Schizencephaly is a birth defect
that can be characterized by abnormal clefts, slits or grey matter which
extends across the cerebral ventricles to the pia mater, or the innermost
delicate layer surrounding the brain and spinal cord. The word itself phonetically
springs from the Greeks and roughly translates as ‘to split brain’.
Like many diseases there are two main
types, Type I has grey matter tissue with ventricular or cortical lips which
form at the end of a hemispheric cleft. Type II exemplifies a cerebrospinal
fluid saturated cleft which usually extends over most, if not all of the cerebral
hemisphere. The inflicted who have these clefts on both hemispheres often
suffer from speech and developmental delays, seizures, and paralysis such as
hemiparesis or quadriparesis as well as varying degrees of mental retardation,
microcephaly, and hypotonia or reduced muscle tone.
Usually, with schizencephaly, the
neurons line the edges of the clefts, which implicates that somewhere in early
fetal development, usually embryogenesis, a disruption
during the formation of grey matter occurred. Scientists have not been
able to pinpoint a specific cause for this, it could be anything from utero
strokes, exposure to toxic chemicals or infection; however scientists are
leaning toward the hypothesis that it is caused by failed neuron migration in
the second trimester. This theory, however is new, it used to be believed that
this disorder had a genetic association with the EMX2 gene.
Treatment for individuals
suffering from this disease consists of KG-ZNS with Vojta Methode, which is a type of physical therapy specific
to many genetic disorders. Sufferers also usually take part in occupational
therapy, seizure treatment and even in some cases a shunt, or a small hole
which moves fluid to other parts of the body. With schizencephaly, certebral shunts
are used, the valve for this shunt is usually located behind the ear. Thus a person suffering this ailment can be identified as such in most cases. -T.H.
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