Hemimegalencephaly

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Early neonatal seizures.

Patient Data

Age: 5 days
Gender: Male
Modality: 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 Brain

Modality: CT

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 Brain

Modality: MRI

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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