A higher level of spinal lesion in SBM-H is a marker for more severe anomalous brain development, which is in turn associated with poorer neurobehavioral outcomes in a wide variety of domains that determine levels of independent functioning for these children at home and school.

This study addressed the incidence of Attention-Deficit/Hyperactivity Disorder (ADHD) subtypes in children with spina bifida meningomyelocele and shunted hydrocephalus (SBH) as well as differences in executive functions among these subtypes. Parent rating scales revealed that 31% of the group with SBH could be identified with AD/HD, mostly the Inattentive type (23%). The group with SBH differed from normal controls on cognitive measures of executive functions, but subtype differences were not significant. Multivariate tests showed that children with SBH were rated with greater difficulties on the Behavior Rating Inventory of Executive Function (BRIEF) compared to controls; those with SBH and any subtype of ADHD differed from those with SBH and no ADHD; and those with ADHD (Combined Type) differed significantly from those with ADHD (Predominantly Inattentive Type). Subtype differences on univariate tests in the latter comparison were significant on the BRIEF Inhibit scale, showing more disinhibition in those with SBH and ADHD (Combined Type), but no significant differences were apparent on the BRIEF Sustain, Shift, and Initiate scales. The results show that the incidence of ADHD in children with SBH exceeds the population rate, is represented by problems with inattention rather than with impulsivity and hyperactivity; and that as with non-brain injured individuals, subtype differences in cognitive function remain to more clearly delineated.

The cerebellum is important for perceptual and motor timing in the mature brain, but the timing function of the cerebellum in the immature brain is less well understood. We investigated timing in children with spina bifida meningomyelocele (SB), a neural tube defect that involves cerebellar dysgenesis, and in age-matched controls. Specifically, we studied perceptual timing (judgements of 400 ms duration) and motor timing (isochronous motor tapping); measured cerebellar volumes; and related perceptual and motor timing to each other and to cerebellar volume measurements. Children with SB had impairments in the perception of duration (around 400 ms) but not frequency (around 3000 Hz), showing that their perceptual timing deficit was not a generalized auditory impairment. Children with SB had motor timing deficits on unpaced but not paced isochronous tapping, and their unpaced timing performance was associated with clock variance rather than with motor implementation. Perceptual and motor timing were correlated, suggesting that children with SB have impairments in a central timing mechanism. Children with SB, especially those with upper spinal cord lesions, had significant cerebellar volume reductions in grey and white matter, as well as different regional patterns of grey matter, white matter and CSF. Duration perception was correlated with cerebellar volumes, and the number of valid tapping trials was correlated with cerebellar volumes in the SB group, which data demonstrate structure-function relations between timing and cerebellar volumes.