Hemimegalencephaly: a study of abnormalities occurring outside the involved hemisphere

Hemimegalencephaly is a rare but well-known congenital malformation with ipsilateral enlargement of the hemicerebrum. However, very little is known about changes in structures outside the involved hemisphere in patients with this condition. We investigated morphologic abnormalities occurring outside the affected hemisphere by MR imaging in a large series of patients with hemimegalencephaly.

MR imaging findings for 30 patients with hemimegalencephaly were retrospectively reviewed and evaluated for structures outside the involved hemisphere on routine MR images, such as cranial nerves (I, II, V), brain vessels, subdural and subarachnoid spaces, brain stem, and cerebellum, on both the ipsilateral and contralateral sides.

The ipsilateral olfactory and optic nerves were enlarged in 8 (26.7%) and 1 (3.3%) of the 30 patients, respectively, without enlargement on the contralateral side. No asymmetry was noted in the trigeminal nerves. Asymmetric vascular dilations in the ipsilateral cerebral hemisphere were observed in 12 of the 30 patients (40%), in deep cerebral vessels in 11 patients (36.7%), and in superficial cerebral vessels in 8 patients (26.7%). Ipsilateral brain stem and hemicerebellar asymmetric enlargement was detected in 2 patients (6.7%) and 14 patients (46.7%), respectively. Abnormal cerebellar folia were observed on the ipsilateral side in 6 patients (20%) and on the contralateral side in 3 patients (10%).

Ipsilateral olfactory nerve enlargement, cerebral vascular dilations, cerebellar enlargement, and bilateral or ipsilateral abnormal architecture of the cerebellar folia are often associated with hemimegalencephaly.