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S-Ketamine Preserves Oxidative Metabolism and Reduces Metabolic Stress During Neuronal Hyperexcitability.

S-ketamine is increasingly used in anesthesia and neurocritical care and has been associated with anticonvulsant and potentially neuroprotective effects. Such neuroprotective effects have been observed particularly in states of severe hyperexcitability, including status epilepticus and spreading depolarizations, in which neuronal energy demand rises sharply and mitochondrial function may become compromised. However, the direct effects of S-ketamine on neuronal oxidative metabolism across different activity states remain incompletely understood. In acute hippocampal slices from 6-12-week-old ma…

Minocycline-induced microglial remodeling restores hippocampal NMDA-dependent synaptic plasticity and reduces anxiety-like behavior in juvenile rats with temporal lobe epileptogenesis.

Microglial activation and neuroinflammation are recognized as key drivers of synaptic dysfunction in temporal lobe epilepsy (TLE), yet the causal mechanisms linking specific microglial phenotypes to neuronal hyperexcitability remain poorly understood, and clinically viable therapeutic strategies to modulate these processes are urgently needed. Using the lithium-pilocarpine model in young male rats (P21-P28), we demonstrate that a 7-day minocycline treatment (100 → 50 mg/kg, i.p.), initiated immediately following status epilepticus, induces robust and selective microglial morphological remodeli…

Neuronal expression of Retinoid-Related Orphan Receptor Gamma (RORγ) and revisiting its role in the Central Nervous System.

Retinoid-related orphan receptor gamma (RORγ) is a lineage-defining transcription factor for T helper 17 (Th17) cells and has long been cons … ectable RORγ expression in the postnatal brain, either at baseline or following pilocarpine-induced status epilepticus. These findings demonstrate that RORγ expression is not restricted to the immune lineage and reveal previously unrecognized developmental expression in the embryonic brain. Consequently, seizures observed following RORγ-Cre-mediated deletion of TSC1 …

The Perilous Journey of Patients With Primary CNS-HLH to Bone Marrow Transplant: A Case Report.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening syndrome of immune dysregulation with an incidence of 1.2-1.5 cases … therapy to specifically target neuroinflammation. Outcomes diverged: Patient 1 developed refractory status epilepticus and died without transplantation, while Patient 2 successfully underwent HSCT and was discharged home. This case report highlights the heterogeneity of CNS involvement in primary HLH and its impact on outcomes. Elevated neopterin and neurologic mani …

Epilepsy in Wolf-Hirschhorn Syndrome: Clinical Insights from a Pediatric Cohort and a Review of the Literature.

Background: Wolf-Hirschhorn syndrome (WHS) is a rare contiguous gene deletion disorder associated with a high incidence of early-onset epile … ple seizure types were common, particularly generalized tonic-clonic and atypical absence seizures. Status epilepticus occurred in 58% of cases, with a high proportion requiring multiple ASMs. Valproic acid and levetiracetam were the most commonly used treatments. Patients with more severe epilepsy tended to have larger deletions (>9 Mb) and poorer developmental out …

Infantile-onset Pompe disease entering adulthood: Insights from 2 decades of enzyme replacement therapy experience.

PURPOSE: This study details the long-term clinical outcomes in adult participants with CRIM-positive infantile-onset Pompe disease treated w … articipants, 8 had survived to adulthood (median age 19.6 years), and 3 died (2 of arrhythmia, 1 of status epilepticus). All survivors began ERT between 0.2 to 6 months of age (7 at 20 mg/kg biweekly; 1 at 40 mg/kg biweekly), with subsequent escalation to 40 mg/kg/week of alglucosidase alfa between ages 8 to 15 years. None of the participants received immune modulat …

A Systematic Review and Clinical Presentation of Central Nervous System Complications of Severe Acute Respiratory Syndrome Coronavirus 2 in Hospitalized Pediatric Patients During the Coronavirus Disease 2019 Pandemic in Israel.

BACKGROUND: Coronavirus disease-associated central nervous system complications (CNS-C) in hospitalized children, especially during the Omic … ith 2.6% (n = 20) experiencing nonfebrile seizures, 1.1% (n = 8) febrile seizures, and 0.7% (n = 5) status epilepticus. More patients with CNS-C experienced seizures during the Omicron wave versus the pre-Omicron period (77.8% vs 41.2%, P = 0.03). Fewer patients were admitted to the intensive care unit in the Omicron wave (7.4%) versus prior waves (7.4% vs 41.2%, P …

First Pediatric Case of Autoimmune Encephalitis Associated With COVID-19 in Costa Rica.

Very few COVID-19-associated autoimmune encephalitis cases have been documented in children. This case report focuses on a previously healthy four-year-old girl who presented to the emergency room of the National Children's Hospital in Costa Rica in a postictal state due to a tonic-clonic seizure that progressed to status epilepticus. She had no previous history of fever or associated trauma. She was considered severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive by epidemiological linkage four weeks prior to the event, and her immunoglobulin G (IgG) levels for SARS-CoV-2 were…

Heidenhain Version of Sporadic Creutzfeldt-Jakob Болезнь: первый случай из Восточной Африки.

Болезнь Спорадик Креуцфельдта-Якоба (SCJD) является редким приионным заболеванием, которое вызывает быстро прогрессирующее смертельное нейродедегенерация. Более редкий вариант SCJD Heidenhain имеет визуальные симптомы и редко упоминается в литературе стран Африки к югу от Сахары. Мы сообщаем о случае с 57-летним мужчиной с трехнедельной историей потери направления при езде домой и визуальных галлюцинаций, описываемых как вид на радугу. Магнитный резонансный снимок (MRI) мозга выявил одностороннее париетозащитное гиперинтенситное сульфатное излучение с ограничением на диффузионно взвешенные изо

Совпадение в аннотации оригинала
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare prion disease that causes rapidly progressive fatal neurodegeneration. The rarer Heidenh … alography (EEG) showed right para-central slowing leading to an initial diagnosis of non-convulsive status epilepticus. He was treated with anti-epileptic medication but was re-admitted less than a month later with worsening spatial memory, aggression, ataxia, dysarthria, myoclonic jerks and a positive startle response, later developing generalised tonic-clonic seiz …

Hyperglycemic Crisis in Patients With Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes (MELAS).

BACKGROUND: Diabetes mellitus is the most commonly encountered endocrinopathy in patients with mitochondrial encephalopathy, lactic acidosis … e children aged four and six years. The hyperglycemic events occurred after surgery, infection, and status epilepticus, respectively. None of the three patients had diabetes mellitus previously based on randomly measured serum glucose levels that were within the normal range before the hyperglycemic crisis. Glycated hemoglobin levels measured during the hyperglycemi …

The Risk of Takotsubo Cardiomyopathy in Acute Neurological Disease.

BACKGROUND: Case series have reported reversible left ventricular dysfunction, also known as stress cardiomyopathy or Takotsubo cardiomyopat … e seen with subarachnoid hemorrhage (odds ratio [OR] 11.7; 95% confidence interval [CI] 10.2-13.4), status epilepticus (OR 4.9; 95% CI 3.7-6.3), and seizures (OR 1.3; 95% CI 1.1-1.5). In a sensitivity analysis including secondary diagnoses of acute neurological diagnoses, associations were also seen with transient global amnesia (OR 2.3; 95% CI 1.5-3.6), meningoence …

MOG antibody-related disorders: common features and uncommon presentations.

Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis ( … ts: patchy spinal cord gadolinium-enhancing lesions, an associated teratoma, and one presented with status epilepticus. MOG-Ab-related disorders shared common clinical and prognostic features, but encompass a spectrum wider than recently reported.

The inhibition of transforming growth factor beta-activated kinase 1 contributed to neuroprotection via inflammatory reaction in pilocarpine-induced rats with epilepsy.

Recently, more and more studies support that inflammation is involved in the pathogenesis of epilepsy. Although TGFβ signaling is involved i … otein expression of TRAF6 and TAK1 were detected in different time points after pilocarpine-induced status epilepticus (SE). 5z-7-oxozeaenol treatment (TAK1 antagonist) was then performed; the changes in TRAF6, TAK1, phosphorylated-TAK1 (P-TAK1), interleukin-1β (IL-1β) levels, neuronal survival and apoptosis, and seizure activity were detected. Our results showed th …

Intravenous methylprednisolone for intractable childhood epilepsy.

BACKGROUND: Steroids have been used for the treatment of certain epilepsy types, such as infantile spasms; however, the use in the treatment … ecognized in 82%. Most children (88%) had daily seizures and 13 (76%) were admitted previously with status epilepticus. The epilepsy was cryptogenic (unknown etiology) in 47% and the seizures were mixed in 41%. Intravenous methylprednisolone was given at 15 mg/kg per day followed by a weaning dose of oral prednisolone for 2-8 weeks (mean 3 weeks). Children were foll …

Neuroprotective effects of imidazenil against chemical warfare nerve agent soman toxicity in guinea pigs.

The chemical warfare nerve agent, soman irreversibly inhibits acetylcholinesterase (AChE) leading to hypercholinergy and seizures which trigger glutamate toxicity and status epilepticus ultimately resulting in neuropathology and neurobehavioral deficits. The standard emergency treatment comprising of anticholinergic, AChE reactivator and anticonvulsant does not completely protect against soman toxicity. We have evaluated imidazenil, a new anticonvulsant imidazo benzodiazepine with high affinity and intrinsic efficacy at α5-, α2-, and α3- but low intrinsic efficacy at α1-containing GABA(A) rece…

Acute human self-poisoning with the N-phenylpyrazole insecticide fipronil--a GABAA-gated chloride channel blocker.

OBJECTIVE: Fipronil, a broad spectrum N-phenylpyrazole insecticide that inhibits GABAA-gated chloride channels, has been in use since the mi … nce is needed to determine whether increased susceptibility to fipronil or larger doses can produce status epilepticus.

Losartan's regulation of PI3K/AKT signaling underlies its combined anti-seizure and antidepressant effects in epileptic rats.

OBJECTIVE: To investigate whether losartan alleviates seizures and depression-like behaviors in epileptic rats, and to explore its potential mechanisms involving the PI3K/AKT signaling pathway and hippocampal neuroprotection. METHODS: Status epilepticus (SE) was induced in Sprague-Dawley rats using lithium-pilocarpine. Post-SE rats were randomized to receive daily intraperitoneal injections of either losartan (60 mg/kg) or saline for 20 days. A control group was also set. Seizure frequency was monitored over a 24-hour period post-intervention. Depression-like behaviors were evaluated through …

Characterizing Onset Limit and Surgical Outcome Factors in Type II Focal Cortical Dysplasia-Related Epilepsy.

OBJECTIVES: To clarify the upper limit of epilepsy onset in focal cortical dysplasia (FCD) Type II (FCD)-related epilepsy and to investigate … , p = 0.03). Focal-to-bilateral-tonic-clonic seizures, focal impaired consciousness seizures, prior status epilepticus, sex, hemisphere, age of epilepsy onset, and age of surgery were not associated with 5-year seizure-free outcome. DISCUSSION: Prior data show that ∼5% of FCDs are discovered incidentally. This study elucidates that the majority of FCD-DRE (includin …

The Expression and Effects of Rev-Erbα in Different Cerebral Regions of Mice With Acute and Chronic Epilepsy.

BACKGROUND: Epileptic seizures exhibit distinct circadian rhythms. Disruption of this rhythm creates a vicious cycle that further exacerbates the seizures. Rev-Erbα is a key circadian regulator that plays a significant role in epilepsy. In this study we aimed to investigate the expression and effects of Rev-Erbα and its agonist SR9009 across different brain regions during acute status epilepticus (SE) and chronic epilepsy (CE). METHODS: C57BL/6J mice were randomly divided into control, epilepsy (SE and CE), and SR9009+epilepsy (SR9009-E) groups. The distribution and expression of Rev-Erbα in …

CA1 parvalbumin interneurons display alterations of myelin patterning driven by microgliosis in temporal lobe epilepsy.

Temporal lobe epilepsy (TLE) is one of the most common forms of epilepsy, characterized by significant reorganization of hippocampal neurona … ly, pharmacological intervention with GW2580, a CSF1R inhibitor, administered for 8 days around the status epilepticus, prevented both PV+ interneuron myelination alterations and changes in oligodendrocyte lineage cell populations by blocking microglial proliferation. These results establish a link between microgliosis and alterations of myelin patterning in CA1 PV+ …