Sporadic Porencephaly: Symptoms, causes, diagnosis and treatment

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Sporadic Porencephaly
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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.

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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.

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Symptoms

• 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.

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Diagnosis 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.

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Treatments

• The treatment of sporadic porencephaly is geared
  toward the specific symptoms that are present in
  each individual. Treatment may include physiothera
  py, 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.

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