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.
Also Known As:
Lissencephalia; Agyrias; Lissencephalias; Lissencephalies; Pachygyrias; Agyria; Pachygyria
Networked: 234 relevant articles (0 outcomes, 10 trials/studies)

Disease Context: Research Results

Related Diseases

1. Lissencephaly
2. Malformations of Cortical Development
3. Tuberous Sclerosis (Bourneville's Disease)
4. Congenital Abnormalities (Deformity)
5. Spinal Muscular Atrophy (Progressive Muscular Atrophy)


1. Wynshaw-Boris, Anthony: 10 articles (03/2013 - 06/2003)
2. Hirotsune, Shinji: 8 articles (01/2013 - 08/2003)
3. Chelly, Jamel: 8 articles (12/2012 - 03/2004)
4. Poirier, Karine: 5 articles (12/2012 - 03/2004)
5. Dobyns, William B: 4 articles (09/2015 - 02/2004)
6. Guerrini, Renzo: 4 articles (08/2014 - 02/2004)
7. Yamada, Masami: 4 articles (01/2013 - 10/2009)
8. Mori, Daisuke: 4 articles (02/2010 - 09/2005)
9. Keays, David A: 3 articles (10/2015 - 01/2007)
10. Kato, Mitsuhiro: 3 articles (02/2014 - 02/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lissencephaly:
1. DNA (Deoxyribonucleic Acid)IBA
2. Dyneins (Dynein)IBA
3. Platelet Activating FactorIBA
12/01/1997 - "Previous studies have shown that LIS1, the defective gene found in patients with lissencephaly, is a subunit of the platelet-activating factor acetylhydrolase. "
01/01/2006 - "In order to test the hypothesis that these features may be related with genes that regulate neuronal migration, we analyzed two genomic regions: the lissencephaly critical region (chromosome 17p) encompassing the LIS1 gene and which is involved in human lissencephaly; and the genes related to the platelet-activating-factor, functionally related to LIS1, in 52 schizophrenic patients, 36 bipolar I patients and 65 normal control subjects. "
05/01/2001 - "Furthermore, abnormal prenatal platelet activating factor (PAF) signalling may result in lissencephaly, a disorder of neuronal migration. "
04/15/2006 - "In cell and animal models, C-terminus-truncated DISC1 disrupts intracellular transport, neural architecture and migration, perhaps because it fails to interact with binding partners involved in neuronal differentiation such as fasciculation and elongation protein zeta-1 (FEZ1), platelet-activating factor acetylhydrolase, isoform Ib, PAFAH1B1 or lissencephaly 1 protein (LIS1) and nuclear distribution element-like (NUDEL). "
08/01/1998 - "Heterozygous mutation or deletion of the beta subunit of platelet-activating factor acetylhydrolase (PAFAH1B1, also known as LIS1) in humans is associated with type I lissencephaly, a severe developmental brain disorder thought to result from abnormal neuronal migration. "
4. Double cortexIBA
5. Technetium Tc 99m Exametazime (Ceretec)FDA Link
6. CollagenIBA
7. Diethylhexyl Phthalate (DEHP)IBA
8. TubulinIBA
9. Retinaldehyde (Retinal)IBA
10. ColpocephalyIBA

Therapies and Procedures

1. Sutures (Suture)
2. Ligation
3. Intraperitoneal Injections