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in this lecture we will discuss Axia in
children at taxia is the decreased
coordination of voluntary movements not
just walking but really any movement in
children causes are varied and depending
a lot on how old the child is and the
associated
symptoms one common cause of axian
children is injury to the brain and in
children in particular common injuries
include birth asphixiation which can
lead to cerebral palsy stroke trauma and
hypoxic injury so all of these are
varieties of injuries that can affect
the brain and result in Axia in children
one of the conditions that can cause
Axia in children is Axia
tanasia Axia tanasia is an auto
autosomal recessive disease the gene is
carried in 1.5% of us Caucasians and
it's on 11 Q
22.3 it's a defect in the gene
responsible for DNA repair it affects
both Central and peripheral nervous
systems basically children with Axia
Tangi Tasia are generally born normal
and continue to develop normally until
about the age when the child walks the
child often then has a progressive
worsening of ambulation and
running the child often has gross and
fine motor skills that deteriorate over
time and also the child will eventually
develop difficulty with eye movements
and
dysarthria the eye movement difficulty
has to do with coordinating the movement
of the head and the eyes so often when
they turn their head they then have t
trouble keeping track of objects in
their
environment these children will also
unfortunately have a decline in
cognition and when we examine them we
can see tangc tasas on their skin face
neck about 70% of these children will
have an Associated immune deficiency and
as a result they often have Progressive
pulmonary disease and that is a major
cause of morbidity and mortality in this
condition so here are some Tanga tasas
in a child you can see them right in the
eye there are these squiggly red lines
where the capillary beds have uh
developed another condition in children
is friedrich's
Axia fried's Axia is autosomal recessive
and is a trinucleotide repeat disorder
in that each generation subsequently has
more and more repeats and more and more
severe uh alteration of that Gene it
usually presents an adolescence and is a
progressive Axia of all four limbs these
patients will have motor weakness and
eventually cerebellar dysarthria
dysphasia and they will suffer hearing
and vision loss L patients often get
kyphoscoliosis and may have hand and
foot
atrophy usually cognition in this case
is
preserved other degenerative problems
that can cause axian children include
genetic causes such as Wilson's disease
which causes an acrel of copper and it
particularly affects the basil ganglia
also children can get spino serabella
axas metabolic disease can cause at
taxia specifically mitochondrial
disorders tend to affect the nerves most
and so those children may have
consequences lastly obviously brain
malformations can cause a taxia example
of those include kiari malformations
dandy walker cysts and cerebellar dis
Genesis let's shift to look at what
could cause acute Axia in a child a
child's walking along fine looks great
and then suddenly
has severe
Axia there are many different causes
that can cause this but most commonly
it's a drug ingestion children
especially young toddlers are always
getting into things and may consume
either an elicit or a prescribed drug in
the home examples of prescribed drugs
that children could get into include
badril Dexter methorphan and that one's
particularly common cough syrup
ingestion often causes a taxia and
abnormal movements 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
Axia also toxins can cause Axia and
usually the one I would implicate is
lead poisoning and we see a lot of lead
poisoning in the United
States also patients can have infectious
Ataxia diseases such as cobitis it's a
viral condition that attacks the
cerebellum and those children will
present with a taxia a severe otitis
media can present with at taxi as well
those usually are very severe and that's
a very unusual presentation of otitis
media also inside The Ear The Labyrinth
itself can become infected we call this
labyrinthitis and in those cases
children can be at taxic in all of these
cases what we do is we simply wait for
the child to get better
very rarely children can get a viral
incilius that can result in a taxia but
also present with other symptoms such as
inability to speak correctly or altered
mental status vericella and several
other viruses are implicated in these
causes of
Axia so that's my brief summary of
causes of Axia in children thanks very
much
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