Ataxia - Here is all you need to know about it

Categories: Neurology

All you need to know about Ataxia

Table of Content:

What is Ataxia?
Causes
Symptoms
Risk Factors & Complications
How is it Diagnosed?
Treatment

What is Ataxia?

Ataxia means loss of control over bodily movements and is a sign of an underlying condition. Ataxia describes a lack of muscle coordination or control while performing voluntary movements. This disorder can affect eating, eye movements, and speech. Various conditions can cause ataxia, including alcohol abuse, certain medications, stroke, cerebral palsy, brain degeneration, and brain tumours and there are several Ataxia treatment options available as well.

Risk factors and causes of ataxia

Many may wonder about Ataxia’s meaning and its causes, and risk factors. Ataxia is the result of damage, degeneration, or loss of nerve cells in the part of the brain that controls muscle coordination, the cerebellum. The cerebellum is situated in the base of the brain and controls balance, eye movements, swallowing, and speech. Damage to the nerves or spinal cord that connect the cerebellum to the muscles can also lead to ataxia. Here are some of the causes of ataxia:

A blow to the head or spinal cord can lead to acute cerebellar ataxia, which can occur suddenly.
Cerebral palsy. This is a general term for a group of disorders that result from damage to the brain in a child’s early years. Brain damage that occurs before, during, or shortly after childbirth is also referred to as cerebral palsy.
Sarcoidosis, multiple sclerosis, and other autoimmune conditions can lead to ataxia.
A stroke can lead to an interruption in the blood supply to a part of the brain leading to the death of the neurons, and in some cases, ataxia.
Ataxia is a rare complication of infections such as HIV and Lyme disease.
Vitamin E, Vitamin B12, or thiamine deficiency, which can also be seen in alcohol abuse, can lead to ataxia.
Adverse effects of certain medications. Drugs like barbiturates, benzodiazepine, chemotherapy, and antiepileptics, can cause ataxia.
Other rare causes for ataxia include thyroid disorders and complications of a COVID-19 infection.

Hereditary causes for ataxia

Inheritance of cerebellar ataxias can be of two types, autosomal dominant and autosomal recessive. Autosomal dominant disorders can be acquired if only one allele of the mutated gene is passed on from a parent to the offspring, whereas autosomal recessive conditions require both alleles of the disease-associated gene for it to manifest.

Autosomal dominant cerebellar ataxias include Spinocerebellar Ataxia and Episodic Ataxia. Spinocerebellar ataxia can be caused by 40 different types of genes and all have common symptoms of cerebellar ataxia and degeneration.

Autosomal recessive cerebellar ataxias include Friedreich’s ataxia, ataxia telangiectasia, congenital cerebellar ataxia, and Wilson’s disease. Ataxia-telangiectasia is a rare childhood disorder that is also associated with immune system dysfunction, making the child prone to malignancies such as lymphomas and leukaemias.

Symptoms of ataxia

Though ataxias can develop suddenly or slowly over a period of time, it is important to identify the first signs:

Unsteady gait often called an ataxic gait or frequent stumbling
Poor coordination
Difficulty in performing fine motor tasks such as buttoning a shirt or writing.
Involuntary eye movements, also known as nystagmus
Slurring of speech
Difficulty in swallowing
Truncal ataxia. This instability of the trunk is seen most often during sitting and is also characterised by a wide-based drunken gait.

If you feel that you are losing balance easily, have difficulty while speaking, or have lost muscle coordination in your arm, hand, or leg, it is important to visit your physician for further evaluation.

Types of ataxia

Ataxia can be divided into different types based on the region affected:

Cerebellar ataxia. This is the most common type of ataxia and symptoms such as an unstable gait, change in voice, dizziness, and fatigue are common.
Sensory ataxia. This is the result of damage to the nerves in the spinal cord or beyond it. Symptoms include difficulty in walking in dim light, tingling sensation in the leg, inability to detect vibrations, and walking with a heavy step.
Vestibular ataxia. This affects the vestibular system which is composed of the inner ear canal that contains fluid. Symptoms include giddiness, nausea, and vomiting.

Complications of ataxia

Different types of ataxias may lead to different complications. Difficulty in breathing, choking may eventually even lead to death in some cases. Inability to move can lead to life-threatening complications such as pressure sores, injury, and blood clots. Other complications associated with ataxia are:

Involuntary tremors are seen in some cases which can be distracting and bothersome.
Loss of balance can make walking or moving difficult.

How is it Diagnosed?

The treating physician begins by taking a full medical and family history, after which a complete physical and neurological examination is done. Here is a list of other investigations that may be done:

Laboratory tests are done on the blood as well as urine
Genetic testing to pick up hereditary causes for ataxia.
Imaging studies. CT or Magnetic resonance imaging (MRI) is done to identify injury, strokes, tumours, or degenerative conditions that may have led to ataxia.
A lumbar puncture or a spinal tap can be done to analyse the spinal fluid.

Treatment of ataxia

There is no specific treatment for ataxia. Identifying the underlying cause is most important, which can help in the resolution of symptoms. In most cases, treatment is only given to prevent complications, manage symptoms, and advise adaptive strategies to make life easier.

Adaptive devices. Ataxia that cannot be cured due to causes such as a stroke or multiple sclerosis can be managed by the use of adaptive devices such as walkers to aid in movement, communication aids for speaking, and modified utensils and cutlery for eating.

Other therapies that are offered include: