Article

Spina bifida myelomeningocele (SBM) is a specific type of neural tube defect
whereby the open neural tube at the level of the spinal cord alters brain
development during early stages of gestation. Some structural anomalies are
virtually unique to individuals with SBM, including a complex pattern of
cerebellar dysplasia known as the Chiari II malformation.
Other structural
anomalies are not necessarily unique to SBM, including altered development of the
corpus callosum and posterior fossa. Within SBM, tremendous heterogeneity is
reflected in the degree to which brain structures are atypical in qualitative
appearance and quantitative measures of morphometry. Hallmark structural features
of SBM include overall reductions in posterior fossa and cerebellum size and
volume. Studies of the corpus callosum have shown complex patterns of agenesis or
hypoplasia along its rostral-caudal axis, with rostrum and splenium regions
particularly susceptible to agenesis. Studies of cortical regions have
demonstrated complex patterns of thickening, thinning, and gyrification.
Diffusion tensor imaging studies have reported compromised integrity of some
specific white matter pathways. Given equally complex ocular motor, motor, and
cognitive phenotypes consisting of relative strengths and weaknesses that seem to
align with altered structural development, studies of SBM provide new insights to
our current understanding of brain structure-function associations.

Langue : ANGLAIS