Ulegyria in Lennox-Gastaut syndrome

Case contributed by Dr Cedric Bohyn

Presentation

African boy, since 2 years in Belgium with an unclear history. His main problems are spastic quadriplegia in Lennox-Gastaut syndrome. Possibly there may have been perinatal asphyxia.

Patient Data

Age: 11
Gender: Male

Global brain atrophy. Mainly pronounced corticosubcortical atrophy left parieto-occipital with shrunken gyri with a 'mushroom'-like appearance and with signal alterations (because of gliosis).

Secondary to the atrophy there is a widening of the left posterior horn of the lateral ventricle as well as very large CSF space bilaterally anterior temporally. Aberrant configuration of the left lateral sulcus (Sylvian fissure).  Left hippocampal atrophy.

Normal location of the falx. No diffusion-restricted brain parenchyma lesions.

Conclusion:
Global brain atrophy mainly of the left hemispherical with bilateral (left more than right) ulegyria.

Case Discussion

The 'mushroom' shape of the gyri and their parasagittal location are typical features of so-called ulegyria.  'Ule' is derived from Latin and means 'scarred'. The lesions are often bilateral and sometimes predominantly unilateral. Diagnostic criteria are atrophic thinning of the cortex, especially at the deep portion with sparing of the apex which causes the mushroom-shape. Underlying white matter abnormalities on T2- and FLAIR-sequences are mostly seen.

They are the result of hypoxic-ischemic encephalopathy due to perinatal asphyxia and typical for full-term infants. They are often associated with pharmacoresistant seizures and surgery may give better outcomes.

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Case information

rID: 59129
Published: 25th Mar 2018
Last edited: 25th Mar 2018
Inclusion in quiz mode: Included
Institution: KU Leuven

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