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Classical Lissencephalies and Subcortical Band Heterotopias Summary

Description: Disorders comprising a spectrum of brain malformations representing the paradigm of a diffuse neuronal migration disorder. They result in cognitive impairment; SEIZURES; and HYPOTONIA or spasticity. Mutations of two genes, LIS1, the gene for the non-catalytic subunit of PLATELET-ACTIVATING FACTOR ACETYLHYDROLASE IB; and DCX or XLIS, the gene for doublecortin, have been identified as the most common causes of disorders in this spectrum. Additional variants of classical (Type I) lissencephaly have been linked to RELN, the gene for reelin, and ARX, the gene for aristaless related homeobox protein. (From Leventer, R.J., et al, Mol Med Today. 2000 Jul;6(7):277-84 and Barkovich, A.J., et al, Neurology. 2005 Dec 27;65(12):1873-87.)

Also Known As: Agyria-Pachygyria-Band Spectrum; Double Cortex Syndrome; Isolated Lissencephaly Sequence; Lissencephaly Syndrome, Miller-Dieker; Lissencephaly, Miller-Dieker Show All >>

Networked: 101 relevant articles (1 outcomes, 2 trials/studies) for this Disease

Key Drugs and Agents for Classical Lissencephalies and Subcortical Band Heterotopias

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Drugs and Important Biological Agents (IBA) related to treatments:

lamotrigine (Lamictal) : 1 outcome in 1 result : FDA 33 Generic
Double cortex : 1 study in 19 results : IBA
Nonsense Codon (Nonsense Mutation) : 1 study in 1 result : IBA
Platelet Activating Factor : 8 results : IBA
DNA (Deoxyribonucleic Acid) : 5 results : IBA
Genetic Markers (Genetic Marker) : 4 results : IBA
X-linked infantile spasm syndrome : 4 results : IBA
Partington X-linked mental retardation syndrome : 4 results : IBA
Transcription Factors (Transcription Factor) : 3 results : IBA
Dyneins (Dynein) : 3 results : IBA

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Diseases Related to Classical Lissencephalies and Subcortical Band Heterotopias

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Key Therapies for Classical Lissencephalies and Subcortical Band Heterotopias

Efficacy Chart >>