[Cerebellar cortical dysplasia: MRI aspects and significance]. / Dysplasie du cortex cérébelleux: aspects en IRM et signification.

Because it is now possible to obtain high-resolution multiplanar MR imaging of the cerebellum and because of the developing interest on the role of the cerebellum on higher brain functions, we have decided to study the process of cerebellar fissuration. All brain MRI examinations performed in children for varied neurological and neurosurgical indications, especially children with non-specific mental retardation and patients with cerebral malformation detected at initial imaging work-up, were reviewed. Fissuration and lobulation anomalies (abnormal orientation of fissures, pseudopolymicrogyria, cortical thickening, subcortical cysts and heterotopia) were identified that we called cerebellar cortical dysplasia (CCD). In order to better understand the origin of this malformation, current data on cerebellar embryogenesis and histogenesis will be reviewed, and the pathological and radiological features will be illustrated. Milder forms of CCD represent a distinct group of anomalies that should be distinguished from other types of cerebellar dysplasia (agenesis, hypoplasia or complex dysplasia with involvement of the cerebellar vermis (rhombencephalosynapsis)) or combined cerebellar and cerebral dysplasia (muscular dystrophies and lissencephaly). Recognition of cerebellar cortical dysplasia could be a first step towards a broader understanding of its pathogenesis and significance.

Cerebellar cortical dysplasia: MR findings in a complex entity.

BACKGROUND AND PURPOSE: 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. CONCLUSION: 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.