In vivo and in vitro effects of melatonin or ganglioside GT1B on L-cysteine-induced brain mitochondrial DNA damage in mice.

The effects of L-cysteine on mitochondrial DNA (mtDNA) in mouse brain were investigated both in vivoandin vitro. An intracerebroventricular (icv) injection of L-cysteine (1.25 micromol/animal) caused mtDNA damage in brain frontal and central portions of the cortex, broad-spectrum limbic and severe sustained seizures in mice, and increased lipid peroxidation in the whole brain. The L-cysteine-mediated effects were prevented by an intraperitoneal (ip) preinjection of melatonin (20 mg/kg) or an intracerebroventricular preinjection of ganglioside GT1b (90 nmol/animal). Furthermore, in in vitroexperiments, L-cysteine (0.05, 0.5, or 1.0 mM) caused damage to brain mtDNA and increased lipid peroxidation in a concentration-dependent manner when incubated at 37 degrees C for 20 or 60 min with a homogenate prepared from whole mouse brains. However, the mtDNA damage and the increased lipid peroxidation were completely abolished by a cotreatment with melatonin (1.5 mM), a potent scavenger of the hydroxyl radical (*OH), or ganglioside GT1b (60 microM), a potent inhibitor of glutamate-receptor-mediated activation and translocation of protein kinase C and lipid peroxidation. These results suggest that reactive oxygen species including the *OH may be involved in l-cysteine-induced brain mtDNA damage, lipid peroxidation, and development of seizures in mice. Therefore, we concluded that *OH scavengers, such as the pineal hormone melatonin and ganglioside GT1b, can protect against brain mtDNA damage, seizures, and lipid peroxidation induced by reactive oxygen species producers such as L-cysteine.     

Proteus syndrome

Abstract: This female Asian (Malay) baby had clinical features of Proteus syndrome. She had a large right facial lipolymphangioma with hyperpigmentation of the overlying skin. There was a smaller lymphangioma over the left side of her neck with excess nuchal folds, macrodactyly and bilateral talipes equinovarus. Despite the extensive hemifacial swelling, there was no evidence of upper respiratory tract obstruction. Generalized seizures developed on the sixth day of life which were controlled with phenobarbital. The lymphangiomas were excised without recurrence.     

Febrile seizures: treatment and prognosis

Recent epidemiologic data indicate that the vast majority of children with febrile seizures have a normal longterm outcome. A precise knowledge of the short- and long-term outcome with or without treatment, and short- and long-term side effects is an important prerequisite for assessing the various treatment strategies. We focus on the impact of short-term or prophylactic treatment on the short- and long-term outcome of various types of febrile seizures. There is universal agreement that daily prophylaxis with antiepileptic agents should never be used routinely in simple febrile seizures, but only in highly selected cases, if at all. Intermittent diazepam (DZP) prophylaxis at times of fever may or may not reduce the recurrence rate, but it does not appear to improve the long-term outcome as compared with short-term seizure control. The treatment may be used to reduce the recurrence rate for a small arbitrarily defined group with multiple simple febrile seizures, complex febrile seizures, especially focal, prolonged or both, febrile status, and when parental anxiety is severe. However, there is no evidence that treatment of simple febrile seizures can prevent the rare cases of later epilepsy, and many children with complex febrile seizures have a benign long-term outcome, even without treatment. Many prefer a "wait and see" policy. An attractive alternative is to treat new febrile seizures with rectal DZP in solution at seizure onset, given by the parents at home to prevent febrile status. Newer, less well documented short-term strategies include nasal, oral, or rectal administration of other benzodiazepines. Short-term seizure control of febrile status and careful parental counseling are the two most important targets of treatment.     

热性惊厥对大鼠情感行为及学习记忆的影响

【目的】　探讨热性惊厥对大鼠情感行为及学习记忆能力的影响。　【方法】　 3 6只雄性SD (Spraque Dawley)大鼠随机分为热性惊厥组 (febrileseizuresgroup ,FS) ,发热对照组 (febrilecontrolgroup ,FG)及正常对照组 (nor malcontrolgroup ,NG)。观察各组大鼠在旷场试验 (openfieldtest ,OFT)、避暗试验 (passiveavoidancetest,PAT)以及在Morris水迷宫 (Morriswatermaze ,MWM )试验中情感行为及学习记忆能力的改变。　【结果】　FS组大鼠与NG、FG组相比 :在OFT中穿格得分分值 ( 3 7.48± 7.0 9)降低 ,后肢性站立的次数 ( 12 .11± 4.41)减少 ,排出粪便粒数 ( 10 .62±2 .3 2 )增多 ,差异有非常显著性 (P <0 .0 1) ;在PAT中 ,FS组大鼠犯错次数 ( 4 .0 2± 1.2 9)增加 ,且记忆潜伏期 [( 169.71± 10 3 .2 2 )秒 ]缩短 ,差异有非常显著性 (P <0 .0 1) ;在Morris水迷宫试验中 ,FS组大鼠搜寻站台的逃逸潜伏期明显延长 ,差异有非常显著性 (P <0 .0 1)。　【结论】　多次热性惊厥发作可使大鼠的情感行为和学习记忆能力受损     

卡马西平和托吡酯联合治疗复杂部分性癫痫29例

目的观察卡马西平和托吡酯联合治疗复杂部分性癫痫的临床疗效及安全性.方法对29例患儿在单用卡马西平治疗的基础上,加用托吡酯联合治疗.结果加用托吡酯后疗效明显,总有效率达69%,发作完全控制率占38%.结论研究结果表明,两药联合治疗复杂部分性癫痫是安全有效的.     

Nursing a canine with presumptive tremorgenic mycotoxicosis following ingestion of mouldy dog food – a case report

A one-year-old neutered female Labrador Retriever presented with twitches and subsequent generalised tonic–clonic seizure activity following ingestion of mouldy dog food. Further complicating factors were hyperthermia and metabolic acidosis. Initial emergency treatment involved seizure management, blood analysis, intravenous fluid therapy, active cooling and decontamination via gastric lavage, administration of activated charcoal and an intralipid infusion. The patient was maintained under general anaesthesia to manage the seizure activity. Post-anaesthetic management included treatment of aspiration pneumonia, physiotherapy, intravenous fluid therapy and management of an indwelling urinary catheter. The patient was discharged 48 h post-admission without further complication.     

Inflammatory mediators in human epilepsy: A systematic review and meta-analysis

BackgroundAccumulating evidence suggests a role for inflammation in the pathophysiology of epilepsy.MethodsWe performed a systematic review and meta-analysis of studies that investigated inflammatory mediators in human epilepsy. Studies reporting on inflammatory mediators in serum, cerebrospinal fluid or brain tissue of epilepsy patients were included. Studies comparing patients to controls were included in a meta-analysis.Results66 articles reporting on 1934 patients were included. IL-1ra, IL-1β, IL-6, IL-10, IFN-γ and TNF-α were the most extensively investigated proteins. Elevated levels for IL-1ra, IL-1β, IL-6 and CXCL8/IL-8 were reported in several different epilepsy etiologies and media, while other proteins were specifically increased for one etiology. IL-1α, IL-7 and IL-13, as well as the chemokines CCL2-5, −19 and −22, were increased exclusively in brain tissue. In an aggregate meta-analysis, we found significantly different protein levels for serum IL-6, IL-17 and CSF IL-1β and IL-10.ConclusionInflammatory pathways are involved in epilepsy. Future studies may further clarify their role, and prove potential of targeted anti-inflammatory treatment.     

Treatment of drug-resistant epilepsy in patients with periventricular nodular heterotopia using RNS® System: Efficacy and description of chronic electrophysiological recordings

ObjectivesDescribe changes in clinical seizure frequency and electrophysiological data recorded in patients with medically-intractable seizures and periventricular nodular heterotopias (PVNH) treated with the RNS® System (NeuroPace, Inc., Mountain View, CA).MethodsClinical seizures from eight patients (mean follow-up of 10.1 years) were analyzed pre- and post-treatment. Chronic ambulatory electrocorticograms (ECoGs) recorded from PVNHs, hippocampus and neocortex were evaluated to identify the earliest electrographic seizure onset type, pattern of spread, and interictal characteristics.ResultsMean reduction in disabling seizures was 85.7 % (n = 8); seven patients had >50% seizure reduction and two were seizure-free in the final year of analysis. Seizure rate showed a progressive reduction over the course of the study with the highest rate of improvement in the first two to three years after implantation. Four of seven patients with one PVNH lead and a second lead in the hippocampus or neocortex had some electrographic seizures first recorded at either lead location, suggesting two foci or seizure propagation patterns. Low voltage fast type activity was the prominent seizure onset pattern. Interictal ECoG power was lower in PVNH than hippocampus.ConclusionsRNS® System treatment substantially reduced clinical seizure frequency in patients with PVNH. Analysis of ictal ECoG records suggests PVNH may be involved in seizure generation.SignificanceChronic ECoG recordings suggest PVNH tissue can actively participate in epileptogenic networks. Direct brain-responsive neurostimulation is a safe and effective treatment option in such patients, progressively reducing seizure rate over a period of years.