In this lecture we will discuss
Ataxia in Children.
Ataxia is the decreased coordination of
voluntary movements not just walking.
But really, any movement in children.
Causes are varied and dependent a lot
on how old the child is
and the associated symptoms.
One common cause of Ataxia in children
is injury to the brain.
And in children in particular,
common injuries include birth
which can lead to cerebral palsy
and hypoxic injury.
So, all of these are a varieties of injuries
that can affect the brain
and result in Ataxia in children.
One of the conditions that can cause
Ataxia in children is Ataxia Telangiectasia.
Ataxia Telangiectasia is an autosomal
The gene is carried in 1.5 percent of U.S.
caucasians and is on 11q22.3
It is a defect in the gene responsible
for DNA repair.
It affects both central and peripheral
Basically, childen with Ataxia Telangiectasia
are generally born normal
and continue to develop normally
until they are about the
age when the child walks.
the child then has a progressive worsening
of ambulation and running.
The child often have gross and fine motor
skills that deteriorate over time
and also child will eventually develop
difficullty with eye movements and dysarthria
The eye movement difficult has to do with
the movement of the head and the eyes.
So, often when they turn their head,
they then have trouble keeping track
of objects and their environment.
These children will also unfortunately
have a decline in cognition.
And when we examine them
we could see Telangiectasia's
on their skin face and neck
About 70% of these children will have an
associated immune deficiency and as a result,
they often have progressive pulmonary
And that is a major cause of morbidity and
mortality in this condition.
So, here are some Telangiectasias in
a child. You can see them right in the eye.
They are these squiggly red lines
where the capillary beds have
Another condition in children is
Friedreich's Ataxia is autosomal recessive
and it is a trinucleotide repeat disorder.
And that each generation have subsequently
more and more
repeats and more and more severe
alteration of that gene.
It usually presents in adolescence,
And it is a progressive Ataxia on all
These patients will have motor weakness
and eventually Cerebellar dysarthria,
dysphagia and they will suffer
hearing and vision loss.
Patients often go Kyphoscoliosis
and may have hand and foot atrophy.
Usually, cognition in this case
Other degenerative problems that can
in children include genetic causes
such as Wilson's disease, which causes
on an acrual of copper
and particularly affect the basal ganglia.
Also, children can also get
Metabolic disease can
specifically mitochondrial disorders
tend to affect the nerves most
and so those children may have consequences.
Lastly, obviously, brain malformations can
Example of those include Chiari
and Cerebellar dysgenesis.
Let's shift to look at what could cause
Acute Ataxia in a child.
A child is walking along fine
looks great and then suddenly,
has severe Ataxia.
There are many different causes that can
cause this but most commonly,
it is a drug ingestion.
Children especially young toddlers
are always getting into things and
may consume either and illicit
or a prescribed drug in the home.
Examples of prescribed drugs
that children can get into include Benadryl
Dextromethorphan and that one
is particularly common.
Cough syrup ingestion often causes
Ataxia and the abnormal movement of the eyes.
One drug of abuse that doesn't show
up on a screen that is around is Ketamine.
and Ketamine causes a profound
Also, toxins can cause Ataxia.
And usually the one that I would implicate
is Lead poisoning.
We see a lot of lead poisoning in
the United States.
patients can have infectious Ataxia.
Diseases such as Cerebellitis. It is a viral
condition that attacks the cerebellum
and those children will present with
A severe Otitis media can present
with Ataxia as well.
Those usually are very severe and that is
a very unusual presentation of Otitis media.
Also, inside the ear, the labrynth itself can
also be infected we call this Labrynthitis.
And in those cases children can be
In all of these cases,
what we do is we simply wait for the child
to get better.
Very rarely, children can get viral
encephalitis that can result in Ataxia
but also presents with other symptoms
such as inability to speak correctly.
Or altered mental status.
Varicella and several other viruses
are implicated in these causes of Ataxia.
So that is my brief summary of causes
of Ataxia in children.
Thanks very much.