Developmental and epileptic encephalopathies: Is prognosis related to different epileptic network dysfunctions?

Developmental and epileptic encephalopathies are a group of rare, severe epilepsies, which are characterized by refractory seizures starting in infancy or childhood and developmental delay or regression. Developmental changes might be independent of epilepsy. However, interictal epileptic activity and seizures can further deteriorate cognition and behavior. Recently, the concept of developmental and epileptic encephalopathies has moved from the lesions associated with epileptic encephalopathies toward the epileptic network dysfunctions on the functioning of the brain. Early recognition and differentiation of patients with developmental and epileptic encephalopathies is important, as precision therapies need to be holistic to address the often devastating symptoms. In this review, we discuss the evolution of the concept of developmental and epileptic encephalopathies in recent years, as well as the current understanding of the genetic basis of developmental and epileptic encephalopathies. Finally, we will discuss the role of epileptic network dysfunctions on prognosis for these severe conditions.

Recreational nitrous oxide abuse related subacute combined degeneration of the spinal cord in adolescents - A case series and literature review.

BACKGROUND: Nitrous oxide (N2O) is a commonly used inhaled anesthetic in outpatient dental procedures. However, the increasing recreational use of N2O may result in vitamin B12 deficiency-related neurologic and psychiatric symptoms. The aim of this study was to demonstrate the clinical features of chronic N2O abuse in pediatric patients. METHODS: Patients under 20 years of age who were diagnosed with N2O-induced subacute combined degeneration of the spinal cord from 2012 to 2018 were enrolled in this study. Clinical presentations, laboratory, imaging, ancillary studies, treatments and outcomes were analyzed. RESULTS: Nine patients were included, all of whom presented with symptoms of myeloneuropathy including limb numbness, limb weakness or unsteady gait. Six patients had low or low-normal vitamin B12 (cyanocobalamin) levels. Eight patients had evidence of subacute combined degeneration of the spinal cord via neuroimaging studies. All of the patients received vitamin B12 supplementation as treatment. All had full recovery of muscle power within 2 months. Five patients had persistent sensory deficits. CONCLUSION: Chronic N2O abuse can cause permanent neurological damage if not treated promptly. Clinical staff should be aware of the various presentations of neurotoxicity related to N2O abuse.

Distribution and temporal trend of polybrominated diphenyl ethers in one Shanghai municipal landfill, China.

The scarcity of information on polybrominated diphenyl ethers' (PBDEs) flow in landfill restricts the life cycle analysis of PBDEs. In this study, eight PBDE congeners (BDEs 28, 47, 99, 100, 153, 154, 183, and 209) in topsoil, vegetation leaves, leachate, and municipal aged refuse collected from Shanghai Laogang Municipal Landfill (SLML) were investigated. The present study revealed elevated PBDE concentrations in topsoil and proved PBDE leakage from SLML and vegetation uptake. BDE-209 was the predominant congener, and this could be due to massive usage of deca-BDE mixture in Shanghai. ΣPBDE concentrations in leachates treated by reed wetland and A(2)/O process fell in the low end of the worldwide range. ΣPBDE concentrations in aged refuse samples rose from under 50 ng/g dw in 1989 to the range of 5,150-5,718 ng/g dw in 2002. PBDE concentrations increase in aged refuse samples throughout the 1990s into the 2000s paralleled municipal solid waste output from 1991 to 2002 in Shanghai. Exponential increase in BDE-209 concentration in aged refuse suggested the increasing market demands for deca-BDE mixture after 1990 in China. Notably, the inventory of PBDEs in SLML was 28.7 MT, and the doubling time of BDE-209 in aged refuse was calculated to be 1.6 year. SLML can be considered as a source of PBDE and one main recipient of PBDE as well, receiving inputs predominantly from the PBDE-containing waste. Priority should be given to formulate regulation on PBDEs and sorting work before landfill disposal.

Treatment of ureaplasma urealyticum infection patients of Qi deficiency blood stasis syndrome by pengyan pill: a clinical observation / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To observe the clinical efficacy of penyan pill (PP) in treating ureaplasma urealyticum (UU) infection patients of qi deficiency blood stasis syndrome (QDBSS).</p><p><b>METHODS</b>Totally 188 UU infection patients of QDBSS were randomly assigned to two groups, the treatment group and the control group. Patients in the treatment group were treated with PP (10 g each time, thrice daily, 14 consecutive days as one therapeutic course), while those in the control group took azithromycin (10 g each day, 7 consecutive days as one therapeutic course). They were continually treated for 3 therapeutic courses. The clinical symptom integrals were observed in the two groups before and after treatment. The short-term efficacy was judged. Their recurrence rates were followed-up to assess their long-term efficacies.</p><p><b>RESULTS</b>The total effective rate of the comprehensive efficacy in the treatment group was 91.4%, while it was 79. 3%in the control group, showing no statistical difference between the two groups (P > 0.05). Better effects were obtained in improving Chinese medical clinical symptoms in the treatment group (P <0.01). There was no statistical difference in the negative conversion rate between the two groups after treatment (P >0. 05). There was statistical difference in the recurrence rate between the two groups (12. 82% vs 54.76%,P <0. 05).</p><p><b>CONCLUSIONS</b>PP showed equivalent effects in treating UU infection patients of QDBSS to those of azithromycin. But PP showed obvious advantages over azithromycin in improving Chinese medical syndromes.</p>

[Control effect of different covering patterns on indigenous nutrient release from sediment].

A laboratory simulation experiment was conducted to study the release of sediment phosphorous and nitrogen under the effects of coating the sediment with plastic, clinoptilolite, calcite, quartz sand, and calcium nitrate, aimed to provide scientific basis and technical support to control the sediment nutrient release under the background of water environment pollution by different concentrations of nitrogen and phosphorus. The control efficacy of test coating materials for sediment total phosphorous release was in the order of plastic > calcium nitrate > clinoptilolite > calcite > quartz sand, and that for sediment total nitrogen release was in the order of clinoptilolite > plastic > calcite > quartz sand > calcium nitrate. As for the release of sediment NO(3-)-N, the control efficacy of test coating materials was calcium nitrate > quartz sand > clinoptilolite > calcite > plastic coating; whereas for the release of sediment NH(4+)-N, the sequence was calcium nitrate > plastic coating > clinoptilolite > calcite > quartz sand. Water temperature had definite relativity to the sediment nutrient release. With the increase of water temperature, the concentrations of water total phosphorous and nitrogen and NO(3-)-N increased, while the concentration of water NH(4+)-N presented a declining trend.

Toxicological effect of joint cadmium selenium quantum dots and copper ion exposure on zebrafish.

Quantum dots (QDs) have strong adsorption capacity; therefore, their potential toxicity to aquatic organisms from the facilitated transport of other trace toxic pollutants when they coexist has received increasing interest. However, the impact of cadmium selenium (CdSe) QDs and copper ion (Cu(2+)) joint exposure on zebrafish (Danio rerio) embryo and larvae remains almost unknown. Therefore, the present study was performed to determine the developmental toxicities to zebrafish exposed to combined pollution with CdSe QDs (500 µg/L) and Cu(2+) (0, 0.1, 1, 10, and 100 µg/L CuC1(2)) compared with single exposure. Our findings for the first time revealed that: (1) QDs facilitated the accumulation of Cu(2+) in zebrafish; (2) QDs caused higher mortality, lower hatch rate, and more malformations of the exposed zebrafish; (3) junction, bifurcation, crossing, particles, and aggregation of the exposed FLI-1 transgenic zebrafish larvae can be observed; (4) embryo cell apoptosis appeared in the head and tail region; and (5) synergistic effects played an important role during joint exposure. These observations provide a basic understanding of CdSe QDs and Cu(2+) joint toxicity to aquatic organisms and suggest the need for additional research to identify the toxicological mechanism.

Mycoplasma pneumoniae-related postencephalitic epilepsy in children.

PURPOSE: Mycoplasma pneumoniae, a common respiratory pathogen, has been implicated as an etiology of encephalitis, but there are few reports about it and postencephalitic epilepsy. This study aimed to investigate clinical factors, electroencephalography, and neuroradiologic features of M. pneumoniae-related encephalitis in a series of children with postencephalitic epilepsy and to examine possible prognostic factors. METHODS: Cases of M. pneumoniae-related encephalitis between January 2001 and June 2010 were retrospectively reviewed. Systematic clinical data were evaluated. KEY FINDINGS: The 99 enrolled patients with M. pneumoniae-related encephalitis were all positive by serology and 47 (47.5%) of them developed postencephalitic epilepsy. During the acute phase, 53 patients (53.5%) had seizures, the most common type of which was primary focal with secondary generalized tonic-clonic seizure (39.6%). The most common initial electroencephalography was focal/diffuse cortical dysfunction (37.4%) and focal epileptiform discharge (26.4%). The time of follow-up ranged from 6-131 months. At the end of the study, 19 (40.4%) of the 47 children with postencephalitic epilepsy had intractable seizures. SIGNIFICANCE: Postencephalitic epilepsy is not a rare complication of M. pneumoniae-related encephalitis. Seizures in the acute phase and focal epileptiform discharges in initial electroencephalography are significant prognostic factors.

Pott's puffy tumor in children.

INTRODUCTION: Pott's puffy tumor is characterized by subperiosteal abscess associated with osteomyelitis of frontal bone. Reports are limited for this rare entity in the antibiotics era but increase during past decade. METHODS: We had clinical analysis of a series with six consecutive pediatric patients of Pott's puffy tumor during 20 years in a tertiary medical center via retrospective chart review. One case was described in detail. RESULTS: Male-to-female ratio was 5:1. The mean age at the time of diagnosis was 13 years-3 months. The risk factors were acute sinusitis in two (33%), chronic sinusitis in two (33%), recent head trauma in two (33%), and acupuncture therapy on skull in one (17%). The commonest presenting symptoms were fever, headache, forehead tenderness, vomiting, and fatigue/malaise (100%). Pott's puffy tumor was diagnosed on average the seventh day after fever, and half had intracranial involvement at diagnosis. All had intracranial infections, and most of them had subdural empyema. The most often involved sinus was frontal sinus (100%). The frontal lobe was the most common site of intracranial infection (100%), two thirds of which are polymicrobial from two or more sites. The initial operation was performed on average on the 5.8th days after diagnosis. Half of the patients underwent reoperation. The mortality rate was 17% (one of six). CONCLUSION: The symptoms of Pott's puffy tumor are inconspicuous even though early intracranial involvement often occurred. The importance of early diagnosis and aggravated and prompt treatment with prolonged antibiotic therapy is emphasized for better outcome.

Involvement of p38 MAPK phosphorylation and nitrate formation in aristolochic acid-mediated antiplatelet activity.

Aristolochic acid (AsA) is produced from Aristolochia fangchi, and has been used as a Chinese herbal medicine. AsA possesses various biological activities including antiplatelet, antifungal, and anti-inflammatory properties. The aim of this study was to examine the mechanisms of AsA in inhibiting platelet aggregation. AsA (75 - 150 microM) exhibited more-potent activity of inhibiting platelet aggregation stimulated by collagen (1 microg/mL) than other agonists. AsA (115 and 150 microM) inhibited collagen-induced platelet activation accompanied by [Ca+2)]i mobilization, thromboxane A2 (TxA2) formation and phosphoinositide breakdown. On the other hand, AsA also markedly increased levels of NO/cyclic GMP, and cyclic GMP-induced vasodilator-stimulated phosphoprotein phosphorylation. AsA inhibited p38 MAPK but not ERK1/2 phosphorylation in washed platelets. In conclusion, the most important findings of this study suggest that the inhibitory effects of AsA possibly involve the (1) inhibition of the p38 MAPK-cytosolic phospholipase A2-arachidonic acid-TxA2-[Ca+2)]i cascade, and (2) activation of NO/cyclic GMP, resulting in inhibition of phospholipase C. These results imply that Aristolochia fangchi treatment alone or in combination with other antiplatelet drugs, may result in alteration of hemostasis in vivo.

Occult infarct with acute hemorrhagic stroke in juvenile diabetic ketoacidosis.

Diabetes ketoacidosis (DKA) is one of the common complications of type I insulin-dependent diabetes mellitus. Neurological deterioration during an episode of DKA is usually assumed to be caused by cerebral edema and cerebral vascular accidents. However, hemorrhagic stroke is a very rare complication of juvenile DKA. We describe a girl who had newly diagnosed insulin-dependent diabetes mellitus with juvenile DKA developed intracerebral hemorrhage.

Inhibitory mechanisms of tetramethylpyrazine in middle cerebral artery occlusion (MCAO)-induced focal cerebral ischemia in rats.

Tetramethylpyrazine (TMPZ) is an active ingredient isolated from a commonly used Chinese herb, Ligusticum wallichii Franchat, which has long been used in China for the treatment of vascular diseases. In the present study, TMPZ significantly attenuated middle cerebral artery occlusion (MCAO)-induced focal cerebral ischemia in rats. Administration of TMPZ at 10 and 20 mg/kg produced concentration-dependent reductions in infarct size compared to that of control rats. MCAO-induced focal cerebral ischemia was associated with increases in both nitrotyrosine and inducible nitric oxide synthase (iNOS) expression in ischemic regions. The expressions of nitrotyrosine and iNOS were markedly inhibited by TMPZ (20 mg/kg) treatment. Furthermore, TMPZ (100-250 microM) concentration-dependently inhibited respiratory bursts in human neutrophils stimulated by fMLP (800 nM) and PMA (320 nM). TMPZ (100-250 microM) also significantly inhibited neutrophil migration stimulated by fMLP (800 nM) and LTB4 (160 nM). An electron spin resonance (ESR) method was used to further study the scavenging activity of TMPZ on free radicals formed in human neutrophils. TMPZ (100 and 200 microM) greatly reduced the ESR signal intensity of hydroxyl radical formation. In conclusion, we demonstrate a neuroprotective effect of TMPZ in MCAO-induced focal cerebral ischemia in vivo. TMPZ mediates at least part of the free radical-scavenging activity and inhibits neutrophil activation, resulting in a reduction in the infarct volume in ischemia-reperfusion brain injury. Thus, TMPZ treatment may represent an ideal approach to lowering the risk of or improving function in ischemia-reperfusion brain injury-related disorders.