European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
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Eur. J. Paediatr. Neurol. · Sep 2012
Beyond the Burke-Fahn-Marsden Dystonia Rating Scale: deep brain stimulation in childhood secondary dystonia.
Deep brain stimulation is now widely accepted as an effective treatment for children with primary generalized dystonia. More variable results are reported in secondary dystonias and its efficacy in this heterogeneous group has not been fully elucidated. Deep brain stimulation outcomes are typically reported using impairment-focused measures, such as the Burke-Fahn-Marsden Dystonia Rating Scale, which provide little information about function and participation outcomes or changes in non-motor areas. The aim is to demonstrate that in some cases of secondary dystonia, the sole use of impairment level measures, such as the Burke-Fahn-Marsden Dystonia Rating Scale, may be insufficient to fully evaluate outcome following deep brain stimulation. ⋯ Sole use of impairment-focused measures, are limited in scope to evaluate outcome following deep brain stimulation, particularly in secondary dystonias. Systematic study of effects across multiple dimensions of disability is needed to determine what deep brain stimulation offers patients in terms of function, participation, care, comfort and quality of life. Deep brain stimulation may offer meaningful change across multiple domains of functioning, disability and health even in the absence of significant change in dystonia rating scales.
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Eur. J. Paediatr. Neurol. · Sep 2012
Upper limb impairments and their impact on activity measures in children with unilateral cerebral palsy.
Insights in upper limb impairments and their relationship with activity measures in children with unilateral cerebral palsy (CP) are important to optimize treatment interventions. ⋯ Classification according to MACS and timing of lesion is important to differentiate within the wide range of impairments and activity limitations. In children with congenital lesions, unimanual capacity and bimanual performance are highly determined by distal strength, supporting the additional use of impairment-based interventions.
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Eur. J. Paediatr. Neurol. · May 2012
Arm and hand function in children with unilateral cerebral palsy: a one-year follow-up study.
In children with unilateral cerebral palsy (CP), development of arm and hand function is often compromised by the underlying motor and sensory impairments. However, knowledge about the evolution of arm and hand function in this population is limited. ⋯ Motor impairments, movement quality and hemiplegic hand use in bimanual tasks do not spontaneously improve over one year, except for an age-related change in grip strength. However, an improvement was observed in manual dexterity, suggesting that some children can learn more adaptive movement strategies.
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Eur. J. Paediatr. Neurol. · Mar 2012
ReviewA diagnostic algorithm for the evaluation of early onset genetic-metabolic epileptic encephalopathies.
Early onset epileptic encephalopathies represent a struggling challenge in neurological clinical practice, mostly in infants and very young children, partly due to an unclear and still debated cathegorization. In this scenario genetic and metabolic epileptic encephalopathies play a central role, with new entries still needing an arrangement. ⋯ Starting from this classification we suggest a possible simplified diagnostic algorithm, discussing main decision making nodes in practical patients management. The aim of the proposed algorithm is to guide through metabolic and molecular-genetic work up and to clarify "where" and "what" to search in biochemical, electroencephalographic and neuroimaging investigations.
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Eur. J. Paediatr. Neurol. · Mar 2012
Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: insights from a retrospective case series.
Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a recognized sequel of febrile partial status in children younger than 4 years. ⋯ In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.