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Disease Hydranencephaly

Name Hydranencephaly
Synonyms Absence of Cerebral Hemispheres, Congenital | Cerebral Hemispheres, Absence, Congenital | Congenital Absence of Cerebral Hemispheres | Hemihydranencephalies | Hemihydranencephaly | Hydranencephalies | Hydranencephaly with Proliferative Vasculopathy
Definition 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)
Categories Congenital abnormality | Nervous system disease
MeSH® ID D006832
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Top ↑ Ancestors

1. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Nervous System Malformations Has associated chemicals Has associated genes Has associated exposure references Hydranencephaly Has associated chemicals Has associated genes
2. DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities Has associated chemicals Has associated genes Has associated exposure references Congenital Abnormalities Has associated chemicals Has associated genes Has associated exposure references Nervous System Malformations Has associated chemicals Has associated genes Has associated exposure references Hydranencephaly Has associated chemicals Has associated genes

Top ↑ Descendants

Hydranencephaly Has associated chemicals Has associated genes
  Encephaloclastic Proliferative Vasculopathy Has associated chemicals Has associated genes
  Hydranencephaly with Renal Aplasia-Dysplasia
  Microhydranencephaly Has associated chemicals Has associated genes