Fact-sheet: Porencephaly
Updated on 09/15/2020 at 3:37 PM
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Definition
Rare congenital disease causing cystic degeneration and encephalomalacia with the formation of porencephalic cysts.
The cysts are lined by white matter and vary in size and location.
Etiologies:
- perinatal cerebral ischemia,
- trauma,
- infection,
- antenatal intracerebral hemorrhage,
- familial form: COL4A1 mutation (--> vessel fragility, antenatal stroke).
Clinical features
Variable:
- asymptomatic patients
- severe manifestations from the first year of life: epilepsy, language disorders, intellectual disability, motor deficits
MRI
- T1: hypointense
- T2: hyperintense
- T2 FLAIR: hypointense
- Diffusion: no restriction
Management
Symptomatic treatment
Differential diagnosis
- Neuroglial cyst
- Arachnoid cyst
- Schizencephaly
