CASE 9275 Published on 14.04.2011
0

Multicystic encephalomalacia

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Weerakkody YD, McCullough KM

Connected authors
Patient

33 years, male

Categories
Area of Interest Neuroradiology brain ; Imaging Technique CT
Download as PDF Print Show related cases Notify admin
Clinical History
A 33-year-old male patient with background history of mild intellectual impairment as well as longstanding epilepsy (well controlled on Phenytoin for a period of well over two decades) was admitted with an accidental overdose. His admission GCS of 7 /15 promptly improved on appropriate Phenytoin dose correction.
Imaging Findings
The patient had not had any previous neuroimaging. A pre and post-contrast CT examination at the time of admission shows a gross global disruption and malformation of both cerebral and cerebellar parenchymal tissue, which is replaced by numerous cystic spaces, some which interconnect with each other (Fig. 1 and 2). A few focal areas of residual disrupted parenchyma additionally contain heterotropic grey matter. Certain early developmental neural structures such as the corpus callosum are absent while the cerebral arterial and venous anatomy is relatively preserved (normal internal cerebral veins and straight sinus shown on Fig. 3). There was no microcephaly. The calvarial inner table overlying some of the peripheral cystic spaces show a degree of bony scalloping (best seen along the left parietal calvarial vault on the coronal image: Fig. 4) implying a longstanding process.
Discussion
Multi-cystic encephalomalacia is an end result of a relatively diffuse brain parenchymal insult that typically occurs in the perinatal period. On imaging, it is often characterised by the presence of multiple, thin-walled cysts replacing the brain parenchyma. With regards to this case, certain imaging features such as the absence of the corpus callosum suggest that the precipitating event would have occurred in the early antenatal period. Survival into adulthood with minimal neurological sequelae is an extremely uncommon scenario where an extensive literature revealed only a handful of other publications showing radiographic evidence of multicystic encephalomalacia in living adult cases. Due to increased use of antenatal imaging, presentation and detection in adulthood will be even rarer in years to come. In this particular case, it is likely that a clastic lesion (probably haemorrhagic) may have occurred before the 25 week gestation, particularly in view of the presence of heterotopic grey matter.
Differential Diagnosis List
Multicystic encephalomalacia
Complex porencephalic cysts
Hydranencephaly
Final Diagnosis
Multicystic encephalomalacia
Case information
URL: https://www.eurorad.org/case/9275
DOI: 10.1594/EURORAD/CASE.9275
ISSN: 1563-4086
Figure 1

Unenhanced axial image through the level of the lateral ventricles

Unenhanced axial image through the level of the lateral ventricles
CT scan shows a gross global disruption and malformation of cerebral and parenchymal tissue, which is replaced by numerous cystic spaces, some of which interconnect with each other.
Figure 2

Unenhanced axial image through the level of the corona radiata

Unenhanced axial image through the level of the corona radiata
CT scan shows a gross global disruption and malformation of both cerebral and parenchymal tissue, which is replaced by numerous cystic spaces.
Figure 3

Post-contrast midline sagittal image

Post-contrast midline sagittal image
Midline sagittal image shows certain early developmental neural structures such as the corpus callosum being absent while the cerebral arterial and venous anatomy being relatively preserved (with normal internal cerebral veins and straight sinus).
Figure 4

Post-contrast coronal image

Post-contrast coronal image
Post-contrast coronal image shows parts of the left calvarial inner table overlying some of the peripheral cystic spaces demonstrating a degree of bony scalloping implying a longstanding process.