Encephalomalacia

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Encephalomalacia
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Overview

What is encephalomalacia?

Also known as cerebral or brain softening, encephalomalacia is the loss of the brain tissue as a result of various mechanisms and is regarded as one of the most serious types of brain damage that can affect people of all ages, even in a developing fetus in utero. Localized softening of the brain tissue occurs and this may take place in a specific part of the brain. These areas may include the frontal, parietal, temporal, and occipital lobes of the brain.

Encephalomalacia leads to complete discontinuation of the functioning of the affected part of the brain.

Causes of encephalomalacia

The main cause of this condition is due to restricted blood flow to the brain and this leads to a process called liquefactive necrosis, which means that the tissue dies and becomes a liquid. The most common causes of this decreased blood flow to the brain include:

Multicystic encephalomalacia, which is the type of cerebral softening that occurs in neonates (newborns) and infants, is caused by the following conditions and scenarios that lead to decreased oxygen supply to the brain (cerebral hypoxia):

  • Twin to twin transfusion syndrome.
  • Asphyxia (conditions affecting the umbilical cord leading to decreased oxygen supply to the fetus).
  • Maternal contraction of any of the TORCH infections, namely: Toxoplasmosis, Rubella, Cytomegalovirus (CMV), or Herpes simplex virus (HSV).
  • Meningitis or encephalitis.

What are the different types of cerebral softening?

Depending on what part of the brain is affected, encephalomalacia can be divided into two groups, namely:

  • Leukoencephalomalacia (where the white matter is involved) – The white matter of the brain is responsible for communication between various parts of the brain and the gray matter. Therefore, this condition may affect vital functions such as the heart rate, breathing rate, body temperature control, and blood pressure of the affected patient.
  • Polioencephalomalacia (where the gray matter in involved) – this type of encephalomalacia is also known as cerebrocortical necrosis and is caused by interruption of thiamine (vitamin B1) production. When an individual has a vitamin B1 deficiency, the gray matter of the brain becomes damaged and neurological activity is inhibited. Essential functions such as muscle control, speech, sensory perception, and memory recall are all affected.

There are three distinct varieties of encephalomalacia depending on the appearance (color) of the cerebral softening and the age of onset:

  • Red Softening/Red Infarct
  • As the name implies, affected brain regions appear red as the cause of the cerebral softening is due to a hemorrhagic/bleeding infarct or stroke which causes accumulation of blood around the brain tissue causing it to soften.

    The condition may also occur after the sudden restoration of blood flow to a brain region that was previously blocked by an embolus. In this case, an embolus is a dislodged clot (thrombus) from the heart valve or carotid artery that has relocated and lodged in one of the blood vessels supplying the brain with nutrients and oxygen. In order to re-establish the blood flow, the embolus disintegrates resulting in hemorrhage and this produces the picture of red softening.

  • White Softening/ White Infarct
  • This type of brain softening is due to a white or ischemic infarct. It is seen in areas that have a poor blood supply (are ischemic) due to occlusion of an artery. They are termed as "white" because of the lack of bleeding. Due to the total blockage of blood flow, the neurons in these areas die resulting in cerebral softening.

  • Yellow Softening
  • Brain tissues appear yellow due to the build-up of atherosclerotic plaques within the arteries. It occurs in instances of brain injury where the yellow lymph also surrounds the choroid plexus.

The distinction of the type of encephalomalacia may also be based on the stage of onset of the condition:

  • Early-Onset
  • The brain can become pliable in newborns that are oxygen deprived, hypoglycemic, or infected. The affected brain areas can develop multiple cystic cavities, the already mentioned multicystic encephalomalacia.

  • Late-Onset
  • The risk factors in adults include stroke (ischemic or hemorrhagic), brain trauma, inflammation, or tumors.

Signs and Symptoms

These vary with the underlying cause and the territory of the brain involved. Some are as follows:

  • Drowsiness or somnolence
  • Headache
  • Vertigo
  • A sensation of increased pressure in the head
  • Weakness or paralysis of one half of the body or a limb
  • Blindness
  • Loss of speech
  • Terminal coma

Complications

If encephalomalacia is left untreated, fatal complications may develop in the affected patient. These may include:

  • Seizures, especially in those patients who consume excessive amounts of alcohol.
  • Functional disability.
  • Going into a coma.
  • Death.

Diagnosis

The most commonly used special investigation to confirm the presence of encephalomalacia in affected individuals is magnetic resonance imaging (MRI) of the brain. Computerized tomography (CT) scan of the brain is another useful diagnostic test if an MRI cannot be performed.

What is visualized when performing these investigations is that cavities are discovered where normal brain tissue used to be, in the case of infants and newborns with the condition. In adults, one can note depleted volumes of certain parts of the brain which is most often noted in the frontal and temporal lobes.

Treatment

Unfortunately, there are is no definitive cure for encephalomalacia because it is simply not possible to stimulate the production of new brain cells or to make damaged ones work again.

Clinical research is being conducted to attempt to determine if it is possible to restore the normal functioning ability of partially affected brain matter.

The management of this condition mainly entails diagnosing the underlying cause of the cerebral softening and then treating the affected patient accordingly. In severe cases, the damaged portion of the brain may be surgically removed. However, the issue here is that the consistency of the unaffected portion of the brain undergoes a considerable change due to the removal of the softened cerebral tissue. It is still not clear if these changes in the normal and functional part of the brain can ever make the sensations return to normal again.

Researchers are looking at the possibility of stem cell therapy helping to treat this brain disorder by stimulating the conversion of these cells to functional nerve cells of the brain.

Prognosis

Infants born with encephalomalacia do not have a good prognosis and in many cases, it is not possible to save the affected infant. These patients often suffer from severe neurological deficits even if the doctor is successful in resuscitating them.

Early diagnosis and adequate management are very important in order to control the condition. However, despite the best efforts of those involved, it is still not possible to cure encephalomalacia completely.

Adults diagnosed with this condition have a better outcome with adequate management. In most cases, surgical removal of the damaged cerebral tissue does not result in any other serious problems.

Life expectancy

The life expectancy of an affected individual depends on the duration of the condition and will generally vary from one person to another.

Since infants with encephalomalacia have a poor prognosis, they often have a very short lifespan. However, those who develop the condition later on in life tend to have a relatively normal and long life on receiving the correct treatment.