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Introduction: Encephalopathy with electrical status epilepticus in sleep (ESES) syndrome is a rare epilepsy syndrome of childhood that is characterized by sleep-induced epileptiform discharges and problems with cognition or behavior. The neuropsychiatric symptoms in ESES syndrome, among which the ADHD-like symptoms are prominent, bear a close resemblance to symptoms in various developmental disorders. Positive response to adrenocorticotropic hormone (ACTH) is associated with the normalization of the EEG and improvement of neuropsychiatric function. This study aimed to determine the improvement in ADHD-like symptoms in response to ACTH and establish a relationship between improvement in clinical symptoms and EEG parameters., Methods: Seventy-five patients with ESES syndrome, who had clinically displayed ADHD-like symptoms, had been treated with ACTH for ESES, and their medical records were retrospectively reviewed. Sleep EEGs were recorded at referral and follow-up visits, and short courses of ACTH were administered when spike-wave index (SWI) was ≥15%. The assessment of treatment effectiveness was based on reduction in SWI and the clinician-reported improvement in ADHD-like symptoms. Statistical analyses were conducted in order to investigate the relationship between the clinical and EEG parameters., Results: Following treatment with ACTH, a reduction in SWI in all the patients was accompanied by a mean improvement of 67% in ADHD-like symptoms. Disappearance/reduction of foci and cessation/reduction of seizures were achieved in patients with formerly antiepileptic-resistant seizures. Multiple linear regressions established that pretreatment SWI and treatment delay predicted posttreatment SWI, while reduction in SWI, treatment delay, and the presence of foci predicted improvement in ADHD-like symptoms., Discussion: Improvement in ADHD-like symptoms showed high correlation and was timely with the resolution of ESES. It is suggested that ESES and ADHD may be the two different expressions of a common neurobiological abnormality. With enhanced interpretation of sleep EEG, a more thorough assessment and treatment of neurodevelopmental disorders is possible., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: Etiology of stuttering remains unknown and no pharmacologic intervention has been approved for treatment. We aimed to evaluate EEG parameters and the effect of adrenocorticotropic hormone (ACTH) therapy in stuttering., Methods: In this retrospective study, 25 patients with attention deficit and hyperactivity (ADHD) or autism spectrum disorder (ASD), and comorbid stuttering were followed and treated with ACTH for electrical status epilepticus in sleep (ESES). Sleep EEGs were recorded at referral and follow-up visits and short courses of ACTH were administered when spike-wave index (SWI) was ⩾15%. The assessment of treatment effectiveness was based on reduction in SWI, and the clinician-reported improvement in stuttering, and ADHD or ASD. Statistical analyses were conducted in order to investigate the relationship between the clinical and EEG parameters., Results: Following treatment with ACTH, a reduction in SWI in all the patients was accompanied by a 72% improvement in ADHD or ASD, and 83.8% improvement in stuttering. Twelve of the 25 patients with stuttering showed complete treatment response. Linear regressions established that SWI at final visit significantly predicted improvement in ADHD or ASD, and in stuttering. If symptoms had recurred, improvement was once again achieved with repeated ACTH therapies. Stuttering always improved prior to, and recurred following ADHD or ASD., Conclusion: The underlying etiology leading to ESES may play a significant role in the pathophysiology of stuttering and connect stuttering to other developmental disorders. ACTH therapy has beneficial effects on stuttering and improves EEG parameters., (Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Recurrence of infantile spasms (ISs) is common subsequent to treatment with adrenocorticotropic hormone (ACTH) for West syndrome, and prolonged hypsarrhythmia results in psychomotor deterioration. The evolution to hypsarrhythmia involves conversion of prehypsarrhythmic EEG findings to sporadic hypsarrhythmia paroxysms (HPs), and when paroxysms reach a certain frequency, ISs begin to occur. This retrospective chart study aimed to determine the HP threshold frequency after which ISs begin. Recorded either prior (Group A) or subsequent (Group B) to IS relapse, 248 EEGs were examined in 42 patients. The number of HPs in non-rapid eye movement (NREM) sleep divided by NREM duration constituted the countable hypsarrhythmia paroxysms index (cHPI). After reaching a rate of approximately 10/min, the cHPI lost its feasibility due to the merging of HPs. The durational HPI (dHPI) was also calculated (total duration of HPs during NREM/NREM sleep time×100). ACTH treatment was administered if cHPI was ≥2/min, with the aim of preventing relapse. The mean cHPI value without a concomitant spasm relapse (in Group A) was 1.20/min. Following relapse, this value rose to 4.10/min. EEGs performed subsequent to relapse (in Group B) were classified into three subgroups (B1, B2, and B3) according to the duration of the time interval between IS relapse and the succeeding EEG recording. One-way analysis of variance (ANOVA) indicated that cHPI values differed significantly between the Group B subgroups. In subgroups B2 and B3, a higher number of EEGs were evaluated via dHPI. Linear regression analysis established that the interval between recurrence and the succeeding EEG recording significantly predicted cHPI values and accounted for 54.2% of the explained variability in cHPI values. Therefore, use of the cHPI for early recognition and intervention may aid in preventing the onset and recurrence of ISs and further deterioration of psychomotor development., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Attention deficit hyperactivity disorder is a syndrome of unknown etiology that affects 3% to 7% of the population. Although no objective test exists to support the diagnosis, EEG discharges related to the neuropsychiatric abnormalities have a high incidence in attention deficit hyperactivity disorder. The aim of this study was to elucidate the quantitative and qualitative characteristics of EEG abnormality in attention deficit hyperactivity disorder. Nonrapid eye movement sleep EEGs were evaluated in 134 patients with attention deficit hyperactivity disorder in terms of age, EEG timing, spike and wave index, foci, and seizures. All patients had spike and wave paroxysms that changed with age at and time to first EEG. Only half of the patients had seizures and 46 patients had neither seizures nor foci. Thirty-eight patients had the EEG findings of benign focal epilepsy of childhood. Our data support the view that spike and wave activity evolves in time and that EEG discharges are related to neuropsychiatric symptoms even when a diagnosable seizure disorder is absent.