Hemimegalencephaly

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Early neonatal seizures.

Patient Data

Age: 5 days
Gender: Male
Ultrasound

Transcranial ultrasound of the brain revealed enlargement of the left lateral ventricle. No evidence of germinal matrix haemorrhage, no intraventricular haemorrhage or hypoxic ischemic insult.

CT

CT Brain

CT examination of the brain shows enlarged dysplastic left cereberal hemisphere with gross cortical dysplasia and enlargement of the left lateral ventricle. Cavum septum pellucidum and cavum verge is noted as a normal variant. A right parietooccipital subgaleal cephalohaematoma is seen mostly related to ventose application. 

MRI

MRI Brain

Hamartomatous over-growth of the entire left cerebral hemisphere is noted associated with cortical dysplasia (agyria / pachygyria) with a broadening of the cortical sulci and shallow gyri & enlargement of the ipsilateral left lateral ventricle with left lateral ventricle small focal subependymal heterotopia. Contralateral displacement of the posterior falx with slight contralateral left-to-right midline shift is noted. Associated calcification of the left inferior frontal deep white matter is noted as well as a small left posterior frontal developmental venous anomaly (DVA).

A small right parieto-occipital subgaleal cephalohaematoma is seen.    

Normal right cerebral hemisphere.    

Normal cerebellum, brain stem and cervico-medullary junction.    

 

Case Discussion

A case of hemimegalencephaly of the left cerebral hemisphere with cortical dysplasia or malformation (agyria / pachygyria), deep white matter calcifications as well as  subependymal grey matter heterotopia & small DVA.

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Case information

rID: 40741
Case created: 1st Nov 2015
Last edited: 9th Jan 2016
Inclusion in quiz mode: Included

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