Antonio Benítez-Burraco , Koji Hoshi , Elliot Murphy
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Language deficits in GRIN2A mutations and Landau–Kleffner syndrome as neural dysrhythmias
We review epilepsy-related aphasias in connection with GRIN2A mutations, focusing on acquired childhood epileptic aphasias such as Landau-Kleffner syndrome (LKS). The spontaneous speech of children with LKS exhibits syntactically simplified utterances, severe word finding difficulties, and severe phonological paraphasias. Characterizing LKS as a neural dysrhythmia, we review how EEG abnormalities typically manifested during non-rapid eye movement (NREM) sleep as electrical status epilepticus are related to sleeping disorders partly caused by GRIN2A mutations. Expanding on speculations originally put forward by Landau & Kleffner, 1957, we explore how neural processes such as sharp-wave ripples, sleep spindles, slow oscillations, and their cross-frequency couplings are necessary for language-related processes which are perturbed in LKS, accounting in part for the linguistic profile of epileptic aphasias.
期刊介绍:
The Journal of Neurolinguistics is an international forum for the integration of the neurosciences and language sciences. JNL provides for rapid publication of novel, peer-reviewed research into the interaction between language, communication and brain processes. The focus is on rigorous studies of an empirical or theoretical nature and which make an original contribution to our knowledge about the involvement of the nervous system in communication and its breakdowns. Contributions from neurology, communication disorders, linguistics, neuropsychology and cognitive science in general are welcome. Published articles will typically address issues relating some aspect of language or speech function to its neurological substrates with clear theoretical import. Interdisciplinary work on any aspect of the biological foundations of language and its disorders resulting from brain damage is encouraged. Studies of normal subjects, with clear reference to brain functions, are appropriate. Group-studies on well defined samples and case studies with well documented lesion or nervous system dysfunction are acceptable. The journal is open to empirical reports and review articles. Special issues on aspects of the relation between language and the structure and function of the nervous system are also welcome.