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The objective of this study was to describe a novel amplitude-integrated electroencephalography (aEEG) pattern in infants with hypoxic–ischemic encephalopathy (HIE) and to assess the clinical significance. The aEEG traces of infants with HIE who were treated with therapeutic hypothermia (TH) from 2012 to 2017 were analyzed. A pseudo-sawtooth (PST) pattern was defined as a periodic increase of the upper and/or lower margin of the trace on aEEG without showing seizure activities on conventional EEG (CEEG). Of the 46 infants, 6 (13%) had the PST pattern. The PST pattern appeared following a flat trace or a continuous low-voltage pattern and was followed by a burst-suppression pattern. On CEEG, the PST pattern consists of alternating cycles of low-voltage irregular activities and almost flat tracing. The PST pattern was associated with neuroimaging abnormalities and with various degrees of neurodevelopmental outcomes. Positive predictive values of the PST or worse pattern for adverse outcomes were high at 12 h after birth. A novel aEEG background pattern in infants with HIE was reported. The PST pattern likely indicates a suppressed background pattern and may be linked to unfavorable outcomes. Further multicenter validation study is needed to clarify its clinical significance.

The epidemiology of patients with traumatic brain injury (TBI) has changed dramatically over recent decades as a result of rapid advances in aging societies. We assessed the influence of age on outcomes of patients with TBI and sought to identify prognostic factors for in-hospital mortality of TBI among elderly patients.Using a nationwide database, we analyzed data from 5651 patients with TBI. Univariate analysis was conducted to compare patient demographics, neurologic status on admission, radiologic findings, systemic complication rates, length of hospital stay, in-hospital mortality, and home discharge rates between elderly and nonelderly groups. Multivariable analysis was conducted to determine prognostic factors for in-hospital mortality among elderly patients.Overall in-hospital mortality was significantly higher in elderly patients (12.8% vs. 19.3%; P0.001). In-hospital mortality of elderly patients with mild TBI increased significantly at7 days after admission, whereas that of elderly patients with moderate or severe TBI was significantly higher immediately after admission. Age (odds ratio [OR], 1.62; P = 0.024), male sex (OR, 1.30; P = 0.004), Japan Coma Scale score on admission (OR, 5.95, P0.001), and incidence of acute subdural hematoma (OR, 1.89; P0.001) were associated with in-hospital mortality in elderly patients with TBI.Elderly patients with TBI showed significantly higher in-hospital mortality. Delayed increases in in-hospital mortality were observed among elderly patients with mild TBI. Level of consciousness on admission was the strongest predictor of in-hospital mortality among elderly patients.

Purpose Although it has been reported that some antiepileptic drugs have inducing or inhibiting effects on lamotrigine (LTG) clearance, whether they have the same effects in Asian epilepsy patients as in those in other countries has not been clarified, especially in children. The aim of this study was to determine the effects of co-medications on LTG clearance in Japanese children with epilepsy. Methods A total of 342 routine serum concentration measurements of LTG in 102 Japanese epilepsy patients under 20 years of age were reviewed. The dose-corrected concentration (DCC) of LTG was calculated as [concentration]/[dose/(body weight)], and the DCC of LTG was compared by co-medication. The difference in the DCC of LTG was compared between patients with and without valproic acid (VPA) and between those with and without drugs inducing glucuronic acid conjugation (phenytoin (PHT), carbamazepine (CBZ), and phenobarbital (PB)). Results The DCC of LTG was significantly higher in patients on VPA and significantly lower in patients on drugs inducing glucuronic acid conjugation than in patients on LTG monotherapy. The DCC of LTG was significantly higher in patients on CBZ than in patients on PHT or PB. There was no correlation between the DCC of LTG and the concentration of VPA or metabolic inducers within the therapeutic range. Other antiepileptic drugs including clobazam, clonazepam, zonisamide, and levetiracetam had little effect on LTG concentration. Conclusion LTG concentration changes dramatically with concomitant antiepileptic drugs in Japanese children, as previously reported from other countries, and special attention is required. Although the dose of LTG should be adjusted when starting or discontinuing VPA or metabolic inducers, no adjustment is needed when changing the dose of VPA or metabolic inducers in the therapeutic range.

Objective This study used quantitative analysis to determine whether increased variability in fetal heart rate (FHR) is related to the risk of developing periventricular leukomalacia (PVL). Methods We analyzed 124 FHR traces of neonates delivered preterm at 27–33 weeks’ gestation to 105 mothers. FHR traces 1–3 h before delivery were translated into power-spectrum curves using a fast Fourier transformation. The total power (the area under the curve of 1–10 cycles per minute), segmental power of every cycle per minute, peak power, and frequency edges were calculated, and their relationship with the subsequent development of PVL was examined. Results Total power was significantly higher in the PVL group (n = 9, median 1813, range 1064–2426) compared to the non-PVL group (n = 114, median 1383, range 381–3324, p = 0.029). Infants in the PVL group had greater segmental power in segments with 1–2, 2–3, and 9–10 cycles per minute, than those in the non-PVL group. Total power of ⩾1550 was significantly correlated with the subsequent development of PVL and premature rupture of membranes. Furthermore, the frequency of pregnancy-induced hypertension was significantly reduced in the fetuses with a total power of ⩾1550. Conclusion Our study suggests that a fetus with increased FHR variability is at risk of developing PVL. This study provides additional evidence supporting the contribution of antenatal factors to the subsequent development of PVL.

Objective To clarify the semiology of febrile seizures (FS) and to determine the frequency of FS with symptoms suggestive of focal onset. Methods FS symptoms in children were reported within 24 h of seizure onset by the parents using a structured questionnaire consisting principally of closed-ended questions. We focused on events at seizure commencement, including changes in behavior and facial expression, and ocular and oral symptoms. We also investigated the autonomic and motor symptoms developing during seizures. The presence or absence of focal and limbic features was determined for each patient. The associations of certain focal and limbic features with patient characteristics were assessed. Results Information was obtained on FS in 106 children. Various events were recorded at seizure commencement. Behavioral changes were observed in 35 children, changes in facial expression in 53, ocular symptoms in 78, and oral symptoms in 90. In terms of events during seizures, autonomic symptoms were recognized in 78, and convulsive motor symptoms were recognized in 68 children. Focal features were evident in 81 children; 38 children had two or more such features. Limbic features were observed in 44 children, 9 of whom had two or more such features. There was no significant relationship between any patient characteristic and the numbers of focal or limbic features. Significance The semiology of FS varied widely among children, and symptoms suggestive of focal onset were frequent. FS of focal onset may be more common than is generally thought.

Objective The aim of this study was to clarify the characteristics of paroxysmal nonepileptic events (PNEs) suspected as being epileptic seizures by families of children with epilepsy. Methods The video-EEG (vEEG) recordings of habitual paroxysmal events in children with epilepsy at Nagoya University Hospital between October 2006 and January 2016 were reviewed. Based on the doctor's suspicion before the vEEG, the PNEs were divided into two groups that included PNEs suspected as epileptic seizures and PNEs suspected as PNEs. PNEs in the former group were classified based on the suspected seizure type. Results Of 886 habitual paroxysmal events, vEEG confirmed that 83 events (68 children) were PNEs. The median age of the 68 children was 3.2 years. Concurrent epilepsies included focal epilepsies (n=33), infantile spasms (n=16), and other types (n=19). The most common types of PNEs were sleep myoclonus (n=11), followed by stereotypies (n=9), awake myoclonus (n=8), paroxysmal ocular deviations (PODs, n=8), and tonic posturing (n=8). Even after direct observation or video viewing, the doctors suspected epileptic seizures in all three of the PODs and two of the tonic posturing children. Before the vEEG, however, the accurate visual information led to the speculation that the four psychogenic and two sleep myoclonus events were all PNEs. Myoclonus, stereotypies, and head drops were often misdiagnosed as epileptic spasms, while PODs and tonic posturing were often misdiagnosed as focal seizures with motor components. Additionally, staring and motion arrest during a drowsy state were often misdiagnosed as focal dyscognitive seizures. Seven of eight patients with PODs had epileptic spasms that were concurrent with epileptic seizures. A diffuse cerebral lesion or reduced visual acuity was seen in seven patients with PODs. Conclusion We re-emphasize that vEEG is essential for accurate diagnosis and provides evidence for listing POD in the differential diagnosis of oculomotor paroxysmal events.

Background and objectives: Conventional electroencephalogram (cEEG) is a reliable predictor of outcome in term infants with hypoxic ischemic encephalopathy (HIE). Early therapeutic hypothermia initiated within 6 h after birth is a beneficial treatment in these infants. However, a classification system with reduced cEEG recording time to determine early intervention has not been reported. The aim of this study is to propose a new classification of depression on cEEG with reduced recording time in infants with HIE and to examine the correlation between the classification and short-term outcome. Patients and methods: We retrospectively investigated 20 term infants with HIE in whom cEEG was performed within 12 h after birth, and deaths or outcomes at 18 months of age were assessed. We determined grades 0–3 EEG depression in each 10-min epoch based on the most common EEG patterns of each 20 s epoch defined by our criteria. Results: Eighteen infants could be assessed by depression grade. The Spearman’s rank correlation coefficient Rs between the maximum depression grade in 10-min epochs and three-grade outcomes was 0.68 ( P = 0.002), and that between the minimum one and outcomes was 0.66 ( P = 0.003). The area under the receiver operating characteristic curve of the maximum and minimum depression grades for predicting abnormal outcome were 0.885 and 0.869, respectively. Conclusions: We demonstrated a new cEEG depression classification with a recording time of at least 10 min in term infants with HIE and a good correlation with short-term outcome.

Mutations in GABRG2, which encodes the γ2 subunit of GABAA receptors, can cause both genetic epilepsy with febrile seizures plus (GEFS+) and Dravet syndrome. Most GABRG2 truncating mutations associated with Dravet syndrome result in premature termination codons (PTCs) and are stably translated into mutant proteins with potential dominant-negative effects. This study involved search for mutations in candidate genes for Dravet syndrome, namely SCN1A, 2A, 1B, 2B, GABRA1, B2, and G2. A heterozygous nonsense mutation (c.118C>T, p.Q40X) in GABRG2 was identified in dizygotic twin girls with Dravet syndrome and their apparently healthy father. Electrophysiological studies with the reconstituted GABAA receptors in HEK cells showed reduced GABA-induced currents when mutated γ2 DNA was cotransfected with wild-type α1 and β2 subunits. In this case, immunohistochemistry using antibodies to the α1 and γ2 subunits of GABAA receptor showed granular staining in the soma. In addition, microinjection of mutated γ2 subunit cDNA into HEK cells severely inhibited intracellular trafficking of GABAA receptor subunits α1 and β2, and retention of these proteins in the endoplasmic reticulum. The mutated γ2 subunit-expressing neurons also showed impaired axonal transport of the α1 and β2 subunits. Our findings suggested that different phenotypes of epilepsy, e.g., GEFS+ and Dravet syndrome (which share similar abnormalities in causative genes) are likely due to impaired axonal transport associated with the dominant-negative effects of GABRG2.

The aim of this study is to clarify the differences of EEG activities according to the presence or absence of disorganized patterns using amplitude spectral analysis. We compared EEGs of 17 preterm infants with disorganized patterns with those of 34 matched controls. Amplitude was defined as a square root of EEG power analyzed by fast Fourier transform algorithm, and was calculated in the 9 frequency bands. Six EEG segments of 10 s were collected from the part of EEG with continuous high voltage slow waves in the absence of artifacts. The results were separately evaluated according to the post-conceptional age at EEG recordings. In patients with disorganized patterns, reduced amplitude of delta waves in the central areas and increased amplitude of beta waves in the occipital areas were observed at 29-30 weeks of post-conceptional age. The results were almost similar at 31-32 weeks of post-conceptional age. Amplitude in theta or alpha frequency bands was not different between those with and without disorganized patterns either at 29-30 or 31-32 weeks of post-conceptional age. Amplitude spectral analyses will contribute to objective evaluation of disorganized patterns.

Objective To investigate the accuracy of conventional electroencephalograms (cEEGs) and amplitude-integrated EEGs (aEEGs) in the evaluation of brain maturation in normal pre-term infants. In addition, extrauterine acceleration of brain maturation was investigated. Methods A total of 129 EEGs recorded between 27 and 37 weeks of postconceptional age (PCA) from 37 clinically stable pre-term infants were analysed. One expert investigator blindly estimated PCA using cEEG and total cerebral function monitoring (CFM) score on aEEG. The estimated PCA (ePCA) on aEEG was calculated from a regression equation. Results The linear regression analysis showed a significant correlation between chronological PCA (cPCA) and ePCA on cEEG and between cPCA and total CFM score on aEEG. The estimation gap was smaller on cEEG, which was between −2 and +2 weeks in 96.9%. No significant correlation was observed between postnatal age and the estimation gap on cEEG or aEEG. Conclusions Estimated brain maturation was well correlated with cPCA on cEEG and aEEG. However, the evaluation of brain maturation was much more accurate on cEEG than aEEG. Extrauterine acceleration of brain maturation was not observed on either cEEG or aEEG. Significance cEEG and aEEG will be valuable tools for evaluation of brain maturation in pre-term infants.

Purpose: To assess the efficacy of diazepam suppositories at preventing febrile seizure recurrence during a single febrile illness to determine how to treat children with a febrile seizure on presentation at the hospital. Methods: We studied 203 children with febrile seizures from December 2004 through March 2006. On admission between December 2004 and May 2005, a diazepam suppository was administered to the patients. Patients seen between June 2005 and March 2006 were not treated with antiepileptic drugs on admission. Results: We saw a significant difference in the rate of recurrence of febrile seizures between children treated with diazepam and those who were not. Recurrences were observed in 2 (2.1%) of 95 children treated with diazepam and in 16 (14.8%) of 108 untreated children. For the 108 untreated patients, the median age was 22.8 months in those with recurrences and 30.6 months in those without, confirming that a younger age was related to a recurrence. Conclusions: A diazepam suppository after a febrile seizure will reduce the incidence of recurrent febrile seizures during the same febrile illness. However, a diazepam suppository after a febrile seizure should be used after carefully considering the benefits and potential adverse effects.

We retrospectively reviewed electroencephalography and magnetic resonance imaging findings for 21 children exhibiting delirious behavior during febrile illness. Among these, five patients had transient callosal lesions with or without white matter lesions on diffusion-weighted images. We compared the clinical characteristics, duration, and components of delirious behavior, the duration and severity of reduced consciousness, and EEG findings among patients with or without callosal lesions. No significant differences were detected in these items according to the presence or absence of callosal lesions. Adding insight into the pathogenesis of this condition, our study revealed that callosal lesions are not uncommon in patients exhibiting delirious behavior during febrile illness.

The authors studied the clinical course and electroencephalograms (EEGs) of 27 patients with periventricular leukomalacia (PVL), to investigate the relation between PVL and West syndrome. Seven of the 27 patients with PVL developed WS; in all seven the PVL was severe. Bilateral parieto-occipital dominant irregular polyspike-and-wave (PO-polySpW) bursts were seen in eight of the 21 patients whose EEGs were recorded within the age of one year (corrected for preterm birth). All eight had severe PVL, and in six, bilateral PO-polySpW bursts were followed by hypsarrhythmia and spasms. None of the 12 patients without polyspike-and-wave bursts developed West syndrome, the study shows that West syndrome is a common complication of severe PVL and that it correlates strongly with the occurrence of bilateral PO-polySpW bursts on follow-up EEGs.

We performed diffusion tensor imaging in two infants with neonatal hypoxic-ischemic encephalopathy. MRI revealed basal ganglia-thalamic lesions in both patients during the neonatal period. Patient 1 had severe neurological sequelae, whereas patient 2 achieved normal development. Conventional MRI at 12 months of age showed abnormal high-intensity areas in bilateral basal ganglia and thalami in patient 1, whereas no abnormal intensities were recognized in patient 2. Diffusion tensor tractography demonstrated poor depiction of white matter tracts above the level of centrum semiovale in patient 1. Region of interest analysis showed that fractional anisotropy of white matter of centrum semiovale and deep white matter was markedly reduced in patient 1 compared with patient 2, although apparent diffusion coefficient was not largely different between them. Our study suggested that abnormalities of diffusion property will be more widely present than those of conventional MRI. Diffusion tensor imaging will be useful to detect white matter abnormalities in normal-appearing white matter on conventional MRI.

Objective The aim of this study was to evaluate the usefulness of a combination of electroencephalogram (EEG) and flash visual evoked potentials (FVEPs) for predicting periventricular leukomalacia (PVL) in the early days of life. Study design Eighty-six of 108 infants admitted to Anjo Kosei Hospital during 1998 through 2000 were enrolled in this study. All subjects underwent EEG and FVEP during the early neonatal period and were followed-up until 18 months of corrected age. EEG was performed once within 72 h after birth, every 1-2 weeks during the first month and every 2-4 weeks during the second month. FVEPs were recorded at least twice, at the first and the second week of life. Results Of the 86 infants, 13 were diagnosed as having PVL. Among them, EEG abnormalities were observed in 11 infants and FVEP abnormalities in 10. The sensitivity and specificity of EEG were 0.85 and 0.95, respectively. The sensitivity and specificity of FVEPs were 0.77 and 0.96, respectively. All except one (92%) infant with PVL had EEG and/or FVEP abnormalities. Conclusions The combination of EEG and FVEPs can increase the sensitivity, but reduces the specificity to identify infants with PVL. The combination can makes up for the shortcomings of each method.

This study evaluated the usability of our MRI interpretation scheme among pediatricians with different skill levels in evaluating MRI of patients with cerebral palsy. We divided MRI findings into three groups: no abnormalities, pre/perinatally acquired lesions, and other abnormalities. Pre/perinatally acquired lesions were divided into six subgroups. Other abnormalities included brain malformations, ventriculomegaly, atrophic changes, and other unclassifiable abnormalities. We compared the interpretations of eight participants, i.e., three nonexpert pediatricians, two junior pediatric neurologists, and three senior pediatric neurologists, in evaluating magnetic resonance images of 73 children with cerebral palsy. The degree of agreement was substantial or near perfect for all participants. When limited to pre/perinatally acquired lesions, the degree of agreement was near perfect for all but one participant. The rate of correct diagnosis did not differ greatly according to participants' experience with pre/perinatally acquired lesions. For patients with basal ganglia thalamic lesions, multicystic encephalomalacia, and posthemorrhagic porencephaly, the rate of correct diagnosis increased according to participants' experience. Pre/perinatally acquired lesions can be appropriately interpreted by nonexpert pediatricians utilizing our interpretation scheme.

We evaluated drug-specific T cell responses in a patient with refractory partial seizures and paroxysmal kinesigenic choreoathetosis successfully treated with clinical desensitization to phenytoin. Drug-induced lymphocyte transformation test before desensitization was negative with a stimulation index of 130%. The frequencies and cytokine-producing phenotypes of phenytoin-specific T cells were examined simultaneously by using a carboxyfluorescein succinimidyl ester (CFSE) dilution assay. Before desensitization, the proportion of CFSE low CD4+ cells in whole CD4+ was 3.09%; 13.6% of CFSE low CD4+ cells were stained with anti-interferon γ antibody. After desensitization, phenytoin-specific CFSE low CD4+ cells decreased to background level. These results indicate that CFSE dilution assay will be useful for the diagnosis and monitoring of drug hypersensitivity.

We studied the relation among serum cytokine levels, EEG changes, and mild neurological complications (delirium and febrile seizure) in children with influenza. The serum levels of interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-alpha), and soluble tumor necrosis factor receptor-1 (sTNFR-1) were measured in 27 children with proven influenza infection with mild neurological complications (10 patients with delirium and 17 with febrile seizures) and seven control children. EEG was recorded in 14 children with neurological complications. EEG showed focal slowing in four of nine patients with delirium and in four of five with febrile seizures. Generalized slowing was observed in one patient with delirium. The median serum IL-6 level was 31.2+/-15.1 pg/ml (range, 7.5-64.5 pg/ml) in the delirium group, 42.3+/-44.0 pg/ml (range, 8.0-196.0 pg/ml) in the febrile seizure group, and 15.4+/-7.0 pg/ml (range, 7.2-28.0 pg/ml) in the control group. Serum TNF-alpha and sTNFR-1 levels were not different among three groups. Mild neurological complications associated with influenza were related to the mildly abnormal serum IL-6 levels and EEG findings. The combination of these parameters will be useful for early diagnosis and differentiation of neurological complications in children with influenza. Further studies will be necessary for investigating that IL-6 has the diagnostic value for differentiation between severe encephalopathy and mild neurological complications in children with influenza.

The aim of this study is to further clarify ictal electroencephalographic findings of patients with benign partial epilepsy in infancy in order to better understand its neurophysiologic features. The study group consisted of 13 infants with definite benign partial epilepsy in infancy, in whom ictal electroencephalograms were recorded and its benignity was confirmed at 8 years or more. The seizure manifestation was reviewed on the basis of video findings in eight patients in whom simultaneous video-electroencephalography recording was available. In the other five patients, the seizure manifestations were determined according to the observations of physicians, nurses, or technicians. Thirteen seizures from eight patients were complex partial, and six seizures from six patients were secondarily generalized ones. Ictal discharges at the onset of a seizure were focal in all seizures. The site of the origin of seizures was in the temporal area in 10 of 13 complex partial seizures, whereas it was in the parietal or occipital area in all 6 secondarily generalized seizures. Among 13 complex partial seizures, paroxysmal discharges remained focal throughout the seizures in 6 seizures, whereas they spread to one hemisphere in the other 7 seizures. Motion arrest or decreased responsiveness was uniformly observed. Lateral eye deviation was commonly recognized in complex partial seizures, whereas head rotation was observed only in seizures in which hemispheric propagation of ictal discharges was observed. Ictal electroencephalographic findings of patients with benign partial epilepsy in infancy were relatively uniform, suggesting the homogeneity of patients with benign partial epilepsy in infancy.

SummaryObjective The aim of this study was to clarify characteristics of post-encephalopathic epilepsy (PEE) in children after acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), paying particular attention to precise diagnosis of seizure types. Methods Among 262 children with acute encephalopathy/encephalitis registered in a database of the Tokai Pediatric Neurology Society between 2005 and 2012, 44 were diagnosed with AESD according to the clinical course and magnetic resonance imaging (MRI) findings and were included in this study. Medical records were reviewed to investigate clinical data, MRI findings, neurologic outcomes, and presence or absence of PEE. Seizure types of PEE were determined by both clinical observation by pediatric neurologists and ictal video–electroencephalography (EEG) recordings. Results Of the 44 patients after AESD, 10 (23%) had PEE. The period between the onset of encephalopathy and PEE ranged from 2 to 39 months (median 8.5 months). Cognitive impairment was more severe in patients with PEE than in those without. Biphasic seizures and status epilepticus during the acute phase of encephalopathy did not influence the risk of PEE. The most common seizure type of PEE on clinical observation was focal seizures (n = 5), followed by epileptic spasms (n = 4), myoclonic seizures (n = 3), and tonic seizures (n = 2). In six patients with PEE, seizures were induced by sudden unexpected sounds. Seizure types confirmed by ictal video-EEG recordings were epileptic spasms and focal seizures with frontal onset, and all focal seizures were startle seizures induced by sudden acoustic stimulation. Intractable daily seizures remain in six patients with PEE. Significance We demonstrate seizure characteristics of PEE in children after AESD. Epileptic spasms and startle focal seizures are common seizure types. The specific seizure types may be determined by the pattern of diffuse subcortical white matter injury in AESD and age-dependent reorganization of the brain network.

The aim of this study is to clarify the detailed clinical features of benign partial epilepsy in infancy. The subject of the study was 33 patients with benign partial epilepsy in infancy confirmed by a long-term follow-up beyond 8 years of age. The data were obtained from medical records of the patients in combination with the data obtained from telephone interview. The median age at the first and last seizure was 5 and 8 months, respectively. In 26 patients, seizures disappeared within 3 months after the onset. Family history of benign partial epilepsy in infancy was seen in 17 patients. The median number of seizures was 7. A cluster of seizures was observed in 26 patients. The type of seizures was complex partial seizures alone in 6 patients, secondarily generalized seizures alone in 9, and both types in 18. Decreased responsiveness, lateral eye deviation, and cyanosis were commonly observed. Initial interictal electroencephalograms were normal in all patients. However, paroxysmal discharges were recognized in 2 patients in the second EEG during the first year of life. The main features of benign partial epilepsy in infancy were a high incidence of a cluster of seizures, short persistence of seizures, and normal initial interictal EEGs.

Objective: The aim of this study is to describe a new objective method of detecting white matter injury by use of refiltering function of digital EEG. Study design: The neonatal EEGs of 9 patients with PVL were analyzed retrospectively, and compared with EEGs of 18 normal infants without PVL. Abnormal brushes were counted on the refiltered EEG where low cut filter were set at 10 Hz so that slow waves were eliminated. Abnormal brushes were defined as spindle-like fast wave bursts with maximal amplitudes higher than 40 mV and frequencies between 13 Hz and 20 Hz, which were easily distinguishable from a flat background. Results: The incidence of abnormal brushes in infants with PVL was 0.25/min, 0.45/min, 2.08/min, and 0.62/min on the frontal, central, occipital, and temporal region, respectively. The incidence in infants without PVL was 0.14/min, 0.08/min, 0.24/min, and 0.19/min, respectively. Abnormal brushes were observed more often in infants with PVL on the central, occipital, and temporal region than in infants without PVL. Localization of abnormal brushes was also correlated with the lesion of white matter injury on MR images and clinical outcome. Conclusion: Our results indicated that abnormal brushes on refiltered EEGs were strongly associated with white matter injury.

Summary Purpose We reviewed our experience with ACTH for the treatment of intractable generalized seizures other than spasms. Methods Efficacy and adverse effects of ACTH against intractable generalized seizures other than spasms were retrospectively investigated in 15 patients treated between 1991 and 2003. The median age of the patients at ACTH therapy was 42 months. The targeted type of seizure was brief tonic in 6, atypical absence in 6, atonic in 2, and myoclonic in 1. The dose of ACTH was 0.01–0.015mg/kg in most patients. The effect of ACTH was determined at the end of ACTH therapy, and 3 months and 1 year after ACTH therapy. Results Seizure freedom was obtained in 13 of 15 patients. However, 6 of them had a recurrence of seizures within 3 months after ACTH therapy. ACTH was the most effective in patients with atypical absence seizures. Statistical analysis showed that the number of injection and total dose of ACTH were smaller in patients with atypical absence seizures than in those with brief tonic seizures. Adverse effects were frequent but serious one was not observed. Conclusion ACTH therapy was effective in patients with intractable generalized seizures other than spasms. However, its efficacy was often transient. ACTH can be useful for generalized seizures other than spasms but its limitation should be considered.

Single photon emission computed tomography was performed in three preterm infants with athetoid cerebral palsy due to kernicterus. No clinical signs and symptoms of kernicterus, or ultrasonographic abnormalities were seen during the neonatal period in any patients. Although MRI during infancy revealed high intensity areas in bilateral globi pallidi in all of them, MRI abnormalities were mild in two of them. On later MRI, subtle high intensity areas in the globi pallidi were recognized in only one of them. Single photon emission computed tomography demonstrated hypoperfusion in the basal ganglia regions in all patients. Regions of interest analyses showed decreased blood flow in the basal ganglia related to the cortical areas. Single photon emission computed tomography will be useful for the diagnosis of kernicterus, whereas MRI abnormalities become less clear beyond infancy.

Background The authors evaluate the efficacy and safety of gefitinib monotherapy in chemotherapy-naive patients with advanced non–small-cell lung cancer (NSCLC). A secondary endpoint is to evaluate the relationship between clinical manifestations and epidermal growth factor receptor ( EGFR ) mutation status. Methods Japanese chemotherapy-naive NSCLC patients were enrolled. They had measurable lesions, Eastern Cooperative Oncology Group performance status of 0 to 2, and adequate organ and bone marrow function. Patients received 250 mg of oral gefitinib daily. EGFR mutations in exon 18, 19, and 21 of DNA extracted from tumor and serum were analyzed by genomic polymerase chain reaction and direct sequence. Results All 30 patients were eligible for the assessment of efficacy and safety. An objective response and stable disease were observed in 10 patients (33.3%) and nine patients (30.0%), respectively. The median time to progression was 3.3 months and the median overall survival was 10.6 months. The 1-year survival rate was 43.3%. Grade 3 toxicities were observed in seven patients. EGFR mutation was observed in four of 13 (30.8%) tumors, and two of them achieved partial response. In serum samples, three of 10 patients with EGFR mutations in the serum before treatment had a response to gefitinib. EGFR mutation was observed in 10 of 27 and significantly more frequently observed in the posttreatment samples from patients with a partial response or stable disease than in those from patients with progressive disease ( p = 0.006). Conclusions Gefitinib monotherapy in chemotherapy-naive NSCLC patients was active, with acceptable toxicities. These results warrant further evaluation of gefitinib monotherapy as a first-line therapy. The EGFR mutation in serum DNA may be a biomarker for monitoring the response to gefitinib during treatment.

Background: The purpose of the present paper was to study the use of antipyretics in children with delirium associated with fever in order to clarify their possible adverse effects. Methods: The use of antipyretics was investigated in 26 children with delirious behavior associated with fever. Temporal relation between delirious behavior and the use of antipyretics was recognized in six children. Results: In four children, delirious behavior was observed soon after administration of antipyretics. The antipyretics used were acetaminophen in two children, mephenamate in one, and diclofenac in one. In the other two children, delirious behavior was observed when body temperature began to fall 1–2 h after administration of antipyretics. The antipyretics used were acetaminophen in one child and mephenamate in one. Conclusion: A temporal relationship between antipyretics and delirious behavior was observed in some patients with febrile delirium. This suggests that antipyretics can be a trigger of delirium.

Summary: Purpose: The aim of this study was to investigate the long-term outcome of children with benign partial epilepsy in infancy (BPEI). Methods: A telephone-interview survey using a structured questionnaire was conducted with patients who were diagnosed as having possible BPEI at age 2 years and who were 8 years or older at the time of the survey. The data from 39 of 48 patients were available. The median age at the time of the survey was 11.3 years; 18 boys and 21 girls were included. Results: Three patients had a recurrence of unprovoked seizure beyond age 2 years. Four patients had cognitive problems (mild mental retardation in three and Asperger syndrome in one). An association of paroxysmal kinesigenic choreoathetosis was observed in three patients, and another three had experienced seizures associated with mild gastroenteritis. Major behavioral problems were not recognized in any patients. Four patients were excluded from having definite BPEI at age 5 years, and another two were excluded for having definite BPEI at the last follow-up. Eventually, 33 of 39 patients were categorized as having definite BPEI beyond 8 years of age. Conclusions: A large majority of patients diagnosed as possibly having BPEI at age 2 years did not have a recurrence of unprovoked seizures and cognitive problems beyond 8 years of age.

Objective To clarify the clinical and radiological spectrum of posterior reversible encephalopathy syndrome (PRES) in children, and to identify the prognostic factors. Methods The records of 40 children with PRES were reviewed. Acute clinical symptoms, MRI including apparent diffusion coefficient (ADC) maps in the acute and follow-up periods and neurological sequelae, including epilepsy, were noted. Results Age at onset ranged from 2 to 16 years. Underlying disorders were hematological or neoplastic disorders ( n = 20), renal diseases ( n = 14) and others ( n = 6). In the acute period, 31 patients had seizures, 25 had altered consciousness, 11 had visual disturbances and 10 had headache. Of 29 patients who had ADC maps in the acute period, 13 had reduced diffusivity as shown by ADC within PRES lesions. Of 26 patients with follow-up MRI, 13 had focal gliosis or cortical atrophy. No patients had motor impairment, and four patients had focal epilepsy. No clinical variables were associated with focal gliosis or cortical atrophy on follow-up MRI, but lesional ADC reduction in the acute period was prognostic for focal gliosis or cortical atrophy on follow-up MRI ( p = 0.005). Conclusions To the best of our knowledge, this is the largest cohort study to date involving PRES in children. Acute symptoms in pediatric patients are similar to those reported in adults, but altered consciousness was more frequent in children. Lesional ADC reduction in the acute period was common and was a good predictor of later, irreversible MRI lesions.

Angelman syndrome (AS) is a genetic disorder with characteristic clinical and EEG findings. We report here the results of long-term follow-up studies on the epileptic seizures and EEG findings of 23 cases of deletion type AS confirmed by FISH analysis, including seven cases previously reported by Matsumoto et al. in 1992. The age at last follow-up in 23 patients was from 1 to 37 years of age (average: 18.0 years), with 10 patients (43.5%) in their 20s, and five over 30. Epileptic seizures were seen in all patients, and the age at seizure onset ranged from 3 to 50 months (average: 21.7 months). Status epilepticus was seen in 11 patients (47.8%). The percentages of cases seizure-free for more than 3 years were 25% (4/16) at 10 years of age, 70% (7/10) at 20, and 80% (4/5) at 30. The EEG findings were classified into six patterns according to the previous report: N (no spike, including focal slow waves), HVS (diffuse high-voltage slow bursts with or without spikes), F (focal spikes or multifocal spikes), S (diffuse spike and waves), C (continuous diffuse spike and waves), Hy (hypsarrythmia or hypsarrhythmia like waves). Hy was noted at ages 0-2 years in two cases. C was observed from the ages 2 to 15 years, being most frequently noted at 3-6 years of age, and it was never seen after 16 years of age. S was observed from ages 1 to 21 years. F was seen from 2 to 21 years of age, and most frequently during the ages of 2-7 years. HVS was seen from 0 years, and still remained after the age of 20. After 22 years of age, all patients showed N pattern including focal slow waves. One of the two patients who had bilateral frontal dominant delta slow waves in their 30s, had a recent seizure. Even if the spikes disappear with age, when bi-frontal focal slow waves remain, seizures may occur even in patients over 30.

The aim of this study was to clarify the efficacy and safety of lidocaine tape therapy (LDT) in patients with convulsions with mild gastroenteritis (CwG). Twenty-one consecutive episodes of CwG were treated with LDT therapy. The dose of LDT was 36 mg in patients with body weights of15 kg, 54 mg in those with body weights between 15 and 20 kg, and 72 mg in those with body weights of20 kg. LDT was attached on the back of each patient every 12 h. Application of LDT was continued for 48 h. The serum levels of lidocaine (LD) were measured 4 h after the first attachment of LDT. The seizures completely ceased in 13 episodes (62%) after the application of LDT, while a recurrence of seizures was observed in the other 8 episodes. Drip infusion of LD was performed in 4 of these 8 episodes. The serum LD levels were only measurable in 6 infants (average, 0.4 microg/ml; range, 0.2-0.5 microg/ml). In the other patients, the serum LD levels were below the lower limit of measurement. Adverse effects of LDT were not observed in any patients. LDT therapy was safe and effective in patients with CwG.

The aim of this study was to clarify the efficacy of antiepileptic drugs during a cluster of seizures in patients with convulsions with mild gastroenteritis (CwG). We retrospectively investigated the details of antiepileptic treatment in 110 consecutive episodes in 103 patients with CwG. The temporal course of the seizures and the use of antiepileptic drugs were investigated in each episode. Drugs were judged as effective when seizure cessation was achieved after administration of the drug. As the first drug, diazepam (DZP)/bromazepam (BZP) was effective in 38%, phenobarbital (PB) in 40%, and lidocaine (LD) in 100%. As the second drug, DZP/BZP was effective in 42%, PB in 69%, and LD in 100%. As the third drug, PB was effective in 70%. When the efficacy of the first doses of PB and LD were compared, the efficacy rate was significantly higher for LD than for PB (P = 0.047). In conclusion, LD was effective for the cessation of seizures in patients with CwG.

We reported three term or near-term infants with parasagittal infarcts. Their Apgar scores were low and the amniotic fluid was meconium-stained. Resuscitation was necessary immediately after birth, but they were not stuporous and no neurological abnormalities were recognized on admission. They showed metabolic acidosis and transient hypoglycemia, and two showed hematoemesis. Seizures were observed between 2 and 15 h of age in all of them. Electroencephalography demonstrated moderate or severe depression, and CT demonstrated bilateral abnormal low densities in the border zones of the middle and posterior cerebral arteries. Two of them had mental retardation and epilepsy, although the other exhibited normal development. Our infants suggest that neonatal seizures can also occur in infants with hypoxic ischemic encephalopathy without apparent neurological abnormalities.

Background: Angiotensin II is a putative mediator in asthma, but the effect of topical administration of type 1 angiotensin II (AT1) receptor antagonists on allergic airway reactions is not known. Objective: To investigate the effect of intranasal administration of CV-11974, an AT1 receptor antagonist, and of PD123319, a type 2 angiotensin II (AT2) receptor antagonist, on antigen-induced airway reactions in guinea pigs. Methods: Thirty minutes after intranasal topical administration of CV-11974 (0.1 or 1.0 mg/ml) or PD123319 (10 mg/ml) into the airways, the animals were given an antigen challenge. Airway hyperresponsiveness and bronchoalveolar lavage fluid were analyzed 24 h after the antigen challenge. Results: Although these compounds did not inhibit antigen-induced early-phase bronchoconstriction or late-phase airway eosinophilia, intranasal administration of CV-11974 (but not PD123319) inhibited antigen-induced airway hyperresponsiveness in a dose-dependent manner 24 h after the antigen challenge. Conclusion: Intranasal administration of an AT1 receptor antagonist reduces antigen-induced airway hyperresponsiveness.

BACKGROUND AND PURPOSE: Developmental and seizure outcomes in patients with cryptogenic West syndrome are variable. Our aim was to clarify the relationship between FDG-PET findings in infancy and long-term seizure and developmental outcome in cryptogenic West syndrome. MATERIALS AND METHODS: From 1991 to 1999, we prospectively performed FDG-PET from the onset of cryptogenic West syndrome in 27 patients. PET was performed at onset and at 10 months of age. In 2012, we evaluated the educational status, psychomotor development, and seizure outcome in 23 of the 27 patients (13–22 years of age). The correlation between PET findings and outcome was evaluated. RESULTS: At onset, PET showed hypometabolism in 13 patients (57%). The second PET after the initial treatment revealed cortical hypometabolism in 7 patients (30%). While hypometabolism at onset disappeared on the second PET in 9 patients, it was newly revealed in 3 patients on the second PET. In 2012, seven patients had persistent or recurrent seizures. Eight patients had intellectual impairment. The first PET did not correlate with seizure or developmental outcome. Five of 7 patients (71%) with hypometabolism seen on the second PET had persistent or recurrent seizures, while 14 of 16 (88%) patients with normal findings on the second PET were free of seizures. Five of 7 patients (71%) showing hypometabolism on the second PET had intellectual impairment. Thirteen of 16 (81%) patients with normal findings on the second PET showed normal intelligence. A significant correlation was found between the second PET and long-term seizure ( P = .01) or developmental outcome ( P = .03). CONCLUSIONS: Cortical hypometabolism is not permanent; it changes with clinical symptoms. Hypometabolism after initial treatment predicts long-term seizures and poor developmental outcome.

The aim of this study is to clarify the timing of brain injuries in preterm infants who later developed West syndrome. We assessed the timing of brain insults with serial EEG recordings beginning immediately after birth in preterm infants less than 33 weeks of gestational age. The timing of brain insult was assessed by considering EEG findings in relation to the time of birth. The timing was considered prenatal if an EEG immediately after birth already showed chronic stage abnormalities, perinatal if it showed acute stage abnormalities, and postnatal if it was normal and a later EEG showed acute stage abnormalities. Eleven infants had periventricular leukomalacia and developed West syndrome. The timing of the brain insult was considered prenatal in 5 infants, perinatal in 2, postnatal in 2, prenatal plus postnatal in one, perinatal plus postnatal in one. The infants of less than 29 weeks of gestation were more likely to suffer brain damage postnatally, whereas those of more than 28 weeks tended to have brain injury prenatally. The timing of brain injury of preterm infants who later developed West syndrome is more often prenatal than generally considered.

The aim of this study is to clarify the long-term outcome of patients with partial epilepsies that begin in the first 2 years of age. Thirty-nine infants who experienced partial epilepsies with onset by 2 years of age were followed until they were at least 5 years of age. Seizure and developmental outcome were investigated in relation to the following items: delayed psychomotor development before onset of seizures; interictal electroencephalogram (EEG) abnormalities; abnormal neuroimaging; family history of epilepsy; and history of neonatal seizures. Thirty-three infants achieved freedom from seizures and 27 achieved normal development. Delayed development before the onset and abnormal interictal EEG correlated with both poor seizure and developmental outcome. Abnormal neuroimaging findings correlated with a poor seizure outcome. A history of neonatal seizures and lack of a family history of epilepsy correlated with poor developmental outcome. The combination of the first four factors predicted a normal outcome with a sensitivity of 0.79 and a specificity of 0.87. The seizure and developmental outcomes were better in this study than those in previous studies. The combination of the four factors will be useful for predicting the outcomes in patients with partial epilepsies in infancy.

We report a Japanese boy who died at Day 28 of life because of severe carbamoyl phosphate synthetase I (CPS1) deficiency that was proven by enzyme assay. By analysis of cDNA and genomic DNA, he was shown to be a compound heterozygote with two point mutations of the CPS1 gene, 840G>C leading to an aberrant splicing and 1123C>T (predicting Q375X). The 840G>C was a mutation described in another Japanese family. Since his parents carried each mutation heterozygously, we performed prenatal diagnosis at 16 weeks of his mother's next gestation by multiplex PCR and melting curve analysis in a single capillary containing two-color fluorescent (LC-Red 640 and LC-Red 705) probes on LightCycler. We analyzed genomic DNA extracted from amniotic cells and found that the fetus was homozygous for the wild-type alleles. At term a healthy girl was born without hyperammonemia.

Neuroimaging findings are usually normal in children with benign partial epilepsy in infancy. However, we found a transient reduction of water diffusion in the corpus callosum in two patients with probable benign partial epilepsy in infancy. The patients were admitted to our hospital because of seizure clusters. No delay in the developmental milestones was seen, and no neurological abnormalities were observed during the interictal period. Interictal electroencephalography was normal in both infants. However, the diffusion-weighted images showed abnormal high intensities in both the genu and splenium in one patient and in the splenium only in the other. No diffusion abnormalities were observed in follow-up magnetic resonance imaging. The clustered seizures may be related to the transient callosal lesions seen in our patients.

We studied MRI findings in patients with symptomatic localization-related epilepsy (S-LRE) beginning in infancy and early childhood and their relation to some clinical features. MRI findings were investigated in 183 patients with S-LRE beginning before 6 years of age. S-LRE was defined as an epileptic syndrome manifesting with only partial seizures at least for a certain period during the clinical course, having specific neuroimaging abnormalities considered to be related to epileptic seizures, moderate or severe mental retardation, or cerebral palsy. The timing of injury was divided into the following four groups; prenatal, perinatal, postnatal and unknown. It was presumed prenatal in 56 patients (31%), perinatal in 25 (14%), postnatal in 32 (18%) and unknown in 70 (38%). The median age at the onset of partial seizures was 24 months in the congenital (prenatal and unknown) group, 39 months in the perinatal group and 25 months in the postnatal group. The onset of partial seizures was later in the perinatal group than in the congenital group. There were no significant differences in the other clinical characteristics among groups except for more frequent association of cerebral palsy in the perinatal group than in the congenital group. Our study suggests that congenital (prenatal and unknown group) disorders may be most common in patients with S-LRE beginning in infancy and early childhood. The onset of partial seizures occurred later in the perinatal group.

Benign partial epilepsies are not rare in infancy and comprise two forms, although both are closely related. One is partial epilepsy with complex partial seizures (CPS) and the other one with secondarily generalized seizures (SGS). The most frequent site of seizure origin was in the temporal area in the former and central, parietal or occipital area in the latter. The former has not been well recognized because of subtle seizure manifestations and a favorable outcome. Its unique characteristics should be emphasized because it may be difficult to diagnose it unless we know its presence. Benign convulsions are also common in infancy. Most of them may belong to partial epilepsy with SGS, although confirmation with ictal EEG recording is necessary for accurate diagnosis. Some are familial and many of them seem to show an autosomal dominant pattern, but some seem to have autosomal recessive inheritance.

The effects of different check sizes on the 100m component of pattern reversal visual evoked magnetic fields (VEF) and the P100 component of visual evoked potentials (VEP) in terms of latency, amplitude and source localization were analyzed. Half field stimuli with or without central occlusion with check sizes of 15', 30', 60', 90' and 180' of visual arc were given to 7 healthy subjects. VEF and VEP were recorded simultaneously. The effect of the check size on the peak latency of both 100m and P100 was significant (P

Near-infrared spectroscopy was used to measure the relative concentration changes of oxygenated hemoglobin (HbO(2)), deoxygenated hemoglobin (HbR), and total hemoglobin (t-Hb) before, during, and after hyperventilation in eight patients with childhood moyamoya disease who underwent surgery. The patients were divided into the following two groups: those with and those without re-build-up phenomenon on electroencephalography. All patients except one exhibited decreased concentrations of HbO(2) and t-Hb during hyperventilation and decreased concentrations of HbO(2) and increased concentrations of HbR after hyperventilation. The difference between the hemoglobin concentration during and after hyperventilation was significantly greater in the group with the re-build-up phenomenon than the group without it (P0.03, Mann-Whitney U test). The patients with the re-build-up phenomenon had lower HbO(2) and higher HbR concentrations after hyperventilation. The concentrations of HbO(2) and HbR reflect an alteration in the oxygenated blood supply and/or oxygen use. Thus, it is strongly suggested that regional cerebral hypoxia and metabolic oxygen disturbances play an important role in the occurrence of the re-build-up phenomenon. Near-infrared spectroscopy is a useful noninvasive method to evaluate the efficacy of surgical treatment in childhood moyamoya disease.

We report two infants with bilateral basal ganglia–thalamic lesions subsequent to prolonged fetal bradycardia. Cardiotocogram revealed severe bradycardia lasting for more than 20 min in both. They demonstrated a significant encephalopathy, abnormal muscle tones and signs of brainstem injury. Clinical or electrical seizures were not observed in either of them. CT during early neonatal period demonstrated decreased tissue attenuation in basal ganglia and thalami in the absence of extensive cortical changes. Both of them developed severe mental retardation and quadriplegia. MRI in late infancy demonstrated abnormal high intensity areas in bilateral basal ganglia, thalami and around central sulci on T2-weighted image. Close correlation between prolonged fetal bradycardia and basal ganglia–thalamic lesion was suggested.

In order to determine the factors responsible for the differentiation of cytomegalovirus (CMV) hepatitis and Epstein-Barr virus (EBV) hepatitis in previously healthy adults, the clinical features and laboratory data of both types of hepatitis were retrospectively analyzed. CMV hepatitis showed a tendency to increase in our department. In comparison with EBV hepatitis, CMV hepatitis occurred in significantly older hosts than EBV hepatitis. We found that lymphadenopathy, cough and sore throat was more common in EBV hepatitis than in CMV hepatitis. The number of peripheral white blood cell count and atypical lymphocytes, and serum GOT, GPT, LDH and CRP levels of CMV and EBV hepatitis showed no significant differences.

The C-C chemokine MCP-1 elicits significant neutrophil emigration in rats with chronic adjuvant-induced inflammation, but not in naive animals. We examined responses to the C-X-C chemokine CINC/gro to determine whether this class of chemokine elicits altered neutrophil responses during chronic inflammation.CINC/gro was superfused over mesenteric venules of naive rats or animals with chronic adjuvant-induced vasculitis. Antibodies were used to characterize adhesive mechanisms.CINC/gro elicited leukocyte transendothelial migration in adjuvant-immunized rats at 100-fold lower concentrations than required to elicit transmigration in naive animals. In both groups, neutrophils constituted95% of the leukocytes recruited by CINC/gro. Using in vitro chemotaxis assays, neutrophils from control and adjuvant-immunized rats responded equally to CINC/gro, suggesting differences in migration were not related to neutrophil phenotype. Differences in adhesion molecule usage were noted in vivo. In control animals, CD18 antibodies blocked CINC/gro-induced neutrophil adhesion and emigration. In adjuvant-immunized animals, an alpha 4-integrin antibody reduced adhesion and emigration, while a CD18 antibody selectively inhibited emigration.This study demonstrates increased sensitivity to a C-X-C chemokine in a model of chronic inflammation, implicates the alpha 4-integrin in neutrophil adhesion, and demonstrates that CD18 mediates leukocyte transendothelial migration independent from firm adhesion.

The objective of our study was to determine the relation between the serum level of endotoxin at birth and the development of periventricular leukomalacia (PVL) in preterm infants. We studied 68 preterm infants whose gestational ages ranged between 27 and 33 weeks, and birthweights between 1000 and 2000 g. The serum endotoxin level was measured in the blood immediately after birth by means of a conventional chromogenic Limulus test. Serum endotoxin was taken as present when it was >5 pg/ml (indicated by ENDO(+)). Premature rupture of membranes (PROM), the mode of delivery, EEG findings and the development of PVL were investigated. Six infants were diagnosed as having cystic PVL on ultrasonography. Endotoxin was detected in 28 infants. PROM was observed in 18 ENDO(+) infants and 16 ENDO(-) infants (P = 0.085). Caesarean section was performed in nine ENDO(+) infants and 14 ENDO(-) infants (P = 0.29). PVL was observed in two ENDO(+) infants and four ENDO(-) infants (P > 0.99). EEG abnormalities were recognized in five ENDO(+) infants and six ENDO(-) infants (P > 0.99). Between ENDO(+) infants and ENDO(-) infants, no significant difference was observed in any of the four parameters. These results may indicate that endotoxin itself does not directly cause PVL.

Summary: Purpose: A large number of children had fits while watching the animated cartoon television (TV) program “Pocket Monster.” To elucidate the seizures associated with the TV program, we administered a questionnaire survey in Aichi Prefecture, Japan. Methods: The questionnaires were sent to 75 hospitals located in and around Aichi prefecture. The presence of epileptic seizures and the types of seizures were determined by three pediatric neurologists. Results: Sixty-one hospitals responded to the questionnaire survey. Among 95 patients living in Aichi prefecture for whom enough information on seizure manifestations and EEG was available, ≤93 patients were considered to have epileptic seizures while watching the TV program. Most seizures occurred at a scene in which red and blue frames alternated at 12 Hz. Sixty-nine (74%) patients had no history of epilepsy. Thirty-nine patients had generalized seizures, and 49 patients had partial seizures. Partial seizures occurred more frequently in the younger age group than did generalized seizures. The EEG revealed a photoparoxysmal response (PPR) in 43% of patients. PPR was present not only in patients with a history of epilepsy (54%) but also in those with no history of epilepsy (38%). Conclusions: Almost all seizures induced by the TV program “Pocket Monster” were epileptic, and partial seizures were induced more frequently than generalized seizures. The incidence of this “Pocket Monster”-induced seizures was roughly estimated as ≥1 in 4,923 individuals aged 6–18 years.

[Case report] A 65-year-old male war admitted to a local hospital because of fever. He was treated with piperacillin and clindamycin without noticeable effect. He began to complain of loss of vision on the third hospital day and culture of the blood specimen yielded Klebsiella pneumoniae. He was diagnosed as endophthalmitis and referred to our hospital for further examination. The hematological laboratory test showed leukocytosis (12,700/microliter) and increased CRP (20.4 mg/dl). A computed tomographic (CT) scan of the thorax revealed multiple lung abscesses. An abdominal ultrasonographic scan and a CT scan of the abdomen revealed multiple liver abscesses. We drained the abscess in the liver and Klebsiella pneumoniae was detected from the sample of aspirated fluid and his sputum. Meropenem was administered intravenously. Fever started to improve on the tenth hospital day and the size of both liver and lung abscesses were reduced. He has lost vision of his right eye. He was discharged after sixty days. He did not have any immunosuppressive underlying disease including HIV infection and diabetes mellitus which cause these lesions.

We reported a child with refractory partial seizures successfully managed by clinical desensitization to phenytoin. The patient had ischemic brain lesions due to cardiopulmonary arrest at 39 weeks of corrected age. He had complex partial seizures refractory to several antiepileptic drugs since 4 years of age. At 8 years 1 month of age, phenytoin was first administered. Fever and maculopapular rashes appeared at 10 days after phenytoin initiation, and then the drug was discontinued. At 8 years 8 months of age, desensitization was attempted because of refractoriness of seizures to drugs other than phenytoin. Desensitization was started at 1 mg daily, and then the dose was doubled every week. His seizures were controlled by 150 mg/day of phenytoin in combination with primidone. No problems have been observed during desensitization.