Descriptors Found: 1
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Descriptor English:   Hydranencephaly 
Descriptor Spanish:   Hidranencefalia 
Descriptor Portuguese:   Hidranencefalia 
Synonyms English:   Absence of Cerebral Hemispheres, Congenital
Cerebral Hemispheres, Absence, Congenital
Congenital Absence of Cerebral Hemispheres
Hydranencephaly with Proliferative Vasculopathy  
Tree Number:   C10.500.450
Definition English:   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 English:   91(64); was see under ANENCEPHALY 1979-90; was see under ANENCEPHALUS 1967-78 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
DG diagnostic imaging DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RT radiotherapy
RH rehabilitation SU surgery
TH therapy UR urine
VE veterinary VI virology
Record Number:   6984 
Unique Identifier:   D006832 

Occurrence in VHL: