Longitudinal correspondence of epilepsy and scalp EEG fast (40-200 Hz) oscillations in pediatric patients with tuberous sclerosis complex.

Introduction: Epilepsy associated with tuberous sclerosis complex (TSC) has very complex clinical characteristics. Scalp electroencephalogram (EEG) fast (40-200 Hz) oscillations (FOs) were recently suggested to indicate epilepsy severity. Epileptic FOs may undergo age-dependent longitudinal change in individual patients, however, and the typical pattern of such change is not yet fully clarified.

Action of antiepileptic drugs on neurons.

The recent development of various new antiepileptic drugs (AEDs) has provided a wide range of therapeutic strategies for epilepsy. Information regarding the mechanisms of the action of AEDs is valuable when selecting drugs for individual epilepsy patients. AEDs can be categorized as those acting at the excitatory synapse, at the inhibitory synapse, on the extrasynaptic neuronal membrane, or with multiple or miscellaneous mechanisms of action.

Complex observation of scalp fast (40-150 Hz) oscillations in West syndrome and related disorders with structural brain pathology.

We investigated the relationship between the scalp distribution of fast (40-150 Hz) oscillations (FOs) and epileptogenic lesions in West syndrome (WS) and related disorders. Subjects were 9 pediatric patients with surgically confirmed structural epileptogenic pathology (age at initial electroencephalogram [EEG] recording: mean 7.1 months, range 1-22 months).

Detection of fast (40-150 Hz) oscillations from the ictal scalp EEG data of myoclonic seizures in pediatric patients.

Objective: We explored fast (40-150 Hz) oscillations (FOs) from the ictal scalp electroencephalogram (EEG) data of myoclonic seizures in pediatric patients to obtain insight into the pathophysiological mechanisms involved in the generation of myoclonic seizures.

Subjects And Methods: The participants were 21 children (11 boys, 10 girls; age ranging from 5 months to 17 years 2 months) with myoclonic seizures associated with generalized (poly)spike-wave bursts in the ictal EEG data. The patients had heterogeneous etiologies and epilepsy diagnoses.

[Importance of the multisystem follow-up in patients with tuberous sclerosis complex].

Objective: Tuberous sclerosis complex (TSC) is a multisystem disorder characterized by the formation of hamartoma in multiple organ systems of the body. However, without a well-established cooperative system involving related departments, some organ lesions might be overlooked until symptoms appear or even until the disorder progresses. Therefore, the purpose of this study is to investigate the current status of follow-ups in the TSC patients in the Department of Child Neurology at Okayama University Medical Hospital.

Fast (40-150Hz) oscillations are associated with positive slow waves in the ictal EEGs of epileptic spasms in West syndrome.

Objective: To elucidate the generative mechanisms of epileptic spasms (ESs) in West syndrome, we investigated the temporal relationship between scalp fast (40-150Hz) oscillations (FOs) and slow waves in the ictal electroencephalograms (EEGs) of ESs.

Methods: In 11 infants with WS, ictal FOs were detected in a bipolar montage based on spectral and waveform criteria. Their temporal distribution was analyzed in terms of the positive peaks (trough point, T) of identical EEG data in a referential montage.

Predictive factors for relapse of epileptic spasms after adrenocorticotropic hormone therapy in West syndrome.

Purpose: To investigate whether serial electroencephalographic (EEG) findings can predict relapse of epileptic spasms after synthetic adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS).

Subjects And Methods: Thirty-nine WS patients (8 cryptogenic and 31 symptomatic) were included in this study. These patients received ACTH therapy for the first time and were regularly followed up for more than three years at our hospital.

Occurrence of bilaterally independent epileptic spasms after a corpus callosotomy in West syndrome.

We report a patient with intractable West syndrome whose epileptic spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery.

A storm of fast (40-150Hz) oscillations during hypsarrhythmia in West syndrome.

Objective: Fast oscillations (FOs) were first explored from scalp electroencephalographic (EEG) data from hypsarrhythmia in West syndrome (infantile spasms) to investigate the meaning of FOs in this epileptic encephalopathy.

Methods: In 17 infants with West syndrome, we conservatively detected fast frequency peaks that stood out from the time-frequency spectral background with square root power > 1µV (spectral criterion) and corresponded to clear FOs with at least 4 oscillations in the filtered EEG traces (waveform criterion) in sleep EEGs.

Results: We found a total of 1,519 interictal FOs that fulfilled both the spectral and waveform criteria.

A Japanese adult case of guanidinoacetate methyltransferase deficiency.

Guanidinoacetate methyltransferase (GAMT) deficiency is a rare disorder of creatine synthesis resulting in cerebral creatine depletion. We present a 38-year-old patient, the first Japanese case of GAMT deficiency. Developmental delay started after a few months of age with a marked delay in language, which resulted in severe intellectual deficit.

Questionnaire-based assessment of behavioral problems in Japanese children with epilepsy.

Behavioral problems in Japanese children with epilepsy were investigated by means of a questionnaire for parents consisting of three checklists: the Child Behavior Checklist (CBCL)/4-18 Japanese Edition, the High-Functioning Autism Spectrum Screening Questionnaire (ASSQ), and the Attention-Deficit/Hyperactivity Disorder (ADHD) Rating Scale-IV (ADHD-RS) for parents. The participants were the parents of 108 children aged 6-18 years with apparently normal intelligence. The CBCL indicated abnormal behavior in 10.

Cortical contribution to scalp EEG gamma rhythms associated with epileptic spasms.

The cortical contribution for the generation of gamma rhythms detected from scalp ictal EEG was studied in unique cases of epileptic spasms and a review of the related literature was conducted. Ictal scalp gamma rhythms were investigated through time-frequency analysis in two cases with a combination of focal seizures and spasms and another case with spasms associated with cortical dysplasia. In the two patients with combined seizures, the scalp distribution of ictal gamma rhythms was related to that of focal seizure activity.

Clinical implications of preceding positive spikes in patients with benign partial epilepsy and febrile seizures.

Purpose: To clarify the clinical implications of the preceding positive spikes (PPSs) observed primarily in rolandic spikes, we analyzed PPSs in the rolandic and occipital spikes observed in the electroencephalograms (EEGs) of patients with two types of benign partial epilepsies (benign childhood epilepsy with centro-temporal spikes [BECT] and Panayiotopoulos syndrome [PS]) and febrile seizures (FS).

Subjects And Methods: We identified patients from our outpatient EEG database that were seen between 2006 and 2008 that had BECT, PS, and FS with rolandic or occipital spikes. We generated an averaged spike for each patient from the rolandic and occipital spikes that were detected using an automatic spike detection and clustering system.

[Peculiar involuntary movements in premature babies with specific cerebellar injuries].

We observed characteristic involuntary movements in premature babies during early infancy. These movements consisted of asymmetrical irregular banging of the extremities, similar to chorea, ballisms, or jitteriness. We investigated the clinical characteristics and neuroimaging findings of the patients with these peculiar involuntary movements to clarify their pathophysiological mechanisms and to find a treatment.

Efficacy of topiramate for intractable childhood generalized epilepsy with epileptic spasms: with special reference to electroencephalographic changes.

Purpose: Epileptic spasms (ES) beyond infancy are a highly refractory type of seizures that require the development of an effective treatment. We therefore studied the efficacy and safety of topiramate (TPM), which is a drug that is indicated to be effective for intractable childhood epilepsy, for ES.

Methods: Out of 58 children with ES, we enrolled 33 patients treated with TPM at ≤ 12 years of age.

[Lamotrigine add-on therapy for childhood-onset refractory epilepsy: comparison of the efficacy between 3 months and 6 months after initiation].

We investigated the effect of lamotrigine (LTG) add-on therapy in 50 patients with childhood-onset refractory epilepsy (25 males and 25 females): 15 with localization-related epilepsy, 33 with generalized epilepsy, and 2 with undetermined epilepsy. Twenty-four patients had experienced a period of West syndrome during their clinical course. Age at the start of LTG therapy ranged from 2 years 6 months to 41 years 2 months: <16 years in 43 and > or = 16 years in 7.

Amplitude-integrated EEG colored according to spectral edge frequency.

Objective: To improve the interpretability of figures containing an amplitude-integrated electroencephalogram (aEEG), we devised a color scale that allows us to incorporate spectral edge frequency (SEF) information into aEEG figures. Preliminary clinical assessment of this novel technique, which we call aEEG/SEF, was performed using neonatal and early infantile seizure data.

Methods: We created aEEG, color density spectral array (DSA), and aEEG/SEF figures for focal seizures recorded in seven infants.

Abnormal fast activity before the onset of West syndrome.

In our sequential EEG study performed on 68 infants with various pre- and perinatal brain insults, we found peculiar abnormal fast activity (AFAs) in 12 patients. 9 of the 12 patients with AFAs later developed West syndrome (WS) compared with only 3 of the 56 patients without AFAs (p<0.001, χ(2) test).

[Treatment of adult patients with childhood-onset epilepsy by child neurologists].

A significant number of patients with childhood-onset epilepsy continue to be treated by child neurologists after reaching adulthood. In this article, the issue of these so-called carry-over cases is discussed based on the results of two studies: our previous study on carry-over patients at the Department of Child Neurology, Okayama University Hospital, and a questionnaire survey on carry-over cases by the Japan Epilepsy Society. These two studies revealed that child neurologists currently treat many carry-over epilepsy patients in Japan, and that these patients, even those who have low seizure frequencies or are in remission, suffer from many problems such as comorbidities and socioeconomic difficulties.

Spectral analysis of EEG gamma rhythms associated with tonic seizures in Lennox-Gastaut syndrome.

Purpose: EEG gamma rhythms, which are found in association with epileptic spasms in infants with West syndrome, were explored in the ictal EEGs of tonic seizures in older patients with Lennox-Gastaut syndrome (LGS) to investigate the pathophysiology of the disease.

Methods: The subjects were 20 patients with LGS (11 males, 9 females; age range: 3 years 1 month to 29 years 3 months) who had at least one digitally recorded tonic seizure with minimal artifacts. A time-frequency analysis was applied to each patient's ictal EEG data.

Relation of spasms and myoclonus to suppression-burst on EEG in epileptic encephalopathy in early infancy.

Purpose: This study was intended to clarify the relation between spasms in series and a suppression-burst (SB) EEG pattern which have a common nature of repetitive bursting activity in epileptic encephalopathy in early infancy.

Methods: The ictal EEG traces of spasms were temporally compressed and expanded to study the beginning and ending phases of series along with their spectral analysis in two patients with Ohtahara syndrome (OS) and one with early myoclonic encephalopathy (EME). The EEG bursts associated with myoclonus were also investigated.

Electroencephalographic changes before the onset of symptomatic West syndrome.

To clarify the characteristics of the mode of appearance and morphology of epileptiform discharges before the onset of West syndrome (WS). The subjects were 25 infants whose electroencephalograms (EEGs) were recorded before the onset of WS and whose first EEG was recorded before 6 months of corrected age (CA). We extensively analyzed the chronological and topographical changes of the epileptiform discharges before the onset of WS.

EEG and seizure exacerbation induced by carbamazepine in Panayiotopoulos syndrome.

We report on a 4-year 8-month-old boy with Panayiotopoulos syndrome who showed atypical evolution with newly developed absence seizures and EEG exacerbation induced by carbamazepine. Soon after the introduction of carbamazepine, EEGs began to worsen, and finally absence seizures and myoclonic seizures appeared. Immediately after we discontinued carbamazepine, the seizures disappeared and the EEG improved.

Disruption of nuclear vitamin D receptor gene causes enhanced thrombogenicity in mice.

Vitamin D metabolites influence the expression of various genes involved in calcium homeostasis, cell differentiation, and regulation of the immune system. Expression of these genes is mediated by the activation of the nuclear vitamin D receptor (VDR). Previous studies have shown that a hormonally active form of vitamin D, 1alpha,25-dihydroxyvitamin D3, exerts anticoagulant effects in cultured monocytic cells.