What is Encephalomalacia? (Symptoms and Treatment)

Encephalomalacia, also known as cerebral softening, is a very serious disorder inflicting permanent tissue damage to the patient’s brain.

The disease is not limited to specific ages, genders or races.

Even embryos in the womb and infants may be affected by this medical condition primarily resulting from trauma.

The precise definition of encephalomalacia is a localized softening of brain tissue.

The softening can actually result in tissue loss or brain scarring, much like that of multiple sclerosis.

If scars form, they are usually a dense network of neuroglia in areas where encephalomalacic damage has occurred.

Because the brain’s tissue becomes so compromised as a result of the disorder, encephalomalacia results in many alterations to the patient’s functioning and health, as well as additional diseases and disorders.

 

Types of Encephalomalacia

Encephalomalacia manifests in one of two forms.

Each of the two presents very differently, in different regions of the brain.

 

1.Polioencephalomalacia (cerebrocortical necrosis) results in damage to the gray matter.(1)

This version of encephalomalacia affects;(2)

muscle control

speech

sensory perception

memory

and emotions

2.Leukoencephalomalacia causes damage to the white matter of the brain.(3,4,5)

This area of the brain is responsible for transmission of nerve signals throughout the cerebrum and between that section of the brain and lower brain centers.

 

The advancement of encephalomalacia, or cerebral softening, is categorized by a color of the damage and its stages.

These categories include:

  • Red softening: This stage is entered when there is a hemorrhagic infarct. A hemorrhagic infarct is the death of tissues due to oxygen starvation caused by blocked veins in the brain. Red blood cells enter the area of the hemorrhagic infarct and cause a reddening of the tissues. The vein blockage, also known as an embolism, may be due to blood clot, gas bubble, fat globule or foreign material in the bloodstream.(6)
  • Yellow softening: Brain tissue becomes yellowed and soft due to atherosclerotic plaque buildup in the brain’s arteries. At the same time, yellow lymph builds around the choroid plexus in the brain, adding to the yellow color.(7,8)
  • White softening: White softening results when an area of the brain does not have blood flow or blood flow is reduced to a very minimal amount. The neuronal tissue dies and softens, as a result.(9)

Encephalomalacia can affect any age. However, there are differences in how the condition presents in early life, versus how adults are affected by it.

Newborns usually suffer encephalomalacia and its degeneration of white matter in their brains due to asphyxia or infection.

The condition was once attributed to birth trauma, but that has been reconsidered by medical researchers, as there is no causal evidence to back up that hypothesis.

Additionally, there is evidence to connect low sugar and high protein levels in cerebrospinal fluid as a link to susceptibility to cerebral softening from a later disease or virus.(10,11)

Infants affected by the condition suffer more severely than adults due to their inability to recover lost brain tissue or use other parts of their brain as compensation for damaged areas.(12)

Adults recover more often, as their brains are able to compensate and correct when tissue is lost.

Adult mortality is less frequently an issue than that of infants affected by cerebral softening.

 

Causes

There are multiple diseases and conditions which can lead to encephalomalacia.

The most frequent cause of cerebral softening is head trauma that is so injurious that it leads to softening of brain tissues.(13,14,15)

Stroke is another frequent cause of the condition.(15)

Bleeding resulting from either injury or stroke can cause encephalomalacia, as the condition often occurs in areas with abnormal pooling of blood.(Hemorrhage)

Likewise, an absence of sufficient blood flow can also trigger encephalomalacia to develop.

Other common causes include:(15)

» Degeneration or deterioration of the brain, as a rare cause

» Cerebral stroke, also known as an infarction, wherein blood flow is stopped due to an obstruction

» Blocked blood vessels which supply blood to the brain

» Cerebral ischemia, which is a mini-stroke, the result of insufficient blood supply and a resulting metabolic deficiency

» Traumatic brain injury, also known as craniocerebral trauma, such as a violent blow to the brain, bullet or foreign object penetration into the brain, or other mechanical force at the brain which causes brain dysfunction

» Infection resulting from a chemical toxin or biological pathogen spreading to the brain or making contact with it directly

 

Common Symptoms

With cerebral softening, there are varied symptoms which range from mild to catastrophic.

The type of symptoms to manifest largely rely upon the area of the brain affected and the functions for which the affected region of the brain is responsible.

Each person is affected uniquely and many factors influence how their symptoms manifest.

Such factors may include age and other existing health conditions.

People with cerebral softening do often exhibit some telltale signs of the encephalomalacia’s onset.

Those include:(16,17)

  • Episodic drowsiness or extreme need to sleep, also known as somnolence
  • Diminished coordination, wobbling and clumsiness in movement, sometimes called ataxia
  • Temporary or permanent onset of visual impairment or blindness
  • Vertigo or a severe head spinning sensation
  • Head pressing
  • Severe headache
  • Memory loss, if the frontal lobe is affected
  • Mood swings, also in the event of the frontal lobe’s involvement
  • Terminal coma

 

Diagnosis of Cerebral Softening

Magnetic resonance imaging, also known as MRI, is an imaging diagnostic test which provides clear pictures of the brain and its health.

MRIs show signs of encephalomalacia, when the condition is present.

Once the softening of brain tissue is discovered, a CT scan, computed tomography, helps medical professionals determine when the softening of the brain tissue began.

A CT scan will indicate:

» Brain region of hypoattenuation, meaning the area where tissue has become less dense than other brain tissue due to cerebral softening

» Volume of tissue lost to softening

» Whether the condition is occurring as is common, in anteroinferior frontal and temporal lobes, or other parts of the brain

» If gliosis and Wallerian degeneration are present

 

Treatment

Unfortunately, once brain tissue is destroyed, there is no regrowth or curing that particular portion of the brain.

This means that there is not a cure for cerebral softening which can reinstill lost capabilities or functioning.

Researchers are working on determination of whether partially affected brain tissues can improve to regain normal functioning.(18)

Treatment of encephalomalacia primarily focuses on early detection of the disorder and determination of its underlying cause of brain consistency changes.

The next step in treatment is to deal with the underlying issue.

If damaged brain tissue is severe, some of that matter may be removed through surgery.

But surgery can cause other problems and it is not clear if removal of damaged tissue can help sensations and functioning improve in remaining areas.

Researchers are working on a variety of possible treatments, both directly and indirectly for encephalomalacia.(19,20)

One such treatment which may offer promise is stem cell therapy.

Stem cell therapy is being pursued as a possible treatment for multiple types of brain disorders and degeneration.(21,22,23)

 

Prognosis

Infants with encephalomalacia do not have a good prognosis attached to their condition.(24)

Most often, such young patients cannot be saved through any treatment.

Survival of the cerebral softening unfortunately carries a high possibility of that infant suffering extreme neurological deficits.

Infants born with cerebral softening are not expected to live long or enjoy a normal life.

Adults, on the other hand, do have a better prognosis when diagnosed with encephalomalacia.

The earlier the condition is discovered, the better the chances are for that adult patient’s recovery.

Adults can still enjoy a long life, despite cerebral softening.

It is just very critical that treatment is pursued and the right treatment for that particular patient is administered to ensure prevention of additional degeneration.

Life span for adults with the condition varies widely from person to person.

There are many factors in life expectancy, including severity of the individual’s case of encephalomalacia and how much damage the condition has already caused.

The patient’s overall health condition, other diseases or disorders and age are also determining factors in individual life expectancy.

Further reading


References

(1)https://en.wikipedia.org/wiki/Polioencephalomalacia
(2)http://www.merckvetmanual.com/mvm/nervous_system/polioencephalomalacia/overview_of_polioencephalomalacia.html?qt=&sc=&alt=
(3)http://www.askjpc.org/vspo/show_page.php?id=455
(4)http://www.ncbi.nlm.nih.gov/pubmed/1995569
(5)http://vet.sagepub.com/content/48/5/1016.full
(6)http://www.ncbi.nlm.nih.gov/pubmed/18108333
(7)https://books.google.hr/books?id=umsSAAAAYAAJ&pg=PA776&lpg=PA776&dq=#v=onepage&q&f=
(8)https://books.google.hr/books?id=GqEEAAAAQAAJ&pg=PA423&lpg=PA423&dq=#v=onepage&q&f=
(9)https://books.google.hr/books?id=v5lUAAAAYAAJ&pg=PA299&lpg=PA299&dq=#v=onepage&q&f=
(10)http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2952976/
(11)http://www.ncbi.nlm.nih.gov/books/NBK398/
(12)http://europepmc.org/abstract/MED/9082884
(13)http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1064998/
(14)http://jnnp.bmj.com/content/73/2/173.full
(15)https://books.google.hr/books?id=o9cbq1eTpsAC&pg=PR5&lpg=PR5&dq=Advances+in+Encephalomalacia+Research+and+Treatment:+2012+Edition&source=bl&ots=8qzR2neRWq&sig=r8vXh13mjkKaLn7oQMk-uoTxC4Q&hl=hr&sa=X&ved=0ahUKEwjG1aCc2YbLAhUD1ywKHYQKDR8Q6AEIGTAA#v=onepage&q=Advances in Encephalomalacia Research and Treatment%3A 2012 Edition&f=
(16)http://thejns.org/doi/abs/10.3171/jns.1956.13.4.0278?journalCode=jns
(17)http://www.ncbi.nlm.nih.gov/pubmed/7425567
(18)http://stemcells.nih.gov/info/Regenerative_Medicine/pages/2006chapter3.aspx
(19)https://www.ncbi.nlm.nih.gov/pubmed/18415716
(20)http://www.maneyonline.com/doi/10.1179/ptr.2001.6.4.233
(21)http://www.ninds.nih.gov/research/stem_cell/
(22)http://www.eurostemcell.org/commentanalysis/stem-cell-therapies-and-neurological-disorders-brain-what-truth
(23)http://www.medicalnewstoday.com/articles/241215.php
(24)http://www.ajnr.org/content/4/5/1111.full.pdf
“http://www.ncbi.nlm.nih.gov/pubmed/22134284
“http://radiopaedia.org/articles/encephalomalacia
“http://www.rightdiagnosis.com/medical/encephalomalacia.htm
“http://www.wisegeekhealth.com/what-is-encephalomalacia.htm
“http://www.lookfordiagnosis.com/faq.php?term=Encephalomalacia〈=1
“http://www.justanswer.com/health/0qfai-cause-effect-cerebral-softening-encephalomalacia.html


 

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