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Sporadic Porencephaly

Introduction of Sporadic Porencephaly

Sporadic porencephaly is a rare disorder affecting the central nervous system. In


porencephaly cysts or cavities form on the surface of the brain. These cysts or
cavities may become filled with cerebrospinal fluid, a colourless fluid that normally
surrounds the brain and spinal cord to provide protection and nourishment. The
severity and associated symptoms of porencephaly vary dramatically from one
person to another based upon the size and exact locations of the fluid-filled
cavities or cysts.
Porencephaly may be classified as sporadic or familial. Sporadic porencephaly can
have many different causes including infection just before or just after birth
(perinatal infection), trauma, maternal disease or sickness, maternal diabetes, or
maternal use of alcohol or drugs such as cocaine during pregnancy.

Causes of Sporadic Porencephaly


Sporadic porencephaly is caused by damage to the cerebral hemispheres of the
brain, which results in the formation of fluid-filled cavities or cysts on the surface
of the brain. Researchers believe that the damage or loss of brain tissue that
characterizes porencephaly results from infection interrupted or obstructed
blood flow (ischemia) to the brain, or bleeding (haemorrhaging) in the brain.
A variety of different conditions can potentially cause the localized brain tissue
degeneration that ultimately leads to sporadic porencephaly. Such conditions
include infection just before or shortly after birth (perinatal infection), too little
oxygen in the body just before or shortly after birth (perinatal asphyxia), birth
trauma, drug or alcohol use by the mother during pregnancy, maternal sickness
or infection, maternal diabetes, or injury or trauma (particularly to the
abdominal area) to the mother during pregnancy.

Symptoms of Sporadic Porencephaly

The symptoms and severity of sporadic porencephaly vary greatly from one
individual to another based on the size and exact location of the fluid-filled cyst
or cavity. Some individuals may only have minor physical complications and
intelligence may be unaffected others can have severe, disabling complications.
Possible symptoms include delays in growth and development, diminished
muscle tone (hypotonia), seizures, and microcephaly or macrocephaly,
conditions in which head circumference is either smaller (micro) or larger
(macro) than would be expected in a child based upon age and weight.

Diagnoses of Sporadic Porencephaly

A diagnosis of sporadic porencephaly can be made before or after birth through a


variety of specialized imaging tests such as an ultrasound, computed tomography
(CT) scan, or magnetic resonance imaging (MRI). During an ultrasound, reflected
sound waves are used to make an image of the developing foetus. During CT
scanning, a computer and x-rays are used to create a film showing cross-sectional
images of certain tissue structures. An MRI uses a magnetic field and radio waves
to produce cross-sectional images of particular organs and bodily tissues.

Treatments of Sporadic Porencephaly


The treatment of sporadic porencephaly is geared toward the specific symptoms
that are present in each individual. Treatment may include physical therapy,
speech therapy, anti-convulsant medications for seizures, and a shunt to treat
hydrocephalus
by
draining
excess
fluid
from
the
skull.
Early intervention is important in ensuring that children with sporadic
porencephaly reach their highest potential. Services that may be beneficial for
some affected individuals include medical, social or vocational services such as
special remedial education.