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  Encyclopedia of Keywords > Forebrain > Holoprosencephaly   Michael Charnine

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  1. Holoprosencephaly is the absence or incomplete cleavage of the forebrain (prosencephalon) into the two cerebral hemispheres.
  2. HOLOPROSENCEPHALY is a disorder characterized by the failure of the prosencephalon (the forebrain of the embryo) to develop.
  3. Holoprosencephaly (HPE) is a malformation sequence where the cerebral hemispheres fail to separate into distinct left and right halves.
  4. Holoprosencephaly is the most common abnormality of the development of the forebrain (front part of the brain) in humans.
  5. Holoprosencephaly (HPE) is a congenital anomaly in which there is incomplete development of the brain.

Lobar Holoprosencephaly

  1. The malformation is of graded severity (alobar, semilobar, and lobar holoprosencephaly) with respect to both the brain and the face.


  1. Semilobar holoprosencephaly, in which the brain's hemispheres have a slight tendency to separate, is an intermediate form of the disease.


  1. In lobar holoprosencephaly there is normal separation of the ventricles and thalami but absence of the septum pellucidum.

Parietal Lobes

  1. Middle Interhemispheric Variant of Holoprosencephaly (MIHV) -- where the middle of the brain (posterior frontal and parietal lobes) are not well separated.


  1. When the hemispheres fail to develop into 2 separate hemispheres but rather form a single, midline mass of cerebral tissue, the result is holoprosencephaly.


  1. Lobar holoprosencephaly is associated with mental retardation.


  1. The result is holoprosencephaly and inhibited development of frontonasal process in to medial nasal swelling and to premaxilla prolabium and columella.


  1. Holoprosencephaly (HPE) with cyclopia is an infrequent congenital anomaly of the forebrain system.


  1. Mutations in the human sonic hedgehog gene (shh) cause holoprosencephaly (HPE) as a result of the loss of the ventral midline.
  2. The sonic hedgehog (Shh) gene has been identified in defects that cause hydrocephalus secondary to holoprosencephaly.


  1. Holoprosencephaly, once called arhinencephaly, consists of a spectrum of defects or malformations of the brain and face.
  2. When the embryonic prosencephalon fails to divide the brain into two lobes, it results in a condition known as holoprosencephaly.
  3. Because holoprosencephaly involves abnormalities of the entire prosencephalon, a major portion of the brain can be affected.

Brain Abnormalities

  1. The diagnosis is made by the demonstration of brain abnormalities, such as holoprosencephaly.


  1. Holoprosencephaly, or a failure in the hemispheres of the forebrain to separate [ 16], is the cause of the cyclopia in cyc, sqt, and oep mutants.
  2. Cyclopia may be associated with holoprosencephaly, the most common developmental defect of the forebrain with an incidence of 1:250 during embryogenesis.
  3. Definition: Alobar holoprosencephaly is the most severe form of cleavage failure of the forebrain (prosencephalon) before 6 weeks of gestation.


  1. If the forebrain fails to divide into normal right and left hemispheres, deformities in the face and brain, or holoprosencephaly, results.
  2. In holoprosencephaly, callosal anomalies are atypical; for example, the splenium may be present without a genu or body.
  3. When the embryonic forebrain (the prosencephalon) fails to sufficiently divide into the two cerebral hemispheres, holoprosencephaly (HPE) results.


  1. Forebrain
  2. Nervous System > Central Nervous System > Cerebrum > Prosencephalon
  3. Brain Abnormalities
  4. Genu
  5. Splenium
  6. Books about "Holoprosencephaly" in

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  Short phrases about "Holoprosencephaly"
  Originally created: April 04, 2011.
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