Intro
Preface
Contents
Chapter 1: Introduction
References
Part I: Landau-Kleffner Syndrome (LKS)
Chapter 2: History of LKS and Its Epidemiology
2.1 History of LKS
2.2 Epidemiology
References
Chapter 3: Diagnostic Paradigms
3.1 Clinical Symptoms
3.1.1 Onset of the Disease
3.1.1.1 Auditory Verbal and Language Symptoms
3.1.1.2 Musical Abilities
3.1.2 LKS Patients' Subjective Awareness of Their Own Disease
3.1.3 Behavioral Dysfunctions
3.1.4 Family History
3.1.5 Physical and Neuropsychological Examinations

3.1.6 Intellectual Ability or IQs (Intellectual Quotients) in LKS Patients
3.1.7 Epileptic Seizures
3.2 Electroencephalography (EEG)
3.3 Audiometric Findings in LKS Patients
3.4 Evoked Responses in LKS Patients
3.4.1 Auditory Brainstem Responses (ABR)
3.4.2 Slow Vertex Responses (SVR)
3.4.3 Combined Evoked Potentials (EPs) and Event-Related Responses (ERPs) in LKS Patients
3.5 Event-Related Potentials
3.5.1 Mismatched Negativity (MMN)
3.5.2 The P300 Evoked Potential
3.6 Blood Exams, Urinalysis, and CSF Measures
3.7 Neuroimaging Examinations

3.7.1 SPECT and PET Scan
3.8 Magnetoenechalography (MEG)
References
Chapter 4: Etiology and Genetics
4.1 Morphological Approach
4.1.1 Gross Anatomical Approach
4.1.2 Microscopic Approach
4.2 Present Status in Delineating the Etiology of LKS
4.2.1 LKS and Autism Spectrum Disorder (ASD)
4.2.1.1 Autistic Regression and Language Regression
4.3 Current Genetic Research
References
Chapter 5: Treatment and Long-Term Prognosis
5.1 Epilepsy
5.2 Surgical Interventions in the Treatment of LKS
5.3 Speech and Language Training
References

Chapter 6: Case Histories
6.1 Patient A: 4 Years Old, Female
6.2 Patient B: 4 Years Old, Female
6.3 Patient C: 6 Years Old, Male
6.4 Patient D: 5 Years Old, Female
6.5 Patient E: 8 Years Old, Male
6.6 Patient F: 14 Years Old, Male
6.7 Patient G: 8 Years Old, Male
6.8 Patient H: 8 Years Old, Male
6.9 Patient I: 10 Years Old, Female
Chapter 7: Fifty Years in the History of Landau-Kleffner Syndrome (LKS)
Part II: Related Central Auditory Disorders
Chapter 8: Adrenoleukodystrophy (ALD)
8.1 Etiology and Clinical Course of the Disease

8.2 Clinical Types of ALD and Their Signs and Symptoms
8.3 Diagnosis of ALD
8.4 Hearing Impairment in ALD
8.5 Evaluation of Hearing in ALD
8.6 Case Reports of Japanese Patients with ALD and Auditory Agnosia (Furushima et al. 2015)
8.6.1 Case Histories of Three Patients
8.6.1.1 Patient 1
8.6.1.2 Patient 2
8.6.1.3 Patient 3
8.6.2 MRIs of the Above Patients
8.6.2.1 Patient 1 (Fig. 8.4a)
8.6.2.2 Patient 2 (Fig. 8.4b)
8.6.2.3 Patient 3 (Fig. 8.4c)
8.6.3 Audiograms
8.6.4 ABR Findings
8.6.5 SVR and MMN
8.7 Summary
8.7.1 Hearing Impairment