G. Hahn (Dresden)  

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Head-Brain and brain nerves  

congenital  

After normal pregnancy and birth in the 39th gestational week, the patient was admitted due to infection. Seizures were observed. Additionally, a 2/6 systolicum was noticed. No skin changes were seen. In sonographic examination, 20 to 21 mm, echogenic rhabdomyomas were seen, which, however, were asymptomatic, and did not change in size.  

The name, "Hemimegalencephaly," is used to describe a hamartous enlargening of one or of parts of the hemisphere. It is associated with defects in the neuronal proliferation, migration and organization. The many different radiological and pathological depictions allow to assume that hemimegalencephaly is heterogenic and includes many different illnesses. The brain can be afflicted by itself, or it can be associated with a hemihypertrophy of the complete (or parts) of the ipsilateral side.  

<- view MRI 1

MRI 1: Skull image (T1-weighted Sequence after contrast): Partial Hemimegalencephaly on the left side of tuberous brain sclerosis. Large left hemisphere with large left lateral ventricle. Thickened, atypically gyrated cortex and subcortical dismyelination (increased signal). In the Foramen of Monro on the right, subependymal nodule and also on the right, subcortical tuberoma.





<- view MRI 2

MRI 2: Skull image (T2-weighted Sequence): Partial Hemimegalencephaly on the left side of tuberous brain sclerosis. Large left hemisphere with large left lateral ventricle. Thickened, atypically gyrated cortex and subcortical dismyelination (decreased signal).


 

Total constellation (Consens)  

Hemimegalencephaly due to other illnesses: Proteus-syndrome, epidermal naevus syndrome, hypomelanosis Ito, Neurofibromatosis Type I  

Often, this develops into hard-to-treat seziures or seizures that are difficult to control by medication. Therefore, a surgical procedure is often performed.  

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Hemimegalencephaly was first described in 1835.  

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Hemimegalencephaly, Epilepsy, seizures, systolicum, heart murmur, rhabdomyoma, hamartous enlargening of the brain, tuberous sclerosis of the brain, child, childhood, pediatric radiology  

G Hahn. Tuberous sclerosis of the brain with hemimegalencephaly. PedRad [serial online] vol 1, no. 12.
URL: www.PedRad.info/?search=20011218135646