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  Porencephaly 
  
Reviewed  06-11-2002  

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Table of Contents (click to jump to sections)

What is Porencephaly?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations
Related NINDS Publications and Information

What is Porencephaly?
Porencephaly is an extremely rare disorder of the central nervous system involving cysts or cavities in a cerebral hemisphere. The cysts or cavities are usually the remnants of destructive lesions (due to stroke or infection), but are sometimes the result of abnormal development. The disorder can occur before or after birth. Most infants show symptoms of the disorder shortly after birth. Diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic hemiplegia (slight or incomplete paralysis), hypotonia (low muscle tone), seizures (often infantile spasms), and macrocephaly (large head) or microcephaly (small head). Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of a muscle), and mental retardation.

Is there any treatment?
Treatment may include physical therapy, medication for seizure disorders, and a shunt for hydrocephalus.

What is the prognosis?
The prognosis for individuals with porencephaly varies according to the location and extent of the lesions. Some patients with this disorder may develop only minor neurological problems and have normal intelligence, while others may be severely disabled. Others may die before the second decade of life.

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 porencephaly.

Select this link to view a list of studies currently seeking patients.

 Organizations

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

National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

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).

  • Children with Porencephaly, Stroke, and Cerebral Palsy Sought for Study

  • Lay-language descriptions of new program announcements and clinical trials seeking patient volunteers.


    NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

    All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.


    Provided by:
    The National Institute of Neurological Disorders and Stroke
    National Institutes of Health
    Bethesda, MD 20892




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