Advances in neurology最新文献

SE in the pediatric age group presents challenges in diagnosis and management. There is need for renewed consensus on the temporal definition of SE, both clinical and electrographic. SE in children exhibits an age-dependent vulnerability, with genetic predisposition and etiology as determinants of susceptibility. Nonepileptic phenomena may mimic SE. Clinical and electrographic SE in neonates are relatively rare, while serial (clinical and electrographic) and repetitive seizures are more common. Neurometabolic disease, chromosomal disorders, and abnormalities of cortical development are important etiological considerations. Abrupt discontinuation of or an aberrant response to AEDs can also precipitate SE. Metabolic perturbations and toxins can further aggravate the situation. Clinical and experimental data suggest that the longer a seizure lasts, the more difficult it becomes to control, and that seizures can have immediate and long-term adverse consequences on the immature and developing brain. Hence, treatment (usually with a benzodiazepine) should be started early in the clinical course. A trial of pyridoxine, biotin, or folinic acid should be considered in the appropriate clinical setting (e.g., neonates or young infants, in particular). Phenytoin/fosphenytoin and phenobarbital remain important treatment options. Pentobarbital and midazolam are preferred choices in the management of RSE. Once metabolic causes are excluded, children with RSE should be evaluated for surgical treatment early in the clinical course. Clinical guidelines based on best available evidence have to be periodically reviewed. The clinical consequences and management of electrographic SE, especially in the neonate, have to be addressed. Guidelines for continuous (video) EEG monitoring are needed to facilitate this task. AEDs that do not have an adverse effect on the developing brain have to be developed. Our review suggests a continuing need for prospective studies into all aspects of SE in the pediatric age group.

Multiple sclerosis is now recognized as more than simply a disease of inflammation and demyelination in the brain and spinal cord. Conventional MRI has been established as the most important paraclinical tool in the diagnostic assessment of patients with suspected MS, and in the monitoring of treatment efficacy in clinical trials, at least in relapsing disease. Magnetization-transfer, diffusion-weighted MRI, 1H-MRS, and fMRI improve our ability to quantify the pathological changes in MS in vivo. Although we have gained some insight into the disease and are starting to uncover some of the structural and physiological substrates for the disability that develops in MS patients, we are far from understanding what causes MS and how to prevent its progression. Imaging can be used as a tool to better understand the pathophysiology of MS and ultimately improve on the treatment of MS.

Because the changes on MRI likely reflect various aspects of the underlying pathology of multiple sclerosis, MRI outcome measures have become an important component of most MS clinical trials, providing objective, supportive evidence for the clinical endpoints. Although there is currently insufficient evidence to support any single or combination of MRI measures as a fully validated surrogate, it is now generally accepted that if the aim of a new therapy is to prevent relapses, new Gd-enhancing and T2 lesions can be considered an appropriate surrogate outcome measure of relapses, and MRI activity outcomes can be recommended as the primary measure of treatment efficacy.