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Angelman syndrome is characterized by developmental delay, absence of speech, motor impairment, epilepsy, and a peculiar behavioral phenotype with happy demeanor. It is caused by lack of expression of the UBE3A gene, which can result from various abnormalities of chromosome 15q11-q13. Interictal high-amplitude rhythmic electroencephalographic (EEG) patterns are distinctive and should be differentiated from epileptic activity. Patterns of seizures, including type, age of onset, other clinical features, and EEG features of patients with Angelman syndrome may show some resemblance to defined epileptic syndromes. Correct characterization of their epilepsy is therefore important for both management and prognosis. Surveys of antiepileptic drugs used in patients with Angelman syndrome have suggested that valproic acid is the most commonly used. Non-pharmacological management is rarely considered, despite the relatively high prevalence of drug resistance. Ketogenic diet has been effective in a few cases.
Neurophysiological studies of sleep have increasingly focused on underlying dynamic processes. This would appear particularly relevant to the developmental aspects of sleep. Involvement of sleep-dependent mechanisms in emotional processing, as well as perceptual-sensory, perceptual-motor, and cognitive learning, mostly studied in adults, may play major roles in development. Rapid eye movement (REM) sleep, which is generated by complex neuronal interactions within the pontine reticular formation, and non-REM sleep, which arises from activities in the thalamocortical network, are specifically implicated in different aspects of long-term memory systems. They evolve from previous physiological and behavioural states which can be traced back to the fetal period. Further studies are needed to clearly identify functions reflected by hallmarks of sleep stages, such as spindles and K complexes. A better understanding of the maturational aspects of sleep should provide important insights into physiological development. Assessment approaches taking dynamic characteristics of sleep into account may contribute to the design of better targeted management of sleep-related problems in neurodevelopmental conditions.
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