The Epilepsy-Aphasia Spectrum: From Landau–Kleffner Syndrome to Rolandic Epilepsy
19 June 2017 (eFirst)
Thierry Deonna and Elaine Roulet-Perez (with contributions from Xavier De Tiège, Serge Goldmann, and Patrick Van Bogaert). The Epilepsy-Aphasia Spectrum: From Landau–Kleffner Syndrome to Rolandic Epilepsy (Clinics in Developmental Medicine). London, UK: Mac Keith Press, 2016 (200 pages). ISBN 978–1-909962–76–7. £ 50.00/EUR 62.50
Thierry Deonna and Eliane Roulet-Perez, two wholehearted clinicians who dedicated an important part of their scientific work to Landau–Kleffner syndrome (LKS) and related syndromes discuss in this volume all facets of the epilepsy-aphasia spectrum (EAS) with a special emphasis on LKS. A chapter on functional imaging written by X. De Tiège, S. Goldmann, and P. van Bogaert completes this book.
The book is organized into 15 chapters. The introduction gives an outlook pointing out the importance of LKS and EAS as a model for the impact of epilepsy on developing higher cortical functions. In chapter 2, the history of LKS is described. It starts with the first description by Landau and Kleffner in 1957, who already suggested that epileptic discharges might lead to a functional ablation of areas necessary for normal linguistic behavior. After an odyssey through various explanations of the syndrome, it ends with the confirmation of the original suggestion that indeed epileptic dysfunction is the main or the only factor leading to aphasia in LKS. Important issues related to classification are addressed in chapter 3, which helps to better understand the literature on LKS and related syndromes. “Speech perception and brain organization of language: relevant features for LKS and the EAS” (chapter 4) is an excursion into the world of language with theoretical models, functional neuroanatomy, and an attempt to apply these models to LKS. In chapter 5, different clinical facets of LKS, including examples of different types of aphasia, deficits in written language, and musical (dis)ability—just to name some—are thoroughly illustrated with a personal note, underpinned with numerous impressive case reports. Chapter 6 discusses the value of electroencephalogram (EEG) in the assessment of children with developmental language delay and autism spectrum disorder. It provides recommendations on how to investigate these children, given the lack of evidence-based guidelines. Chapter 7 delineates the very variable courses of LKS, again with many exemplary case reports. It is followed by chapter 8 dedicated to Rolandic epilepsy and other idiopathic focal epilepsies of childhood within the perisylvian region and beyond. Besides clinical pictures, distinctive anatomical features and genetics are discussed here. Chapter 9 outlines possible investigations in EAS including electrophysiology, imaging, immunological/inflammatory parameters, and genetics. “Physiopathology of speech, language, and other prolonged epileptic dysfunction in LKS, epilepsy with continuous spike-waves during sleep (CSWS) and related syndromes” (chapter 10) gives an overview on currently discussed hypotheses about how epileptic discharges might have a negative impact on cortical function. This directly leads over to the chapter written by De Tiège, Goldmann, and van Bogaert who thoroughly explain how various functional imaging technics (fluorodeoxyglucose-positron emission tomography, functional magnetic resonance imaging) and their combination with EEG/magnetoencephalography may contribute to the more profound understanding of the interaction between epileptic discharges in sleep and neuropsychological deficits. The following chapter 12 is dedicated to drug (and surgical) treatments of cognitive impairments in EAS, whereas chapter 13 considers educational and psychological remediation and here most importantly the possible benefit of sign language. The last chapter before the summary gives patients a voice to tell their testimonies.
The richness of illustrative case reports is one of the big merits of this volume. While the book is not written to serve as a quick reference or short review, it rather invites the reader to dive into the subject and to benefit from the author's broad clinical experience and overview of the literature.
In summary, this “tour de LKS” is not only useful for professionals dealing with LKS but also an enrichment for everybody interested in childhood epilepsy and especially in the relationship between epilepsy, neurological, and developmental aspects of language, cognition, and sleep. It is equally attractive for clinically as well as scientifically oriented child neurologists, speech, and language therapists, psychiatrists, neuropsychologists, and neurobiologists.