Hemihydranencephaly: living with half brain dysfunction.

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Abstract

Hemi-hydranencephaly is a very rare condition characterized by complete or almost nearcompleteunilateral absence of the cortical cortex, which is filled by a sac of cerebrospinalfluid. Prenatal vascular disruption with occlusion of the carotid artery territories ipsilateral tothe damaged brain is the presumed pathogenesis.We have selected nine cases that fit the clinical and pathologic characteristics of hemihydranencephaly,demonstrating that destruction of one hemisphere may be not alwaysassociated with severe neurologic impairment and may allow an almost normal life. Thisdisorder is an example of a possible prenatal re-organization in which the right and leftcerebral hemispheres present functional potentiality to make up the damaged brain.The cases reported in the literature are discussed, including a patient previously reported andfollowed-up for 10 years. A review of the cases is performed with an evaluation of the mostimportant aspect of this rare and mysterious disorder.

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Pavone, P., Nigro, F., Falsaperla, R., Greco, F., Ruggieri, M., Rizzo, R., … Pavone, L. (2013). Hemihydranencephaly: living with half brain dysfunction. Italian Journal of Pediatrics. https://doi.org/10.1186/1824-7288-39-3

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