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double cortex/X-linked lissencephaly syndrome; X-linked subcortical laminar heterotopia/lissencephaly syndrome; subcortical band heterotopia X-linked
Pathology:
- mild brain malformation of the lissencephaly spectrum
- bilateral & symmetric plates or bands of gray matter found in the central white matter between the cortex & cerebral ventricles
- cerebral convolutions usually appear normal
Genetics:
- associated with defects in DCX
Related
lissencephaly X-linked type 1 (LISX1)
General
central nervous system (CNS) malformation
X-linked disease
Database Correlations
OMIM correlations
MORBIDMAP 300121
References
- OMIM :accession 300067
- des Portes V et al.
A novel CNS gene required for neuronal migration and involved
in X-linked subcortical laminar heterotopia and lissencephaly
syndrome.
Cell 92:51-61, 1998
PMID: 9489699
- Gleeson JG et al.
Doublecortin, a brain-specific gene mutated in human X-linked
lissencephaly and double cortex syndrome, encodes a putative
signaling protein.
Cell 92:63-72, 1998
PMID: 9489700