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Hemihydranencephaly; a Case Report

Author(s): Damodar Nanaji Balpande | Chandra Shekhar Pathak | Amit Agrawal | Brij Raj Singh

Journal: Iranian Journal of Pediatrics
ISSN 2008-2142

Volume: 19;
Issue: 2;
Start page: 180;
Date: 2009;
Original page

Keywords: Hemihydranencephaly | Brain | Congenital anomalies | Carotid artery | Aplasia

Background: Hemihydranencephaly is a rare disorder of the brain characterized by complete or almost complete unilateral absence of cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx. Case Presentation: Thirteen year-old male child presented with left sided upper and lower limb weakness with facial asymmetry since the age of six months. His magnetic resonance imaging (MRI) scans demonstrated a nearly complete absence of the right cerebral hemisphere including basal ganglion, which was replaced by cerebrospinal fluid with a small residual rim of the occipital cortex. The imaging features were suggestive of right-sided hemihydranencephaly. Conclusion: Patients with hemihydranencephaly provide an experiment of nature with potential implications for normal cognitive development and illustrate how much there is still to be learned about human development.
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