MULTICYSTIC ENCEPHALOPATHY - keywords
multicystic encephalopathy
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references to multicystic encephalopathy
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MCE
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This entity appears to be be an intermediary between leukomalacia/stroke and hydranencephaly. In perinatal asphyxia diffusion-weighted images showing a so-called bright cerebrum may be a precursor of MCE. Following extensive cortical and white matter destruction, multicystic necrosis (laminar cortical necrosis plus diffuse axonal injury) may also lead to postnatal onset ‘hydranencephaly’ with total disruption of the hemispheres and atrophy of the basal ganglia and thalamus.
paraventricular porencephaly
Lane et al. 2021: parechovirus on day 6, demise five days later with collapsing hemispheres
schizencephaly
Congenital polycystic disease of the brain is the term chosen to describe early macrocephaly due to the presence of a multiloculated glio-ependymal cystic process displacing normal underlying brain tissue. Midline interhemispheric cysts of similar complex nature have been reported with consistent features, permitting classification into several types, one where the cysts do not communicate with the third ventricle and without disorder of neuronal migration (see callosal agenesis).
focal
diffuse
multicystic encephalopathy: neuropathology and causes
simple porencephaly
Bilateral multiloculated cavities, a mixture of large and small juxtaposed cysts with irregular borders and shapes, may form in white matter of (third trimester) fetuses or (near) term newborns. They do not communicate with the ventricle, which is lined by a thick glio-ependymal layer, and therefore the term porencephaly is not really appropriate. This condition is referred to as polyporencephaly or multicystic encephalopathy (MCE). Orbitofrontal and basal temporal tissue can be spared. The cysts reach as far as the cortex but do not include the molecular layer, thickened by gliosis. Cavities can be found in striatum and brainstem, proof of associated deep grey matter necrosis: they are symmetrical as expected after asphyxia. The corticospinal tracts are atrophied.
The lesion is picked up in admission ultrasound scans (Alaracon et al. 2025). Focal variants (often posterior) exist, one such variant is seen following (most often temporal lobe) parenchymal destruction in severe antenatal onset alloimmune thrombocytopenia.
Classification of the clastic process is not readily done on the basis of (inter)arterial or venous domains, so that global forebrain hypoperfusion without cardiac arrest is the probable mode of injury, perhaps in association with profound metabolic dysfunction (e.g. hypoglycaemia)(Alkhulaifat et al. 2024).
Causes of MCE- fetal or neonatal encephalitis
(e.g. due to CMV, toxoplasmosis, HSV, mumps or enterovirus-parechovirus, bacterial encephalitis, brain abcess)
- the death of a monozygous cotwin , twin to twin transfusion syndrome
- mitochondrial disorder, sulfite oxidase and molybdenum cofactor deficieny
- asphyxia (ante-, intra-, postpartum)
- bilateral carotid artery occlusion
- multiple haemorrhages
26 weeks gestation infant with Bartter syndrome; the history was complicated by episodes of cardiac arrest due to hyperkalemia because of sudden oliguria superimposed on potassium treatment; in the second month bilateral multicystic cavitation was observed in the posterior parietal and anterior occipitotemporal regions, defying simple within-artery classification
acceptor in a twin to twin transfusion syndrome with complete cerebral destruction in the first month of life; the twin was born at 30 weeks of gestation, the donor died from complete renal necrosis; the first day scan had only presented flaring, suggesting the ischaemic event was perinatal
examples of instances where less extensive but still multicystic brain destruction was recorded, all within (sub)cortex and white matter
multicystic encephalopathy: examples
CT scan in infancy of infant with microcephaly, hypertonia and delay in development: atrophic thalami with calcification and asymmetrical hemisphere destruction with multiple large cysts
total brain destruction following demise of monozygous cotwin
multicystic encephalopathy: examples in twin related conditions
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antepartum cystic cavitation of both occipital lobes in a 34 weeks gestation acceptor of a twin to twin transfusion syndrome, the cotwin having an uneventful perinatal history; ultrasound scans on day 2; this child developed visual handicap without cerebral palsy
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