BACKGROUND AND PURPOSE: MR imaging findings of cerebellar cortical dysplasi
a have been described as a new cerebellar malformation. The purpose of this
study was to assess the association of cerebellar cortical dysplasia with
other cerebral malformations.
METHODS: We retrospectively reviewed 46 MR examinations of patients present
ing with developmental delay, hypotonia, and facial deformities to identify
abnormal folia or fissures or both within cerebellar hemispheres or vermis
suggesting cortical dysplasia.
RESULTS: Cerebellar cortical dysplasia was diagnosed in 17 patients. In two
patients, it was isolated. In the remaining 15 patients, the malformation
was associated with vermian malformation (n=11), cerebral cortical dysplasi
as (n=8), dysplasia of corpus callosum (n=6), and heterotopia (n=5). A wide
spread malformation of the posterior fossa was observed in eight patients (
Dandy-Walker, Chiari II and III, and hypoplasia of brain stem). One patient
with hypertrophied cerebellar hemisphere had minor enlargement of the righ
t cerebral hemisphere and lateral ventricle, He also had nodular heterotopi
a, suggesting unilateral megalencephaly.
CONCLUSION: Our study suggests that cerebellar cortical dysplasias are comm
on in cases with more widespread cerebral malformations. Technical progress
providing high-quality tridimensional MR imaging of the cerebellum may exp
lain its recent descriptions.