NINDS Schizencephaly Information Page: NINDS
Article title: NINDS Schizencephaly Information Page: NINDS
Main condition: Schizencephaly
Conditions: Schizencephaly
What is Schizencephaly?
Schizencephaly is an extremely rare developmental disorder characterized by abnormal slits, or clefts, in the brain's cerebral hemispheres. Schizencephaly is a form of porencephaly in which there is a cyst or cavity in the cerebral hemispheres. Individuals with clefts in both hemispheres (bilateral clefts) are commonly developmentally delayed and have delayed speech and language skills and corticospinal dysfunction. Individuals with smaller, unilateral clefts (clefts in only one hemisphere) are often paralyzed on one side of the body and may have normal intelligence. Patients with schizencephaly may also have varying degrees of microcephaly (abnormally small head), mental retardation, hemiparesis or quadriparesis (partial or complete paralysis), and reduced muscle tone (hypotonicity). Most patients have seizures. Some may have hydrocephalus.
Is there any
treatment?
Treatment for individuals with schizencephaly generally
consists of physical therapy, treatment for seizures, and, in cases that
are complicated by hydrocephalus, a shunt (a surgically implanted tube
that diverts fluid from one pathway to another).
What is the prognosis?
The
prognosis for individuals with schizencephaly varies depending on the size
of the clefts and the degree of neurological deficit.
What research is being
done?
The NINDS conducts and supports a wide range of studies that
explore the complex mechanisms of normal brain development. The knowledge
gained from these fundamental studies provides the foundation for
understanding how this process can go awry and, thus, offers hope for new
means to treat and prevent developmental brain disorders, including
schizencephaly.
Selected references
Asbury, A, et al (eds).
Diseases of the Nervous System:
Clinical Neurobiology. vol. I, 2nd edition, W.B. Saunders Co.,
Philadelphia, pp. 618-621 (1992).
Barth, P.
Schizencephaly and Nonlissencephalic Cortical
Dysplasias. American Journal of Neuroradiology, 13; 104-106
(January/February 1992).
Barkovich, A, and Kjos, B.
Schizencephaly: Correlation of
Clinical Findings with MR Characteristics. American Journal of
Neuroradiology, 13;85-94 (January/February 1992).
Aniskiewicz, A, et al.
Magnetic Resonance Imaging and
Neurobehavioral Correlates in Schizencephaly. Archives of Neurology, 47;
911-916 (August 1990).
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Related NINDS Publications and Information
Cephalic Disorders
fact sheet compiled by the National Institute of Neurological Disorders
and Stroke (NINDS).
Porencephaly
information sheet compiled by NINDS, the National Institute of
Neurological Disorders and Stroke.
Microcephaly
information sheet compiled by NINDS.
This fact sheet is in the public domain. You may copy it.Provided
by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD
20892
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