Distinct multi-joint control strategies in spastic diplegia associated with prematurity or Angelman syndrome
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TLDR
The kinematics and kinetics of the squatting movement and associated electromyographic activities in 20 children with spastic diplegia associated with periventricular leucomalacia or Angelman syndrome and 18 unimpaired children were studied, speculating that they reflect corticospinal impairment in SDPL and combined cortICospinal and cerebellar dysfunction in AS.About:
This article is published in Clinical Neurophysiology.The article was published on 2001-09-01 and is currently open access. It has received 36 citations till now. The article focuses on the topics: Spastic diplegia & Body movement.read more
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Behavior and neuropsychiatric manifestations in Angelman syndrome.
TL;DR: The main features of this phenotype, characterized by happy demeanor with prominent smiling, poorly specific laughing and general exuberance, associated with hypermotor behavior, stereotypies, and reduced behavioral adaptive skills despite proactive social contact are reviewed.
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Sleep Disturbances in Neurodevelopmental Disorders
TL;DR: An overview of causes, diagnosis, and treatment of sleep disturbances in neurodevelopmental disorders that present primarily with symptoms of hypersomnia and/or insomnia is provided.
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Are there distinctive sleep problems in Angelman syndrome
TL;DR: Sleep problems in Angelman syndrome reflect abnormal neurodevelopmental functioning presumably involving dysregulation of GABA-mediated inhibitory influences in thalamocortical interactions.
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Fast cerebellar oscillation associated with ataxia in a mouse model of angelman syndrome
TL;DR: In vivo cerebellar activity in a mouse model of Angelman syndrome produced by null mutation of the maternal Ube3a gene is recorded and fast oscillation in the cerebellary cortex sustained by abnormally increased Purkinje cell firing rate and rhythmicity is found.
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Cerebellar network plasticity: From genes to fast oscillation
TL;DR: Recent evidence suggests a relationship between fast oscillation and cerebellar long term depression (LTD), which may have major implications for future therapeutic targeting.
References
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Signal-dependent noise determines motor planning
Chris Harris,Daniel M. Wolpert +1 more
TL;DR: This theory provides a simple and powerful unifying perspective for both eye and arm movement control and accurately predicts the trajectories of both saccades and arm movements and the speed–accuracy trade-off described by Fitt's law.
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The gross motor function measure: a means to evaluate the effects of physical therapy.
TL;DR: Correlations between scores for change on this measure and the judgments of change by parents, therapists and ‘blind’ evaluators supported the hypothesis that the instrument would be responsive to both negative and positive changes.
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‘Puppet’ Children A Report on Three Cases
TL;DR: Three unrelated children are described with similar physical abnormalities of congenital origin, reminiscent of puppet children, and profound mental retardation.
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Imprinted expression of the murine Angelman syndrome gene, Ube3a , in hippocampal and Purkinje neurons
Urs Albrecht,James S. Sutcliffe,Bruce M. Cattanach,Colin V. Beechey,Dawna L. Armstrong,Gregor Eichele,Arthur L. Beaudet,Arthur L. Beaudet +7 more
TL;DR: The major phenotypic features of AS correlate with the loss of maternal-specific expression of Ube3a in hippocampus and cerebellum as revealed in the mouse model.
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Mice devoid of γ-aminobutyrate type A receptor β3 subunit have epilepsy, cleft palate, and hypersensitive behavior
Gregg E. Homanics,Timothy M. DeLorey,Leonard L. Firestone,Joseph J. Quinlan,Adrian Handforth,Neil L. Harrison,Matthew D. Krasowski,Caroline E. Rick,Esa R. Korpi,Esa R. Korpi,Riikka Mäkelä,Riikka Mäkelä,Murray H. Brilliant,Nobuko Hagiwara,Carolyn Ferguson,Kimberly Snyder,Richard W. Olsen +16 more
TL;DR: Hyperactivity, lack of coordination, and seizures in β3-deficient mice are consistent with reduced presynaptic inhibition in spinal cord and impaired inhibition in higher cortical centers and/or pleiotropic developmental defects.