CASE 1095 Published on 30.07.2001
0

Multicystic encephalomalacia

Section

Neuroradiology

Case Type

Clinical Cases

Authors

S. Cakirer

Connected authors
Patient

6 months, male

Categories
No Area of Interest ; Imaging Technique MR
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Clinical History
A 6 month-old baby boy with a history of placental insufficiency and premature birth at 7 months of gestational age referred with failure in motor development.
Imaging Findings
A 6 month-old baby boy with a history of placental insufficiency and premature birth at 7 months of gestational age referred with failure in motor development. MRI study of the cranium was performed on a 1.5 T MR scanner, with SE T1, FSE T2 weighted, FLAIR sequences on three planes.
Discussion
Multicystic encephalomalacia (MCE) results from a diffuse insult to the brain late in gestation, during birth, or after birth. MCE may occur as a result of ischemia, infection, or developmental insults. Multiple cystic cavities of variable size form in the necrotic area, separated from one another by glial septations. The location of the lesions varies with the nature of the insult. If caused by thromboembolic infarction, the affected area will be in the distribution of a major cerebral artery. If the injury is a result of partial asphyxia, it will tend to be located in the cortex and peripheral white matter, primarily in the watershed areas. When the insult is very severe, only the immediate periventricular white matter may be spared. When the injury is a result of an infection, the site of the encephalomalacia is nonspecific, which will vary with the region of the brain that was injured by the infection. The computed tomography appearance is characterized by hypodense tissues containing cysts of various sizes, and septations are often seen within the damaged region. MRI reveals the affected areas as ill-defined areas of prolonged T1 and T2 relaxation containing loculations of fluid, and separated by glial septae which are composed of glial cells and some viable neurons. Ultrasonography is the most sensitive modality in the detection of glial septa, but is less useful in the overall evaluation of the brain. Differential diagnosis of MCE includes encephaloclastic porencephaly, hydranencephaly. The differentiation of an encephaloclastic porencephaly is not difficult by its smooth-walled, fluid-filled cavity ( a porencephalic cyst) in contrary to the encephalomalacic cavity which contains septations and an irregular wall composed primarily of reactive astrocytes. Hydranencephaly is characterized by the destruction and resorption of most of the brain mantle except frontal regions, and by CSF-filled thin-walled cerebral hemispheres.
Differential Diagnosis List
Multicystic encephalomalacia
Final Diagnosis
Multicystic encephalomalacia
Case information
URL: https://www.eurorad.org/case/1095
DOI: 10.1594/EURORAD/CASE.1095
ISSN: 1563-4086
Figure 1

Figure 1

Figure 1
Axial FSE T2-weighted MR image shows multiple cysts of various sizes, with a signal intensity almost isointense to CSF, which are separated by linear septae composed of glial cells and some viable neurons. The immediate periventricular white matter is spared. The overlying cerebral cortex is thinned. Note the ex vacuo dilatation of third and lateral ventricles are associated, and diencephalic structures are preserved.
Figure 1
Axial SE T1-weighted MR image shows multiple cysts of various sizes, with a signal intensity almost isointense to CSF, which are separated by linear septae. The immediate periventricular white matter is spared. The overlying cerebral cortex is thinned. Note the ex vacuo dilatation of bilateral lateral ventricles are associated.
Figure 1
Sagittal SE T1-weighted MR image shows multiple cysts of various sizes, with a signal intensity almost isointense to CSF, which are separated by linear septae. The corpus callosum is atrophic in nature. The immediate periventricular white matter and somewhat anterior frontotemporal areas are spared. The overlying cerebral cortex is thinned. Note the ex vacuo dilatation of bilateral lateral ventricles are associated. Posterior fossa and diencephalic structures are preserved.
Figure 1
Coronal FLAIR MR image shows multiple cysts of various sizes, with a signal intensity isointense to CSF, which are separated by linear septae composed of glial cells and some viable neurons. The immediate periventricular white matter and diencephalic structures are spared. The overlying cerebral cortex is thinned. Note the ex vacuo dilatation of the ventricles.
Figure 1