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The EEG in Degenerative Disorders of the Central Nervous System: Congenital Malformations, Neurocutaneous Disorders, Inherited Disorders of Metabolism, Cerebral Palsy, and Autism Spectrum Disorders 

The EEG in Degenerative Disorders of the Central Nervous System: Congenital Malformations, Neurocutaneous Disorders, Inherited Disorders of Metabolism, Cerebral Palsy, and Autism Spectrum Disorders
Chapter:
The EEG in Degenerative Disorders of the Central Nervous System: Congenital Malformations, Neurocutaneous Disorders, Inherited Disorders of Metabolism, Cerebral Palsy, and Autism Spectrum Disorders
Author(s):

John Gaitanis

, Phillip L. Pearl

, and Howard Goodkin

DOI:
10.1093/med/9780190228484.003.0013
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date: 15 October 2019

Nervous system alterations can occur at any stage of prenatal or postnatal development. Any of these derangements, whether environmental or genetic, will affect electrical transmission, causing electroencephalogram (EEG) alteration and possibly epilepsy. Genetic insults may be multisystemic (for example, neurocutaneous syndromes) or affect only the brain. Gene mutations account for inborn errors of metabolism, channelopathies, brain malformations, and impaired synaptogenesis. Inborn errors of metabolism cause seizures and EEG abnormalities through a variety of mechanisms, including disrupted energy metabolism (mitochondrial disorders, glucose transporter defect), neuronal toxicity (amino and organic acidopathies), impaired neuronal function (lysosomal and peroxisomal disorders), alteration of neurotransmitter systems (nonketotic hyperglycinemia), and vitamin and co-factor dependency (pyridoxine-dependent seizures). Environmental causes of perinatal brain injury often result in motor or intellectual impairment (cerebral palsy). Multiple proposed etiologies exist for autism, many focusing on synaptic development. This chapter reviews the EEG findings associated with this myriad of pathologies occurring in childhood.

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