title: Evidence of a developmental cerebello-cerebral disorder.
abstract: The objective of this study was to examine the cognitive and neuroanatomical features of an adolescent with developmental hypoplastic left cerebellum who presented with executive and visuo-spatial deficits, nonverbal learning disabilities and interpersonal difficulties. He underwent a neuropsychological assessment, MRI and diffusion tensor imaging MRI. The neuropsychological impairments were primarily in executive functions, spatial and visual perception, graphomotor skills, arithmetic, social perception and comprehension. Fractional anisotropy, which is measured by diffusion tensor imaging and enables assessment of axonal integrity, was reduced in the right cerebral peduncle and right hemisphere white matter (p < 0.001). Based on the results, we hypothesize that disruption of neural circuits linking the hypoplastic left cerebellum to the right hemisphere may contribute to the evolution of a neurocognitive syndrome with characteristics of the developmental right hemisphere syndrome and suggestive of the cerebellar cognitive-affective syndrome.
authors.affiliation: Neuropediatric Unit, Shaare Zedek Medical Center, Jerusalem 91031, Israel.
authors.forename: Varda
authors.initials: V
authors.lastname: Gross-Tsur
authors.forename: Dafna
authors.initials: D
authors.lastname: Ben-Bashat
authors.forename: Ruth S
authors.initials: RS
authors.lastname: Shalev
authors.forename: Miriam
authors.initials: M
authors.lastname: Levav
authors.forename: Liat Ben
authors.initials: LB
authors.lastname: Sira
mesh_terms: D000293:Adolescent; D001927:Brain Diseases / Q000150:complications / Q000473:pathology*; D001931:Brain Mapping; D002531:Cerebellum / Q000503:physiopathology*; D002540:Cerebral Cortex / Q000503:physiopathology*; D002658:Developmental Disabilities / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D007859:Learning Disabilities / Q000209:etiology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D010465:Perception / Q000502:physiology; D011340:Problem Solving / Q000502:physiology