title: Three-year-old child with meroacrania - neurological signs.
abstract: Neurological findings in a three-year-old child with meroacrania provide new insights into how the nervous system develops and functions in the absence of superior levels of control from the time of origin. The girl is the first child of a non-consanguineous white Brazilian couple, born at term, weighing 2650 g and measuring 44 cm in length. Upon examination at 43 months, she had quadriplegia, global hypotonia with occasional body hypertonia in a decorticate posture, hyperreflexia, ankle clonus, and extensor plantar response. This case allowed us to verify that, in the absence of upper structures and subcortical nuclei, there are clear signs that suggest corticospinal primacy in motor functions without a substitute pathway. Sound orientation responses suggest the independence of the vestibular-acoustic-ocular system, and manifestations of responsiveness to the environment raise questions about consciousness.
authors.affiliation: Department of Neurosciences and Behavioral Sciences, School of Medicine of Ribeirão Preto, São Paulo University, Ribeirão Preto - SP, Brazil. carfunay@fmrp.usp.br
authors.forename: Carolina Araújo Rodrigues
authors.initials: CA
authors.lastname: Funayama
authors.forename: Luzia Iara
authors.initials: LI
authors.lastname: Pfeifer
authors.forename: Ester Silveira
authors.initials: ES
authors.lastname: Ramos
authors.forename: Patrícia Zambroni
authors.initials: PZ
authors.lastname: Santucci
authors.forename: Israel
authors.initials: I
authors.lastname: Gomy
authors.forename: Adolfo Marcondes Amaral
authors.initials: AM
authors.lastname: Neto
mesh_terms: D000757:Anencephaly / Q000473:pathology* / Q000503:physiopathology*; D002675:Child, Preschool; D003243:Consciousness; D005260:Female; D006801:Humans; D009122:Muscle Hypertonia; D011187:Posture; D011247:Pregnancy; D011782:Quadriplegia; D012021:Reflex, Abnormal