What is encephalomalacia?

What is Encephalomalacia?

Encephalomalacia is the softening or loss of brain tissue that occurs after cerebral infarction, ischemia, infection, craniocerebral trauma, or other injury to the brain. 1

Pathophysiology and Definition

• Encephalomalacia represents the liquefaction of brain parenchymal necrosis following various insults including cerebral ischemia, infection, hemorrhages, and physical trauma 1, 2
• The term is typically used during gross pathologic inspection to describe blurred cortical margins and decreased consistency of brain tissue after infarction 1
• The damaged area is often surrounded by glial cell proliferation (gliosis) in response to the injury 2
• This represents permanent brain tissue damage that cannot be reversed, so management focuses on symptom control and preventing complications 3

Common Etiologies

In Infants and Children

• Multicystic encephalomalacia refers to multiple cystic cavities of various sizes in the cerebral cortex, most notably after perinatal hypoxic-ischemic events 1
• Results from various damages to the immature brain in early infancy, typically causing severe psychomotor retardation 4

In Adults

• Cerebrovascular events (stroke, cerebral infarction) are the most common cause 5
• Physical trauma including penetrating brain injuries and severe head trauma 1, 5
• Iatrogenic injury such as complications from endoscopic sinus surgery 1
• Anoxic-ischemic episodes can occur at any age 4

Clinical Presentation

• Symptoms are non-specific and depend on the location and extent of brain tissue loss 5
• May include neurobehavioral deficits, motor weakness, seizures, cognitive impairment, aphasia, vertigo, and giddiness 5, 2
• In cases of frontal lobe involvement, patients may develop intractable epilepsy requiring surgical intervention 6
• There can be marked discrepancy between morphological changes on imaging and actual neurological deficits, particularly when injury occurs during periods of brain plasticity 4

Diagnostic Approach

• Brain MRI is the most useful imaging modality for evaluating encephalomalacia and gliosis, as it is more sensitive than CT for detecting subtle findings adjacent to the calvarium and small white matter lesions 3
• Susceptibility-weighted imaging should be included to increase sensitivity for detecting paramagnetic blood products from previous hemorrhage 3
• Assessment should evaluate the extent of encephalomalacia, presence of gliosis, and any associated structural abnormalities such as calvarial defects 3
• CT and MRI demonstrate the characteristic appearance of tissue loss with cystic changes and surrounding gliosis 5, 4

Management Principles

Medical Management

• Anticonvulsant treatment should be prescribed for patients who have experienced seizures, with first-line treatment being single-drug therapy 3
• Appropriate analgesic treatment should be provided if the patient experiences headaches or other pain 3
• Corticosteroids may be considered if there is evidence of edema causing mass effect or neurological symptoms 3
• Treatment is symptomatic and supportive, as the brain tissue damage is irreversible 3, 5

Surgical Considerations

• Surgery should be considered only for specific indications: recurrent seizures unresponsive to medical management, progressive neurological deficits, or evidence of mass effect causing significant symptoms 3
• For intractable frontal lobe epilepsy secondary to encephalomalacia, surgical resection is very effective, with 70% of patients achieving seizure freedom or rare seizures 6
• Presence of focal fast frequency discharge (focal ictal beta pattern) on scalp EEG predicts seizure-free outcome after surgical resection 6
• Complete resection of the encephalomalacia should be attempted when feasible, as this may be a favorable predictive factor for seizure control 6
• For patients with calvarial defects, cranioplasty may be considered to protect the brain and improve cosmesis 3

Rehabilitation

• Physical therapy and rehabilitation must be tailored to address specific neurological deficits 2
• Proprioceptive neuromuscular facilitation (PNF) methods can be effective for regaining motor function 2
• Tele-rehabilitation can play a crucial role in maintaining continuity of care 2

Follow-up and Monitoring

• Regular clinical follow-up to assess neurological status and seizure control is necessary 3
• Periodic brain MRI to monitor for any changes in the encephalomalacia or gliosis is recommended 3
• Monitoring should continue long-term, as the clinical picture is typically static but complications like epilepsy may develop 4

Important Clinical Caveats

• Surgical intervention in eloquent brain areas carries significant risks and must be carefully weighed against potential benefits 3
• The neurological deficit may be minimal despite severe morphological changes, particularly when injury occurs during periods of brain plasticity in childhood 4
• Associated conditions such as vitamin D deficiency may be present and should be evaluated 2
• The interval between injury and symptom onset can be years, requiring careful history-taking to identify the original insult 5, 4