Lissencephaly

Case contributed by Dr Amro Omar

Presentation

Generalised tonic-clonic seizures and developmental delay.

Patient Data

Age: 3 months
CT

CT demonstrates primitive Sylvian fissures leading to an "hour-glass" configuration of the brain. Ventriculomegaly, frontal pachygyria, and occipital agyria are also presented. 

MRI

MRI shows only a few shallow sulci and shallow Sylvian fissures, the cortex is markedly thickened measuring 12-20mm (rather than the normal 3-4mm), and a diffuse white matter demyelination with white matter cysts. 

Case Discussion

Lissencephaly type I is a heterogeneous group of disorders of cortical formation characterised by a smooth brain, with absent or hypoplastic sulci and is strongly associated with subcortical band heterotopia (see classification system for cortical malformations).

Be ware that agyric (smooth) cortex is normal up to 26 weeks of gestation age

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Case information

rID: 32628
Case created: 6th Dec 2014
Last edited: 11th Dec 2016
Inclusion in quiz mode: Included

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