Schizencephaly
is a rare developmental disorder
characterized by abnormal slits, or
clefts, in the cerebral hemispheres.
Schizencephaly is a form of
porencephaly. Individuals with
clefts in both hemispheres, or
bilateral clefts, are often
developmentally delayed and have
delayed speech and language skills
and corticospinal dysfunction.
Individuals with smaller, unilateral
clefts (clefts in one hemisphere)
may be weak on one side of the body
and may have average or near-average
intelligence. Patients with
schizencephaly may also have varying
degrees of microcephaly, mental
retardation, hemiparesis (weakness
or paralysis affecting one side of
the body), or quadriparesis
(weakness or paralysis affecting all
four extremities), and may have
reduced muscle tone (hypotonia).
Most patients have seizures and some
may have hydrocephalus.
In schizencephaly, the neurons
border the edge of the cleft
implying a very early disruption in
development. There is now a genetic
origin for one type of
schizencephaly. Causes of this type
may include environmental exposures
during pregnancy such as medication
taken by the mother, exposure to
toxins, or a vascular insult. Often
there are associated heterotopias
(isolated islands of neurons) which
indicate a failure of migration of
the neurons to their final position
in the brain.
Treatment for individuals with
schizencephaly generally consists of
physical therapy, treatment for
seizures, and, in cases that are
complicated by hydrocephalus, a
shunt.
The prognosis for individuals
with schizencephaly varies depending
on the size of the clefts and the
degree of neurological deficit.