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Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Congenital Abnormalities > Nervous System Malformations > Hydranencephaly
Nervous System Diseases > Nervous System Malformations > Hydranencephaly

Preferred term

Hydranencephaly  

Type

  • Topical Descriptor

Broader concept

Entry terms

  • Absence of Cerebral Hemispheres, Congenital
  • Cerebral Hemispheres, Absence, Congenital
  • Congenital Absence of Cerebral Hemispheres

Scope note

  • A congenital condition where the greater portions of the cerebral hemispheres and CORPUS STRIATUM are replaced by CSF and glial tissue. The meninges and the skull are well formed, which is consistent with earlier normal embryogenesis of the telencephalon. Bilateral occlusions of the internal carotid arteries in utero is a potential mechanism. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. (Menkes, Textbook of Child Neurology, 5th ed, p307)

History note

  • 91(64); was see under ANENCEPHALY 1979-90; was see under ANENCEPHALUS 1967-78

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URI

http://www.yso.fi/onto/mesh/D006832

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