Handbook of clinical neurology
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Whereas there is no specific neurosurgical technique in pediatric epilepsy, the frequency of each type of surgery is very different from epilepsy surgery applied in adults, and reflects the underlying etiologies, which are much more diverse in children, with malformations of cortical development and tumors as the prevailing etiologies. Extensive resective or disconnective procedures for extratemporal epilepsy are more frequently performed in infants and younger children, whereas temporo-mesial resection is by far the most common surgical treatment for adults with epilepsy. More recently, less invasive techniques in children with an extensive epileptogenic zone, such as multilobar disconnection, hemispherotomy and other functional hemispherectomy variants, have been introduced in order to reduce duration of surgery, perioperative morbidity and length of hospital stay. ⋯ This development has been encouraged with the introduction of image-guided navigation systems for the preoperative planning and during surgery. Historically, epilepsy surgery for children has been established much later than for adults. Apart from the particular aspects in perioperative management of younger infants, surgery-related morbidity as well as seizure outcome is in general similar to those in adults, depending rather on each type of surgery.
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The diagnosis of brain death should be based on a simple premise. If every possible confounder has been excluded and all possible treatments have been tried or considered, irreversible loss of brain function is clinically recognized as the absence of brainstem reflexes, verified apnea, loss of vascular tone, invariant heart rate, and, eventually, cardiac standstill. This condition cannot be reversed - not even partly - by medical or surgical intervention, and thus is final. ⋯ Generally, the concept of brain death has been accepted by all major religions. But patients' families may have different ideas and are mostly influenced by cultural attitudes, traditional customs, and personal beliefs. Suggestions are offered to support these families.
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Stroke is the major cause of long-term disability worldwide, with impaired manual dexterity being a common feature. In the past few years, noninvasive brain stimulation (NIBS) techniques, such as transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS), have been investigated as adjuvant strategies to neurorehabilitative interventions. These NIBS techniques can be used to modulate cortical excitability during and for several minutes after the end of the stimulation period. ⋯ Differential modulation of cortical excitability in the affected and unaffected hemisphere of patients with stroke may induce plastic changes within neural networks active during functional recovery. The aims of this chapter are to describe results from these proof-of-principle trials and discuss possible putative mechanisms underlying such effects. Neurophysiological and neuroimaging changes induced by application of NIBS are reviewed briefly.
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Neurorehabilitation is based on the assumption that motor learning contributes to motor recovery after injury. However, little is known about how learning itself is affected by brain injury, how learning mechanisms interact with spontaneous biological recovery, and how best to incorporate learning principles into rehabilitation training protocols. Here we distinguish between two types of motor learning, adaptation and skill acquisition, and discuss how they relate to neurorehabilitation. ⋯ The emphasis in current neurorehabilitation practice is on the rapid establishment of independence in activities of daily living through compensatory strategies, rather than on the reduction of impairment. Animal models, however, show that after focal ischemic damage there is a brief, approximately 3-4-week, window of heightened plasticity, which in combination with training protocols leads to large gains in motor function. Analogously, almost all recovery from impairment in humans occurs in the first 3 months after stroke, which suggests that targeting impairment in this time-window with intense motor learning protocols could lead to gains in function that are comparable in terms of effect size to those seen in animal models.
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Epilepsies associated with inborn errors of metabolism (IEM) represent a major challenge. Seizures rarely dominate the clinical presentation, which is more frequently associated with other neurological symptoms, such as hypotonia and/or cognitive disturbances. Although epilepsy in IEM can be classified in various ways according to pathogenesis, age of onset, or electroclinical presentation, the most pragmatic approach is determined by whether they are accessible to specific treatment or not. ⋯ Folinic acid-dependent seizures are allelic with pyridoxine dependency. Incompletely treatable IEMs include pyridoxal phosphate, serine, and creatine deficiencies. The main IEMs that present with epilepsy but offer no specific treatment are nonketotic hyperglycinemia, mitochondrial disorders, sulfite oxidase deficiency, ceroid-lipofuscinosis, Menkes disease, and peroxisomal disorders.