NINDS Schizencephaly Information Page: NINDS


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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).

 Organizations

The ARC
1010 Wayne Avenue
Suite 650
Silver Spring, MD 20910
Info@thearc.org
http://www.thearc.org/
Tel: 301-565-3842
Fax: 301-565-3843 or -5342

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
resourcecenter@modimes.org
http://www.modimes.org/
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203

National Information Center for Children and Youth with Disabilities
P.O. Box 1492
Washington, DC 20013-1492
nichcy@aed.org
http://www.nichcy.org/
Tel: 202-884-8200 800-695-0285
Fax: 202-884-8441

National Institute of Child Health and Human Development (NICHD)
National Institutes of Health
Bldg. 31, Rm. 2A32
Bethesda, MD 20892-2425
NICHDClearinghouse@mail.nih.gov
http://www.nichd.nih.gov/
Tel: 301-496-5133 800-370-2943

Related NINDS Publications and Information

  • Cephalic Disorders Fact Sheet
    Cephalic Disorders fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
  • Porencephaly Information Page
    Porencephaly information sheet compiled by NINDS, the National Institute of Neurological Disorders and Stroke.
  • Microcephaly Information Page
    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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