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, 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 paresis (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.

Treatment may include physical therapy, medication for seizure disorders, and a shunt for hydrocephalus.

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.

Information provided by the
National Institute of Neurological Disorders and Stroke,
National Institutes of Health

Each year, Medical College of Wisconsin physicians care for more than 180,000 patients, representing nearly 500,000 patient visits. Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.