Hydranencephaly

Not to be confused with Hydrocephalus.
Hydranencephaly
Hydrancephaly
Light passing through the skull of a hydranencephalic baby, underscoring the absence of the forebrain
— Image courtesy of D. P. Agamanolis
SpecialtyMedical genetics Edit this on Wikidata

Hydranencephaly[1] is a condition in which the brain's cerebral hemispheres are absent to a great degree and the remaining cranial cavity is filled with cerebrospinal fluid.[2] "Cephalic" is the scientific term for "head" or "head end of body".

Hydranencephaly[3] is a type of cephalic disorder. These disorders are congenital conditions that derive from damage to, or abnormal development of, the fetal nervous system in the earliest stages of development in utero. These conditions do not have any definitive identifiable cause factor. Instead, they are generally attributed to a variety of hereditary or genetic conditions, but also by environmental factors such as maternal infection, pharmaceutical intake, or even exposure to high levels of radiation.[4]

Hydranencephaly should not be confused with hydrocephalus, which is an accumulation of excess cerebrospinal fluid in the ventricles of the brain.

In hemihydranencephaly, only half of the cranial cavity is affected.[5]

  1. ^ Kandel, Eric R. (2013). Principles of neural science (5. ed.). Appleton and Lange: McGraw Hill. p. 1020. ISBN 978-0-07-139011-8.
  2. ^ "Hydranencephaly: Definition, Information, Diagnosis & Prognosis". 29 September 2012. Retrieved 24 February 2018.
  3. ^ National Organization for Rare Disorders. "Hydranencephaly". Rare Diseases Information.[permanent dead link]
  4. ^ National Institute of Neurological Conditions and Stroke, NINDS. "Hydranencephaly Information Page". Disorders. Archived from the original on 2016-12-02. Retrieved 2011-06-19.
  5. ^ Ulmer S, Moeller F, Brockmann MA, Kuhtz-Buschbeck JP, Stephani U, Jansen O (2005). "Living a normal life with the nondominant hemisphere: magnetic resonance imaging findings and clinical outcome for a patient with left-hemispheric hydranencephaly". Pediatrics. 116 (1): 242–5. doi:10.1542/peds.2004-0425. PMID 15995064. S2CID 45671819.