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Hypoxic ischemic encephalopthy with bilateral frontoparietal and temporal leucomalacia

Case contributed by Dr Mohammad A. ElBeialy


Convulsions and delayed developmental milestones. Enlarged head on clinical examination.

Patient Data

Age: 10 months
Gender: Male

Bilateral rather symmetrical moderate dilatation of the supra-tentorial ventricular system (namely the lateral ventricles as well as the third ventricle). Associated bilateral periventricular and fronto-parietal demyelinating lesions with hyperintense T2/ flair signal intensity.

Bilateral rather symmetrical subcortical cystic lesions are seen predominantly at the fronto-temporal lobes displaying bright T2W and low T1W/FLAIR signal. 

Case Discussion

Hypoxic ischemic encephalopthy with bilateral frontoparietal and temporal leucomalacia.

The differential diagnosis would be megalencephalic leucoencephalopathy with white matter disease involving the subcortical U fibers as well as anterior fronto-temporal subcortical cysts is diagnostic of Van Der Knaap disease. The cysts are usually intraparenchymal ---what we are seeing is bilateral encephalomalacia of the temporal lobes with compensatory ventricular dilatation. For van der Knaap disease, the cysts are intraparenchymal, usually at the temporal poles.

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Case information

rID: 51685
Published: 17th Oct 2019
Last edited: 18th Oct 2019
Inclusion in quiz mode: Included

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