The Epilepsy Aphasia Spectrum

Landau-Kleffner syndrome (LKS) is a rare childhood neurological syndrome. It is characterised by a sudden or gradual loss of ability to understand of express language (aphasia) and usually occurs in children between the ages of 3 and 7 years. Rolandic epilepsy is the most common epilepsy syndrome in childhood with involvement of the oropharyngeal manifestations, hypersalivation and arrest of speech. These two syndromes are considered to be aspects of the ‘epilepsy-aphasia spectrum’ with common rolandic epilepsy being the mildest form and LKS the most severe.

Authors’ Appointments vii Forewords ix Preface xi Acknowledgements xiii 1. Introduction 1 2. History of the Landau–kleffner Syndrome 4 3. Classification Issues 18 4. Speech Perception and Brain Organization of Language: Relevant Features for Landau–kleffner Syndrome and The Epilepsy-aphasia Spectrum 26 5. The Different Clinical Facets of Landau–kleffner Syndrome 34 6. Developmental Aspects of Landau–kleffner Syndrome and The Epilepsy-aphasia Spectrum and The Overlap with Developmental Language Disorders and Autism Spectrum Disorder 60 7. Evolution of Landau–kleffner Syndrome: Short-,mid- and long-term Outcomes 70 8. From Rolandic Epilepsy to Landau–kleffner Syndrome 78 9. Laboratory Investigations in Landau–kleffner Syndrome and The Epilepsy-aphasia Spectrum 95 10. Physiopathology of Speech, Language and Other Prolonged Epileptic Dysfunction in Landau–kleffner Syndrome, Epilepsy with Continuous Spike Waves During Sleep and Related Syndromes 106 11. Functional Neuroimaging Investigations in Idiopathic Focal Epilepsies of Childhood with Cognitive and Behavioural Impairment 119 Xavier de Tiège, Serge Goldman, and Patrick van Bogaert 12. Drug Management of Cognitive Impairments in Landau–kleffner Syndrome and Other Epilepsy Aphasia Spectrum Syndromes 127 13. Speech and Language, Educational and Psychological Remediation 137 14. Personal Testimonies and Unpublished Cases 156 15. Summary and Conclusions 167 References 170 Index 187