Neuropsychological Evaluation and Follow-Up of Children with Cerebellar Cortical Dysplasia.

Aim  To describe neuropsychological disturbances and the developmental course associated with cerebellar cortical dysplasia (CCD). Method  The neuroimaging findings from 10 children (five males, five females; aged 3–10y) with CCD were reviewed and classified. These children all underwent clinical neurological examination and neuropsychological assessment (NPA) on admission, then were followed for an average of 6 years using the cognitive Wechsler Scale, Vineland Adaptive Behavior Scales, and Rey–Osterrieth Complex Figure/McCarthy Drawing subtests. Results  Based on magnetic resonance imaging, CCD was categorized as minor (n=4), moderate (n=1), and severe (n=5). The first NPA disclosed mental retardation* in six (profound, three; moderate, one; mild, two) and normal intelligence in four (low, two; average, one; high, one), but with verbal/performance dissociation in three cases. Socio-adaptive functions were altered in all children except one. Visuospatial abilities were delayed in eight children. In the follow-up, no progression was observed in the three cases with profound mental retardation, whereas the remainder showed homogeneous or disharmonic progression, including improvement or deterioration of verbal/performance function. Cognitive impairment and evolution was not associated with the degree of cerebellar involvement. Interpretation  The neuropsychological profile and evolution associated with CCD do not appear to be predictable, and some features might improve over time.