Ataxia in children

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Have you ever seen a person walking in abnormal gait? Did you notice that he is unsteady as if shacking? Was he looked unbalanced? This is ataxia.

Ataxia in children

What is ataxia?

It is a benign neurological condition characterized by incoordination of the muscular activity,

i.e.:

The patient’s muscles are not contracting in an organized manner to perform a certain movement.

The patient here has lost his control over voluntary movements.

If you are interested in the origin of words, let me tell you that ataxia comes from the Greek word ataktos, which means “lack of order.”

Symptoms and signs:

The patient is lacking balance.

If manifested early life, it will show itself as difficulty in sitting

The patient has an abnormal gait, which is called “Staggering gait”

Also, he looks unsteady with loss of coordination and equilibrium, with stumbling.

The patient has abnormal and uncoordinated eye movements i.e.: nystagmus secondary to the loss of coordination.

In addition, the patient has difficulty in speech and swallowing.

In general:

The patient is awake, conscious and oriented.

He has the signs of abnormal cerebellar function e.g.: Negative Rom berg’s sign

His gait is wide, in a trial to keep balance.

The joint sense and vibration sense are normal.

Causes of ataxia in children

In general, there is a damage of the cerebellum. The cerebellum is the part of the CNS responsible for coordination.

This damage is secondary to several causes. For example:

Congenital cause

Hereditary ataxia: i.e.: inherited defective gene

Neurological insult:

The most common cause is acute cerebellar ataxia:

It happens secondary to autoimmune reaction after an infection by varicella, mycoplasma and parvovirus B19 as well.

Acute cerebellar ataxia

It affects children 2-7 years old with acute onset of ataxic gait, nystagmus and dysarthria )that’s to say difficulty in speech).

It is of symmetrical distribution i.e.: both sides of the body are equally affected

Other causes

Cerebral palsy

Post infectious: It is rare after chickenpox

Post traumatic: trauma to the brain can lead to acute cerebellar ataxia

Toxicity: for example: alcohol

Metabolic: deficiency of thiamine, B12 and vitamin E as well.

Systemic autoimmune disease: such as multiple sclerosis and celiac disease

Vascular causes: i.e.: stroke due to oxygen deprivation

Neoplastic cause:

Secondary to brain tumor. Usually is of gradual onset but sometimes show acute presentation.

Diagnostic aids in cases of ataxia

They are done to exclude serious conditions which can be treated.

Computed tomography and Magnetic resonance imaging:

They help to diagnose and localize neurological causes, for instance, brain tumor or vascular problem such as intracranial hemorrhage

Blood tests:

For metabolic conditions such as blood glucose level

Cerebro-spinal fluid analysis:

Is used to exclude central infection. In addition to cytology in cases of brain tumors.

Genetic studies:

Is used to identify some gene defects and hereditary causes of ataxia. However, they are not able to identify all the hereditary causes

What is pseudo-ataxia in children?

It is unsteadiness because of muscle weakness.

Dangerously ill and frightened children look to have false ataxia.

How to differentiate between pseudo ataxia and true ataxia?

In pseudo(false) ataxia: The child has generalized weakness, absent reflexes as well as, signs of upper and lower motor neuron lesions. On the contrary of true ataxia.

Management of ataxia in children

Definitely, it depends on the cause as well as severity of ataxia.

Acute cerebellar ataxia in a child indicates immediate hospitalization and treatment under supervision of pediatrician.

Adaptive measures:

Measures to facilitate life and to conduct some independent activities, for instance:

Walkers and sticks will help in walking independently.

Modifying the feeding tools.

Speech therapy: to facilitate speech as well as feeding.

Role of physiotherapy in the management of pediatric ataxia:

Physiotherapy aims at enhancing the coordination of movements and improving the gait.

Fitness training: e.g.: yoga

Hydrotherapy: Water therapy and swimming have good effect on health as well as quality of life

Finally, Balance training

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