for example, alcohol or seizure medications), or cardiac or respiratory arrest. Other conditions may cause ataxia to appear gradually, such as hypothyroidism, some vitamin deficiencies (vitamins E or B1), exposure to certain drugs or toxins (heavy metals, chronic alcohol use, some cancer drugs), certain kinds of cancer (ovarian and lung cancer), congenital abnormality, heredity disorders such as ataxia-telangiectasia and Friedreich ataxia, neurodegenerative disorders and cerebellar atrophies, multiple sclerosis, celiac disease, and human immunodeficiency virus (HIV) infection.

Treatment and Therapy

A careful neurologic and general physical examination, including blood tests and brain magnetic resonance imaging (MRI), can determine whether other parts of the nervous system are impaired and whether a medical illness may be causing ataxia. No medicine specifically treats the symptom of ataxia. If ataxia is the result of a stroke, a low vitamin level, or exposure to a toxic drug or chemical, then treatment involves minimizing the effects of the current stroke and preventing further ones, instituting vitamin therapy, or avoiding the toxic drug or chemical, respectively. In some cases, the metabolic disorders that cause ataxia may be treated with controlled diet and medication. If ataxia is the result of a tumor, then surgery may be necessary. Many people with hereditary or idiopathic forms of ataxia have additional symptoms. Genetic testing may be required to detect hereditary spinocerebellar ataxia-6, Friedreich ataxia, or fragile X tremor/ataxia syndrome (FXTAS). Medications or other therapies might be appropriate for some of these symptoms, such as tremor, stiffness, spasticity, depression, and sleep disorders.

The mainstay of treatment for ataxia is the provision of physical and occupational therapy directed at maintaining function for as long as possible. Gait training and assistive devices such as canes, crutches, or a walker are useful to prevent falls and enhance mobility. Other adapted utensils and tools may be helpful to assist with writing, feeding, and self-care if hand or arm coordination is impaired, as may be speech therapy and communication devices for those with impaired speech.

Extremity ataxia that interferes with activities of daily living, such as feeding or dressing, may be treated with proximal splinting. Distal weights may dampen intention tremor. If the ataxia is exacerbated by weakness, strengthening exercises can be beneficial. Specific coordinated exercises are often helpful in treating static causes of ataxia (stroke or head injury) but not as useful in treating progressive disorders such as hereditary ataxias. Ataxia of eye movements rarely requires treatment, but it may be disabling if the patient has difficulty reading.

Bibliography

"Ataxia." Mayo Clinic. 9 Apr. 2022, www.mayoclinic.org/diseases-conditions/ataxia/symptoms-causes/syc-20355652. Accessed 31 July 2023.

Cifu, David X., et al., eds. Braddom’s Physical Medicine & Rehabilitation. 5th ed. Philadelphia: Elsevier, 2016. Print.

Ellison, David, et al. Neuropathology: A Reference Text of CNS Pathology. 3rd ed. Edinburgh: Mosby, 2013. Print.

Gillen, Glen. “Improving Activities of Daily Living Performance in an Adult with Ataxia.” American Journal of Occupational Therapy 54.1 (2000): 89–96. Web. 28 Apr. 2016.

Gillen, Glen. “Improving Mobility and Community Access in an Adult with Ataxia.” American Journal of Occupational Therapy 56.4 (2002): 462–66. Web. 28 Apr. 2016.

Hong, Sung Hoi, ed. Ataxia: Causes, Symptoms, and Treatment. New York: Nova, 2012. Print.

Larner, Andrew J., et al. A–Z of Neurological Practice: A Guide to Clinical Neurology. 2nd ed. Dordrecht: Springer, 2011. Print.

Manto, Mario Ubaldo. Cerebellar Disorders: A Practical Approach to Diagnosis and Management. New York: Cambridge UP, 2010. Print.