Article

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 3-10 y) 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.
CI - (c) The Authors. Developmental Medicine & Child Neurology (c) 2011 Mac Keith
Press.

Langue : ANGLAIS