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Hemihydranencephaly: living with half brain dysfunction.

Piero Pavone1, Francesco Nigro, Raffaele Falsaperla

  • 1Unit of Pediatrics and Pediatric Emergency, Azienda Ospedaliera Universitaria Vittorio-Emanuele-Policlinico, Via Plebiscito 767, 95123, Catania, Italy. ppavone@unict.it

Italian Journal of Pediatrics
|January 18, 2013
PubMed
Summary
This summary is machine-generated.

Hemi-hydranencephaly, a rare brain condition, involves one hemisphere

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Area of Science:

  • Neurology
  • Developmental Biology
  • Pediatrics

Background:

  • Hemi-hydranencephaly is an extremely rare congenital disorder.
  • It is characterized by unilateral absence of the cerebral cortex, replaced by cerebrospinal fluid.
  • Presumed pathogenesis involves prenatal vascular disruption and carotid artery occlusion.

Purpose of the Study:

  • To analyze nine cases of hemi-hydranencephaly.
  • To investigate the clinical and pathological characteristics of this rare condition.
  • To evaluate the potential for normal life and prenatal brain reorganization.

Main Methods:

  • Selection and analysis of nine clinical cases fitting hemi-hydranencephaly criteria.
  • Review of existing literature on hemi-hydranencephaly.
  • Longitudinal follow-up of a previously reported patient for 10 years.

Main Results:

  • Destruction of one cerebral hemisphere does not invariably lead to severe neurological deficits.
  • Some individuals with hemi-hydranencephaly may achieve near-normal life outcomes.
  • The study highlights the brain's potential for prenatal functional reorganization.

Conclusions:

  • Hemi-hydranencephaly can present with variable neurological impairment.
  • Prenatal brain plasticity allows functional compensation by the unaffected hemisphere.
  • This condition serves as a model for studying brain reorganization after injury.