Porencephalic cyst in adult
Porencephalic cyst is a rare entity affecting the central nervous system, whose etiopathogenesis has not yet been clearly defined. It is a cavity within the cerebral hemisphere
From an epidemiological point of view, porencephalic cysts are described in newborns with an estimated incidence of 3.5 per 100,000 live births,
The symptoms that characterize porencephaly are numerous
For the diagnosis of porencephaly, the electroencephalogram may help but the findings are not specific, and radiological techniques are necessary. A cerebral CT scan shows a hypodense intracranial cyst, with a well-defined border and central attenuation due to the cerebrospinal fluid. Mass effect on the adjacent parenchyma is not usually observed, but very large cysts can cause it locally. Brain MRI shows a well-defined, white matter-coated brain cyst with or without gliosis, with the cerebrospinal fluid signal. Fetal MRI is also helpful to differentiate this entity from extra-axial lesions such as arachnoid cysts but also rare tumors.
At the end of the autopsy, the cause of death was identified in acute circulatory failure in a subject suffering from severe coronary artery disease and porencephaly. Therefore, the finding of the porencephalic cyst was incidental, and the origin of this disease was likely attributed to a congenital cause. Indeed, the man had always had good health, except only for recurrent episodes of mild headache.
We deemed this finding of interest both for the rarity of the detected cerebral anomaly and for the man’s survival until adulthood in the absence of severe symptoms. Indeed, the first diagnosis occurred only postmortem and the histopathological analysis confirmed the porencephalic nature of the large cerebral cavitated defect.
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