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DeCS
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Descriptor English:
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Hydranencephaly
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Descriptor Spanish:
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Hidranencefalia
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Descriptor Portuguese:
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Hidranencefalia
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Synonyms English:
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Absence of Cerebral Hemispheres, Congenital
Cerebral Hemispheres, Absence, Congenital
Congenital Absence of Cerebral Hemispheres
Hemihydranencephalies
Hemihydranencephaly
Hydranencephalies
Hydranencephaly with Proliferative Vasculopathy
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Tree Number:
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C10.500.450
C16.131.666.450
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Definition English:
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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) |
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History Note English:
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91(64); was see under ANENCEPHALY 1979-90; was see under ANENCEPHALUS 1967-78
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Allowable Qualifiers English:
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Record Number:
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6984
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Unique Identifier:
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D006832
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Occurrence in VHL:
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Similar:
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DeCS
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