Brain & development
-
Brain & development · Mar 2011
ReviewFilling the evidence gap: how can we improve the outcome of neonatal encephalopathy in the next 10 years?
Neonatal encephalopathy associated with perinatal hypoxia-ischaemia is one of the most common causes of death and permanent disability worldwide. However, of a wide range of "experimentally neuroprotective treatments" invented so far, only therapeutic hypothermia has been promoted into a standard clinical practice. Such a wide gap in the efficacy of neuroprotective treatments between the experimental setting and clinical practice may be attributed to the strategic flaw in translating basic knowledge into clinical care. ⋯ The use of translational large animal models allows the practical simulation and fine-tuning of clinical protocols, which may further assist successful translation of basic knowledge. In addition to the effort to develop alternative therapeutic options, it is important to maximise the effect of the current only neuroprotective option, or therapeutic hypothermia. Independent variables which influence the efficacy of hypothermia have to be elucidated to improve its therapeutic protocol, and to increase the number of patients who will benefit from this treatment.
-
Brain & development · Feb 2010
ReviewLeukodystrophies and other genetic metabolic leukoencephalopathies in children and adults.
Abnormalities of CNS white matter are frequently detected in patients with neurological disorders when MRI studies are performed. Among the many causes of such abnormalities, a large group of rare genetic diseases poses considerable diagnostic problems. ⋯ The table contains essentials such as age at onset of symptoms, clinical and MRI characteristics, basic defect, and useful diagnostic studies. The table serves as a diagnostic check list.
-
Brain & development · Jan 2010
ReviewEpileptic encephalopathy in children possibly related to immune-mediated pathogenesis.
Severe epilepsy in the paediatric population negatively influences neurological and cognitive development. Different etiological factors could be responsible of these severe epilepsies, and an early diagnosis could change, in some cases, the neurological and cognitive development. Immune mechanisms have been reported in epilepsy. ⋯ Among children with acquired symptomatic severe epilepsy, we identified a group of previously normal children who had developed severe partial epilepsy after an acute/sub-acute illness resembling encephalitis. The etiological factors for those patients seems to remain unknown, and a possible immune-mediating or inflammatory process as pathogenesis of the disease could be hypothesized. More studies need to be addressed to finally define this peculiar epileptic entity.
-
Apoptosis occurs physiologically in the mammalian brain during the period of the growth spurt, which in human starts in the 3rd trimester of gestation and ends by the third year of life. Environmental factors can interact with programmed cell death mechanisms to increase the number of neurons undergoing apoptosis and thus produce neuropathological sequelae in the brain. In a series of studies it could be shown that classes of drugs which block N-methyl-D-aspartate (NMDA) glutamate receptors, promote gamma-aminobutyric-acid (GABA(A)) receptor activation or block voltage gated sodium channels, when administered to immature rodents during the period of the brain growth spurt, trigger widespread apoptotic neurodegeneration throughout the developing brain. ⋯ Pathomechanisms involved in the proapoptotic action of sedative and anticonvulsant drugs and oxygen include decreased expression of neurotrophins, inactivation of survival signaling proteins, activation of inflammatory cytokines as well as oxidative stress. These findings raise concerns pertaining to the treatment of infants and young children with sedative and anticonvulsant drugs and premature infants with oxygen. The experimental findings imply that new approaches should be developed for patients within these vulnerable age groups.
-
Brain & development · Aug 2008
Review Meta AnalysisFactors associated with adverse neurodevelopmental outcomes in infants with congenital heart disease.
To review reported neurodevelopmental outcome data for patients with congenital heart disease, identify risk factors for adverse neurodevelopmental sequelae and summarize potential neuromonitoring strategies that have been described. ⋯ As advances in the medical and surgical management improves survival in patients with CHD, increasing knowledge about neurodevelopmental outcomes and the factors that affect them will provide for strategies to optimize long-term outcome in this high-risk population.