Cerebellar motor function in spina bifida meningomyelocele.
Spina bifida meningomyelocele (SBM), a congenital neurodevelopmental disorder, involves dysmorphology of the cerebellum, and its most obvious manifestations are motor deficits. This paper reviews cerebellar neuropathology and motor function across several motor systems well studied in SBM in relation to current models of cerebellar motor and timing function. Children and adults with SBM have widespread motor deficits in trunk, upper limbs, eyes, and speech articulators that are broadly congruent with those observed in adults with cerebellar lesions. The structure and function of the cerebellum are correlated with a range of motor functions. While motor learning is generally preserved in SBM, those motor functions requiring predictive signals and precise calibration of the temporal features of movement are impaired, resulting in deficits in smooth movement coordination as well as in the classical cerebellar triad of dysmetria, ataxia, and dysarthria. That motor function in individuals with SBM is disordered in a manner phenotypically similar to that in adult cerebellar lesions, and appears to involve similar deficits in predictive cerebellar motor control, suggests that age-based cerebellar motor plasticity is limited in individuals with this neurodevelopmental disorder.
Dennis
Maureen
M
Program in Neurosciences and Mental Health, Department of Psychology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. maureen.dennis@sickkids.ca
Salman
Michael S
MS
Juranek
Jenifer
J
Fletcher
Jack M
JM
D002531:Cerebellum / Q000503:physiopathology*; D005121:Extremities / Q000503:physiopathology; D005133:Eye Movements; D006801:Humans; D007858:Learning; D008591:Meningomyelocele / Q000473:pathology* / Q000503:physiopathology*; D008954:Models, Biological; D009068:Movement / Q000502:physiology*; D009434:Neural Pathways / Q000503:physiopathology; D013060:Speech; D016135:Spinal Dysraphism / Q000473:pathology*