Ataxia - Coordination disorder

Ataxia is a balance and coordination disorder, a movement disorder.

Balance and coordination are affected. Someone with ataxia has an unsteady gait and moves arms, legs, and trunk jerkily and uncontrollably. While walking, they place their feet far apart to avoid falling, taking short, irregular strides. This gives them a clumsy appearance.
Flowing movement is no longer present.

The arms and legs don't function as intended.
The hands may misgrip and tremble. Speech can also be affected. The patient may have difficulty pronouncing words clearly (dysarthria).

These symptoms are similar to those of drunkenness.
In more severe forms of ataxia, the person may choke (dysphagia). The eyes may also jerk.
Several brain areas that, if damaged, can lead to ataxia. The cerebellum, the basal ganglia, and the putamen in particular are particularly important.

Cerebellar Ataxia

There are as many as fifty different forms of ataxia. However, coordination disorders usually stem from a disorder of the cerebellum and its surrounding connections.

In that case it is called cerebellar ataxia.

Ataxia may be caused by neurological disorders in the cerebellum, such as inflammation or a brain tumor, a hemorrhage or stroke, or a degenerative condition like multiple sclerosis or other degenerative conditions (premature wear and tear of nerve cells), which gradually cause a person to develop more ataxia symptoms.

If the lesion affects one half of the cerebellum (in one cerebellar hemisphere), it can cause hemilateral ataxia, ipsilateral (on the same side as the lesion).

Well-known examples of ataxia are Friedreich's ataxia, Multiple System Atrophy / MSA, Autosomal Dominant Cerebellar Atrophy / ADCA, Spinocerebellar Ataxia / SCA, SCA2 and Parkinson's disease.

People with cerebellar ataxia may have difficulty with:

• the direction of movement
  
• the speed of muscle movement
  
• regulating the force of movement
  
• the precision of movement
  
• the correct rhythm of movement
  
• double vision or seeing moving images
  
• sitting or standing upright (trunk ataxia with damage to the mid-cerebellum, in the vermis)
  walking straight ahead. They have a wide gait (with damage to the mid-cerebellum, in the vermis).

Sometimes ataxia is caused by medication side effects or a hereditary condition, such as ataxia telangiectasia, or by alcohol or drug poisoning or other toxic substances, by a metabolic disorder, or by a gluten sensitivity (gluten ataxia/neuroceliac disease).

Gluten is a protein found in certain types of grain, including wheat, barley, and rye. Some people with gluten sensitivity may experience no symptoms other than the balance disorder/ataxia.

Temporary symptoms of ataxia occur after consuming several glasses of alcohol.

Postviral ataxia (paraviral ataxia) is a movement disorder characterized by jerky, uncoordinated movements resulting from a previous viral infection (chickenpox, Ebstein-Barr virus/mononucleosis, mycoplasma virus, and others).

Postviral ataxia can also develop after vaccination. Symptoms may include uncoordinated movements, slurred speech, double vision or jerky eye movements, and shaking arms, legs, or head tremors.

Other forms of ataxia

• Sensory ataxia, caused by a disorder of the sense of position or proprioception. Thanks to this sense, the body "knows" the position and position of the body and limbs. Sensory ataxia is usually caused by a polyneuropathy affecting both legs.
• Vestibular ataxia, caused by a disorder of the balance organ. Usually accompanied by vertigo and nausea or vomiting.

Polyneuropathy and Foot Problems

In case a person has difficulty walking or other motor or postural problems, this may result in foot pain.
Regardless of the cause, many people with ataxia suffer from polyneuropathy. This is a painful condition affecting the long nerve endings in the lower legs and hands. Increased muscle tension in the legs (spasticity) can cause discomfort. All of this can result in pain and loss of strength.

PHARC

PHARC stands for Polyneuropathy, Hearing Loss, Ataxia, Retinitis Pigmentosa and Cataract.

It is a rare autosomal recessive inherited disorder in which ataxia is accompanied by polyneuropathy, hearing loss, and eye symptoms.

Eye symptoms in particular include a grainy retina (retinitis pigmentosa), a cloudy lens (cataract), and a thinning optic nerve (optic atrophy).

The nerves in the body increasingly struggle to transmit signals from the spinal cord to the rest of the body and vice versa.
This condition typically appears between the ages of six and seventeen, sometimes earlier, and sometimes in (young) adulthood. It often begins with weakness in the feet. Children are therefore more likely to fall. Gradually, the weakness spreads to the hands, forearms, and upper arms.

PHARC may be accompanied by numbness where the nerve fibers are affected, pain, cold and/or swollen hands and feet, loss of balance, loss of strength that makes any movement strenuous and can cause significant fatigue, muscle wasting (atrophy), an inability to raise the arms above the head, blurred vision, hearing loss, and problems with swallowing (dysphagia), chewing, and speaking. A curvature of the spine (scoliosis) can also occur.

Sleep disorders with respiratory arrest (sleep apnea) can occur, causing a person to wake up repeatedly during the night and wake up with headaches and fatigue. This should always be evaluated by a doctor.

Eye problems

Eye and vision problems may occur with ataxia. Read here and here about possible treatment.

Depression

If depression-like symptoms are present alongside the ataxia, CCAS, cognitive-cerebellar affective syndrome, which may occur with cerebellar disorders, may also be considered. Read more on the CCAS page.

Read about Bram Bakker, a Dutch psychiatrist who, after months of searching, recognized his complaints as being the Cognitive Cerebellar Affective Syndrome. He had recognized these complaints on our Dutch website. Read his story (in Dutch) : https://joop.bnnvara.nl/opinies/levenslust.

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