Anencephaly

A malformation of the nervous system caused by failure of the anterior neuropore to close. Infants are born with intact spinal cords, cerebellums, and brainstems, but lack formation of neural structures above this level. The skull is only partially formed but the eyes are usually normal. This condition may be associated with folate deficiency. Affected infants are only capable of primitive (brain stem) reflexes and usually do not survive for more than two weeks. (From Menkes, Textbook of Child Neurology, 5th ed, p247)

Also Known As:

Aprosencephaly; Absence of Brain, Congenital; Anencephalia; Anencephalus; Congenital Absence of Brain; Hemicranial Anencephaly; Incomplete Anencephaly; Partial Anencephaly; Anencephalias; Anencephalies, Partial; Anencephaly, Hemicranial; Anencephaly, Incomplete; Anencephaly, Partial; Aprosencephalies; Brain Congenital Absence; Partial Anencephalies

Nervous System Diseases: 14178
- Nervous System Malformations: 35
  - Neural Tube Defects: 4299
    - Anencephaly: 426
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
  - Nervous System Malformations: 35
    - Neural Tube Defects: 4299
      - Anencephaly: 426
  - Severe Teratoid Abnormalities
    - Anencephaly: 426

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