Ataxia in children
By 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.
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
