Although imperfect, MRI is the best way of distinguishing type 1 lisse
ncephaly from other, less classical lissencephalic malformations. We r
eported a case in which correlation of MRI and neuropathology was poss
ible. Besides the classical radiological features of lissencephaly, i.
e., agyria and excessive thickness of the cortex, an additional sign w
as observed: a thin cortical band, which gave high signal on T2-weight
ed images, represented a paucicellular and partially myelinated band,
1500 mu m thick, lying under the true superficial cortex. This MRI fea
ture could be characteristic of the particular cortical lamination obs
erved in true type 1 lissencephaly.