Cerebellar cortical dysplasia: MR findings in a complex entity.
OBJECTIVE
MR imaging findings of cerebellar cortical dysplasia 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 presenting 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 dysplasias (n=8), dysplasia of corpus callosum (n=6), and heterotopia (n=5). A widespread 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 right cerebral hemisphere and lateral ventricle. He also had nodular heterotopia, suggesting unilateral megalencephaly.


CONCLUSIONS
Our study suggests that cerebellar cortical dysplasias are common in cases with more widespread cerebral malformations. Technical progress providing high-quality tridimensional MR imaging of the cerebellum may explain its recent descriptions.
Soto-Ares
G
G
Department of Neuroradiology, Hôpital Roger Salengro, Chru Lille France.
Delmaire
C
C
Deries
B
B
Vallee
L
L
Pruvo
J P
JP
D000293:Adolescent; D061085:Agenesis of Corpus Callosum; D001139:Arnold-Chiari Malformation / Q000175:diagnosis; D001933:Brain Stem / Q000002:abnormalities / Q000473:pathology; D002525:Cerebellar Cortex / Q000002:abnormalities* / Q000473:pathology*; D002531:Cerebellum / Q000002:abnormalities; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003337:Corpus Callosum / Q000473:pathology; D003616:Dandy-Walker Syndrome / Q000175:diagnosis; D005260:Female; D006801:Humans; D021621:Imaging, Three-Dimensional; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies