Hemihydranencephaly

Hemihydranencephaly
Hemihydranencephaly
Specialty	Neurology

Last updated November 17, 2025

Hemihydranencephaly is a severe cephalic disorder characterized by complete or almost complete absence of one hemisphere of the cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx. It is a special type of hydranencephaly.

It is a very rare disease. As it stands, only 9 cases have been reported. Cognitive and language functions may be largely or totally normal, although motor function deficits in one half of the body are typical.^[1]

References

↑ Pavone, P.; Nigro, F.; Falsaperla, R.; Greco, F.; Ruggieri, M.; Rizzo, R.; Praticò, A. D.; Pavone, L. (2013). "Hemihydranencephaly: living with half brain dysfunction". Italian Journal of Pediatrics. 39: 3. doi: 10.1186/1824-7288-39-3 . PMC 3564735 . PMID 23324549.

Greco F, Finocchiaro M, Pavone P, Trifiletti RR, Parano E. Hemihydranencephaly: case report and literature review. J Child Neurol. 2001;16 :218–221

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[1] Pavone, P.; Nigro, F.; Falsaperla, R.; Greco, F.; Ruggieri, M.; Rizzo, R.; Praticò, A. D.; Pavone, L. (2013). "Hemihydranencephaly: living with half brain dysfunction". Italian Journal of Pediatrics. 39: 3. doi: 10.1186/1824-7288-39-3 . PMC 3564735 . PMID 23324549.

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