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Porencephaly

Porencephaly is a type of Cephalic disorder. This is an extremely rare disorder of the central nervous system involving a cyst or cavity 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. more...

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Porencephaly most likely has a number of different, often unknown causes, including absence of brain development and destruction of brain tissue. The presence of porencephalic cysts can sometimes be detected by transillumination of the skull in infancy. The diagnosis may be confirmed by CT, MRI, or ultrasonography.

More severely affected infants show symptoms of the disorder shortly after birth, and the diagnosis is usually made before age 1. Signs may include delayed growth and development, spastic paresis (slight or incomplete paralysis), hypotonia (decreased muscle tone), seizures (often infantile spasms), and macrocephaly or microcephaly.

Individuals with porencephaly may have poor or absent speech development, epilepsy, hydrocephalus, spastic contractures (shrinkage or shortening of muscles), 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 lesion. 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.

Read more at Wikipedia.org


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Possible Prenatal Causes of Cerebral Palsy
From OB/GYN News, 9/15/01

Any one of these factors greatly reduces the chance that acute intrapartum hypoxia caused cerebral palsy, according to the statement:

* Umbilical arterial base deficit less than 13 mmol/L or pH greater than 7.

* Infants who have major or multiple congenital or metabolic abnormalities.

* Central nervous system or systemic infection.

* Infants with signs of intrauterine growth restriction.

* Early imaging evidence of long-standing neurologic abnormalities, such as porencephaly.

* Extensive chorioamnionitis.

* Microcephaly at birth (head circumference less than the third centile).

* Major prenatal placental abruption.

* Reduced fetal heart rate variability from the onset of labor.

* Congenital coagulation disorders in the child.

* Presence of other major prenatal risk factors for cerebral palsy (such as preterm irth at less than 34 weeks' gestation, multiple pregnancy or an autoimmune disease).

* Presence of major postnatal risk factors for cerebral palsy (such as postnatal encephalitis, prolonged hypotension, or hypoxia due to severe respiratory disease).

* A sibling with cerebral palsy, especially of the same type.

Source: BMJ 319(7216):1054-59, 1999

COPYRIGHT 2001 International Medical News Group
COPYRIGHT 2001 Gale Group

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