Search on: LISSENCEPHALY 
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Descriptor English:   Lissencephaly 
Descriptor Spanish:   Lisencefalia 
Descriptor Portuguese:   Lissencefalia 
Synonyms English:   Agyria
Pachygyria  
Tree Number:   C10.500.507.249
C16.131.666.507.186
Definition English:   A "smooth brain" malformation of the CEREBRAL CORTEX resulting from abnormal location of developing neurons during corticogenesis. It is characterized by an absence of normal convoluted indentations on the surface of the brain (agyria), or fewer and shallower indentations (pachygryia). There is a reduced number of cortical layers, typically 4 instead of 6, resulting in a thickened cortex, and reduced cerebral white matter that is a reversal of the normal ratio of cerebral white matter to cortex. 
Indexing Annotation English:   note specifics; MICROLISSENCEPHALY is also available
History Note English:   2008 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
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
RA radiography RI radionuclide imaging
RT radiotherapy RH rehabilitation
SU surgery TH therapy
US ultrasonography UR urine
VE veterinary VI virology
Record Number:   52594 
Unique Identifier:   D054082 

Occurrence in VHL:
 

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