Spinocerebellar Ataxia Life Expectancy

Spinocerebellar ataxia refers to involuntary discoordination of the body's muscles. This condition can affect speech, movement, and ambulation. In this article, we will discuss spinocerebellar ataxia life expectancy, its symptoms, and the different forms of this genetic disease.

Spinocerebellar ataxia is a genetically inherited ailment that's characterised by abnormalities in the brain. People with ataxia carry various gene mutations and present a 50 per cent chance of passing this disorder to their child. They can also experience spinal cord degeneration later in their adult life.

The various types of spinocerebellar ataxia

There are 3 types of ataxia including acquired, idiopathic cerebellar, and ataxia with vitamin E deficiency. All these types of SCA have similar symptoms. The acquired ataxia type tends to affect people at any age and usually the symptoms develop very quickly, in a matter of hours or days. The idiopathic late-onset cerebellar type usually starts at around the age of 50 and gets worse over time. If the body is unable to use the vitamin E from diet, it's often referred to as ataxia with vitamin E deficiency. The symptoms can often be controlled by taking vitamin E supplements.

Symptoms of spinocerebellar ataxia

The symptoms and signs of SCA can vary from one person to another, however, the most common ones include: unsteady walk due to the patient's inability to sustain balance and coordination. This is caused mainly by uncoordinated muscle contraction that involves different muscles in the body. As a consequence, the patient starts losing his or her upper and lower muscle function due to lack of coordination. They will no longer be able to brush their hair, walk steadily, or use their hands for carrying out everyday tasks.

Speech abnormality is another common symptom that most SCA patients experience. This is due to the fact that their uncoordinated tongue muscle contraction causes abnormal speech and sounds. What's more, the uncoordinated eye muscle contraction also causes nystagmus, which means the patient experiences involuntary eye movements.

Another sign of SCA includes difficulty swallowing food. It becomes an arduous task for the patient to swallow food into esophagus so its content remains in the pharynx.

The symptoms of ataxia usually first develop during the teenage years. Certain triggers such as caffeine, alcohol, stress, or sudden movement can cause episodes that last from a few minutes to several hours.

Diagnosing Ataxia Spinocerebellar

Most disorders that result in SCA cause parts of the patient's brain cells to degenerate. To come up with the correct diagnosis, the person's age and the underlying cause of the nervous system degeneration must be assessed. Most people inherit ataxia in an autosomal recessive way (both parents pass a copy of their faulty gene to the child) or in an autosomal dominant way (one parent passes a faulty gene to the child).

Treatment for SCA

Although there is still no cure for hereditary ataxias, if the person is experiencing ataxia caused by another condition, the doctor treats the underlying condition first. For example, some ataxias are caused by metabolic disorder, which can be treated with medications and controlled diet. Vitamin E deficiency is treatable with supplements and there are a number of medications that can assist with swallowing. For people who have difficulty carrying out day-to-day activities, physical therapy can help strengthen their muscles.

Spinocerebellar ataxia life expectancy

As the symptoms of ataxia usually get worse over time, people with this condition have a shorter life expectancy. Most patients live into their 50s or beyond, depending on the type of SCA they are diagnosed with. Those who have a slowly progressive form of ataxia don't have a shorter life expectancy; however, they can face a combination of other illnesses such as dementia, involuntary tremor, rigidity, and neurological abnormalities. These patients often end up confined to wheelchair within 5 years, with chronic incontinence and respiratory failure that leads to premature death. The other sporadic forms of SCA that are less severe include hypothyroidism, chronic ethanol abuse, and Creutzfeldt-Jakob disease. All in all, the life expectancy of SCA sufferers depends on the form of ataxia they're diagnosed with.