Objective Approach for Titration of Continuous Positive Airway Pressure in Patients with Obstructive Sleep Apnea Using Daytime Continuous Positive Airway Pressure Titration Based on Respiratory Movement Regularity.

: Continuous positive airway pressure (CPAP) is used to treat patients with obstructive sleep apnea (OSA) and has proven clinical efficacy for this condition. However, the objective method to determine the appropriate CPAP level for treatment is still unclear. Patients with OSA typically exhibit irregular respiratory efforts due to obstruction or narrowing of the upper airway during sleep.

Predictive modeling based on functional connectivity of interictal scalp EEG for infantile epileptic spasms syndrome.

Objective: This study aims to delineate the electrophysiological variances between patients with infantile epileptic spasms syndrome (IESS) and healthy controls and to devise a predictive model for long-term seizure outcomes.

Methods: The cohort consisted of 30 individuals in the seizure-free group, 23 in the seizure-residual group, and 20 in the control group. We conducted a comprehensive analysis of pretreatment electroencephalography, including the relative power spectrum (rPS), weighted phase-lag index (wPLI), and network metrics.

Potentiality of Evaluating Flash Electroretinography with a Montage of Skin Electrodes Connecting the Inner Canthus to the Ear in Younger Children: A Pilot Study.

This study aimed to determine whether more stable electroretinography parameters could be obtained in younger children using skin electrodes placed at the inner canthus and ears compared to using contact lens electrodes. The study included three Japanese patients (two girls and one boy) who presented with epileptic spasms before the age of 3 years. We compared the electroretinography parameters obtained with skin electrodes and contact lens electrodes.

Phase Lag Analysis Scalp Electroencephalography May Predict Seizure Frequencies in Patients with Childhood Epilepsy with Centrotemporal Spikes.

Background: Childhood epilepsy with centrotemporal spikes (CECTS) is the most common epilepsy syndrome in school-aged children. However, predictors for seizure frequency are yet to be clarified using the phase lag index (PLI) analyses. We investigated PLI of scalp electroencephalography data at onset to identify potential predictive markers for seizure times.

Quantitative pretreatment EEG predicts efficacy of ACTH therapy in infantile epileptic spasms syndrome.

Objective: This study aimed to determine the correlation between outcomes following adrenocorticotrophic hormone (ACTH) therapy and measurements of relative power spectrum (rPS), weighted phase lag index (wPLI), and graph theoretical analysis on pretreatment electroencephalography (EEG) in infants with non-lesional infantile epileptic spasms syndrome (IESS).

Methods: Twenty-eight patients with non-lesional IESS were enrolled. Outcomes were classified based on seizure recurrence following ACTH therapy: seizure-free (F, n = 21) and seizure-recurrence (R, n = 7) groups.

Predicting Mathematical Learning Difficulties Using Fundamental Calculative Ability Test (FCAT).

Background: Mathematical learning difficulty (MLD) during school years results from several factors, including dyscalculia. Traditional diagnostic tests for dyscalculia are time intensive and require skilled specialists. This prospective cohort study aimed to reveal that the less time intensive Fundamental Calculative Ability Test (FCAT), administered in first grade, can predict the outcome of mathematical school achievement, which was measured with the curriculum-based mathematical test for second grade (1.

Power and connectivity changes on electroencephalogram in postoperative cerebellar mutism.

Introduction: Cerebellar mutism syndrome is a debilitating postoperative neurological complication following posterior fossa surgery in children. It is characterized by a significant lack or loss of speech. Injury to the dentato-thalamo-cortical pathway is thought to be the main anatomical substrate of cerebellar mutism syndrome; however, few studies have investigated the physiological changes using computed electroencephalogram.

Influence of gender on diaphragm thickness using a method for determining intima media thickness in healthy young adults.

Background: To clarify the differences in diaphragm thickness between male and female participants in healthy young adults with ultrasonography using the mean intima media thickness (IMT) method and to investigate the relationship between diaphragm thickness and respiratory pressure.

Methods: Twenty-nine healthy individuals (16 females and 13 males) participated in the study. Diaphragm thickness was measured at total lung capacity (TLC) and at functional residual capacity (FRC) in each participant.

Gait disturbance in a patient with de novo 1.0-kb SOX2 microdeletion.

Background: Sex-determining region Y-box 2 (SOX2) plays an important role in the early embryogenesis of the eye, forebrain, and hypothalamic-pituitary axis. Anophthalmia, microphthalmia, and hormonal abnormalities are commonly observed in patients with SOX2-related disorders. Although gait disturbance, particularly ataxic gait, has recently been observed in several cases, detailed data regarding the clinical course of gait disturbance in SOX2-related disorders are limited.

Phase Lag Analyses on Ictal Scalp Electroencephalography May Predict Outcomes of Corpus Callosotomy for Epileptic Spasms.

We aimed to clarify the patterns of ictal power and phase lag among bilateral hemispheres on scalp electroencephalography (EEG) recorded pre-operatively during epileptic spasms (ESs) and the correlation with the outcomes following corpus callosotomy. We enrolled 17 patients who underwent corpus callosotomy for ESs before 20 years of age. After corpus callosotomy, seven patients did not experience further ESs (favorable outcome group), and the remaining 10 patients had ongoing ESs (unfavorable outcome group).

Frequent epileptic apnoea in a patient with Pitt-Hopkins syndrome.

Pitt-Hopkins syndrome is a rare genetic disease, characterised by severe intellectual disability, distinctive dysmorphic features, epilepsy and distinctive breathing abnormalities during wakefulness. Here, we describe the case of a 22-year-old woman with Pitt-Hopkins syndrome who presented with intractable generalised tonic seizures from the age of 11 years, which increased in frequency with age and onset of menstruation despite usage of some anticonvulsant drugs. From the age of 16 years, polysomnography and video-EEG led to the detection of frequent epileptic apnoea during sleep.

Olfactory function deteriorates in patients with Parkinson's disease complicated with REM sleep behavior disorder.

Introduction: It is not concluded whether the association between olfactory dysfunction and REM sleep behavior disorder (RBD) were worsen cognitive function in patients with Parkinson's disease (PD). We sought to evaluate the impact of these symptoms in PD.

Methods: We examined 62 patients with PD using an olfactory test (Odor Stick Identification Test for Japanese: OSIT-J) and polysomnography (PSG).

Insufficient Efficacy of Corpus Callosotomy for Epileptic Spasms With Biphasic Muscular Contractions.

Corpus callosotomy (CC) is the surgical strategy for drug-resistant epileptic seizures including epileptic spasms (ES). In this study we report a subtype of ES which is accompanied by two consecutive muscular contractions. This subtype has not been previously classified and may emerge via a complex epileptic network.

Symmetry of ictal slow waves may predict the outcomes of corpus callosotomy for epileptic spasms.

We aimed to analyse the ictal electrographic changes on scalp electroencephalography (EEG), focusing on high-voltage slow waves (HVSs) in children with epileptic spasms (ES) and tonic spasms (TS) and then identified factors associated with corpus callosotomy (CC) outcomes. We enrolled 17 patients with ES/TS who underwent CC before 20 years of age. Post-CC Engel's classification was as follows: I in 7 patients, II in 2, III in 4, and IV in 4.

Atypical gamma functional connectivity pattern during light sleep in children with attention deficit hyperactivity disorder.

Introduction: We examined functional connectivity analyses in electroencephalograms (EEGs) of patients with attention deficit hyperactivitydisorder (ADHD) and in those of typically developing children (TDC) to uncover neurobiological abnormalities.

Methodology: We enrolled 31 children with ADHD (mean age 11.1 years; 23 boys) and 17 sex-, age-, and intelligence-matched TDC to undergo 19-channel EEGs during light sleep.

High-frequency component in flash visual evoked potentials in type 3 Gaucher disease.

Objective: To characterize the visual evoked potentials (VEP) in patients with type 3 Gaucher disease (GD) with or without progressive myoclonus epilepsy.

Methods: Three young adults with progressive myoclonus epilepsy (type 3a GD) and two children without progressive myoclonus epilepsy (type 3b GD) were enrolled. Flash visual and somatosensory evoked potentials (F-VEP and SEP, respectively) were retrospectively reviewed in all patients under enzyme replacement therapy.

Developing a mouse model of acute encephalopathy using low-dose lipopolysaccharide injection and hyperthermia treatment.

Acute encephalopathy (AE), mainly reported in East Asia, is classified into four categories based on clinical and neuropathological findings. Among them, AE caused by cytokine storm is known as the severest clinical entity that causes cerebral edema with poor prognosis. Because suitable and convenient model animal of AE had not been developed, the treatment of patients with AE is not established.

Atypical Symptoms in Migraine-Related Alice in Wonderland Syndrome: Expansion of the Phenotype and Reflection on the Pathomechanism.

We report an 8-year-old girl who experienced daily episodes of visual and somesthetic distortion and was diagnosed with Alice in wonderland syndrome (AIWS). Ophthalmologic assessment revealed best-corrected visual acuity of 0.2 in both eyes, and bilateral constricted tubular or spiral visual fields.

Decreased Wave V Amplitude in Auditory Brainstem Responses of Children with Cerebellar Lesions.

Background: This study aims to elucidate the effect of isolated cerebellar lesions sparing the brainstem on the auditory brainstem responses in children.

Methods: We enrolled 10 children (aged 1-16 years) with cerebellar lesions on neuroimaging but lacking clinical brainstem involvement signs and with normal brainstem volumes on magnetic resonance imaging.

Results: The interpeak latency of waves I and V was normal in 9 patients and was marginally prolonged in 1 patient.

Use of high b value diffusion-weighted magnetic resonance imaging in acute encephalopathy/encephalitis during childhood.

Aim: To determine the use of high b value diffusion-weighted imaging (DWI) in the diagnosis and assessment of acute febrile encephalopathy/encephalitis in childhood.

Subjects And Methods: We enrolled 22 children, for whom we examined DWI with b=1000s/mm, DWI with b=3000s/mm, and apparent diffusion coefficient (ADC) map with b=1000 during the acute phase of febrile encephalopathy/encephalitis. Clinical diagnoses included acute encephalopathy with biphasic seizures and late reduced diffusion (AESD; n=6), clinically mild encephalopathy/encephalitis with a reversible splenial lesion (MERS; n=6), and herpes simplex virus encephalitis (HSE; n=3), unclassified acute encephalopathy/acute encephalitis (n=2); acute encephalitis with refractory, repetitive partial seizures (AERRPS; n=1); other encephalopathy (n=1); infarction (n=1); head injury (n=1); or mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (n=1).

Neurodevelopmental disorders in children with macrocephaly: A prevalence study and PTEN gene analysis.

Purpose: To clarify the relationship between macrocephaly and neurodevelopmental disorders, as well as identify the prevalence of PTEN mutations in autism spectrum disorders with macrocephaly in Japan.

Subjects And Methods: Diagnostic and other medical information of children with macrocephaly younger than 4years (n=93) were collected for analysis. PTEN gene mutation analysis was conducted in another set of 16 macrocephalic individuals aged 3-22years.

Surface electromyogram and muscle ultrasonography for detection of muscle fasciculations in pediatric peripheral neuropathy.

A 12-year-old girl presented with talipes equinus of both legs, attenuation of upper and lower limb tendon reflexes, thermal hyperalgesia, and reduction of vibratory sensation. On clinical examination, muscle twitches of fingers of both hands, as well as the abductor halluces and the dorsal interossei muscles of the right foot were observed. Nerve conduction velocity was significantly declined in the upper and lower extremities.

EEG Power Spectrum Analysis in Children with ADHD.

Background: Attention deficit disorder/hyperactivity disorder (ADHD) is a pathological condition that is not fully understood. In this study, we investigated electroencephalographic (EEG) power differences between children with ADHD and healthy control children.

Methods: EEGs were recorded as part of routine medical care received by 80 children with ADHD aged 4-15 years at the Department of Pediatric Neurology in Tottori University Hospital.

Distinguishing Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion from Prolonged Febrile Seizures by Acute Phase EEG Spectrum Analysis.

Background: To differentiate the features of electroencephalography (EEG) after status epileptics in febrile children with final diagnosis of either febrile seizure (FS) or acute encephalopathy for an early diagnosis.

Methods: We retrospectively collected data from 68 children who had status epilepticus and for whom EEGs were recorded within 120 h. These included subjects with a final diagnosis of FS (n = 20), epileptic status (ES; n = 11), acute encephalopathy with biphasic seizures and late reduced diffusion (AESD; n = 18), mild encephalopathy with a reversible splenial lesion (MERS; n = 7), other febrile encephalopathies (n = 10), hypoxic-ischemic encephalopathy (n = 1), and intracranial bleeding (n = 1).