Cerebellar Ataxia – Inherited or Acquired: Causes, Symptoms and Treatments

Definition:

It is characterized mainly by a lack of muscular coordination (ataxia) or awkwardness of movement resulting from atrophy or disease of the cerebellum. This brain region organizes sensory information related to balance and locomotion.

Causes

Cerebellar ataxia can be inherited or acquired. Hereditary forms may be present at birth or manifest later in life and may be autosomal recessive (when two genes are defective) or autosomal dominant (when a defective gene exists).

Ataxia Friedrich, caused by mutations in a gene known as FXN, is one of the cerebellar ataxia’s most common hereditary forms.

Acquired cerebellar ataxia can result from damage to it or damage to the pathways to and from the cerebellum. It is usually caused by a stroke, certain diseases, or tumors.

Manifestations of cerebellar ataxia

If there is damage to only one side of the cerebellum, symptoms resulting from ataxia manifest on the same side of the body (for example, right cerebellar damage causes ataxia on the right side). Damage to the cerebellum can cause a variety of problems, including:

  • Abnormal movements of the eyes (nystagmus).
  • Involuntary trembling
  • Dysarthria (damage to language).
  • Difficulty in swallowing.

The most common impairment of cerebellar damage is gait ataxia, characterized by a lack of coordination when walking, often described as a “drunken gait,” with distinctive features, including variable placement. The feet have irregular trajectories, a widened position, a turning of movement when walking, and the poor general coordination of the legs.

 

Impacts on daily life

The impact of cerebellar ataxia on life expectancy varies depending on the type of condition, age of onset, severity, and other factors.

Many affected individuals have an average life expectancy and learn to cope with their condition; some even enjoy everyday life. For others, however, it can impact work, family life, and recreation. Many affected people find that their jobs become difficult to perform.

Ataxic hand movements make illegible handwriting and can cause difficulties typing on the computer, while dysarthria can make talking on the phone a problematic task. Balance problems and gait ataxia can affect the performance of people who perform physical work.

Often, people find that they must modify their jobs or become trained in another area to continue working.

At home, modifications such as installing handrails and grab bars can help people better manage conditions such as walking. Recreational activities can also be modified to increase opportunities for the participation of people with this disease.

Treatment for cerebellar ataxia

There are few treatments for cerebellar ataxia, and the drugs that can slow the progression of degenerative diseases of the cerebellum are insufficient.

Therefore, the primary treatment available to patients is rehabilitation training, which may include physical and occupational speech. These therapies can improve physical strength and mobility, the performance of day-to-day tasks, communication, and swallowing.