The distribution and morphology of neuronal degeneration were observed and analyzed in each sector of the zona incerta in a lithium‐pilocarpine (LiCl) Wistar rat model of status epilepticus in 12, 15, 18, 21, and 25‐day‐old rats and survival intervals of 4, 8, 12, 24, and 48 hours. Status epilepticus was induced via intraperitoneal (IP) injection of LiCl (3 mmol/kg) 24 hours before an injection of pilocarpine (40 mg/kg, IP). Motor seizures were suppressed by paraldehyde (0.3‐0.6 ml/kg, IP) two hours after status epilepticus onset. Animals were anesthetized using urethane and perfused with phosphate‐buffered saline followed by 4% paraformaldehyde. Brains were sectioned and Nissl stained for map guidance, with fluoro‐Jade B fluorescence used to detect degenerated neurons. Fluoro‐jade B‐positive neurons were plotted to a standard stereotaxic atlas, their distribution was quantified, and their long‐axis diameter was measured. Fluoro‐jade B‐positive neurons were found in pups aged 15 days and older 24 hours after status epilepticus, in which their numbers increased, and their perikaryon size decreased with advancing age. Thus, neuronal damage severity was dependent on age and survival interval. Neuronal damage was only found in the rostral sector of the zona incerta, a region that exhibits a small number of inhibitory neurons and is reciprocally connected to the limbic cortex. This system of hyperactivity, coupled with inhibitory neurons, may be the underlying cause of the neuronal degeneration and explain why it was confined to the rostral sector of the zona incerta.
- MeSH
- Lithium Chloride toxicity MeSH
- Nerve Degeneration * pathology etiology MeSH
- Fluoresceins MeSH
- Rats MeSH
- Disease Models, Animal MeSH
- Neurons * pathology MeSH
- Animals, Newborn MeSH
- Pilocarpine toxicity MeSH
- Rats, Wistar MeSH
- Status Epilepticus * pathology chemically induced complications MeSH
- Age Factors MeSH
- Zona Incerta * pathology MeSH
- Animals MeSH
- Check Tag
- Rats MeSH
- Male MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
This study investigated the striatopallidal complex's involvement in status epilepticus (SE) caused by morphological neurodegenerative changes in a post-natal immature developing brain in a lithium-pilocarpine male Wistar albino rat model of mesial temporal lobe epilepsy. One hundred experimental pups were grouped by age as follows: 12, 15, 18, 21, and 25 days. SE was induced by lithium-pilocarpine. Brain sections were microscopically examined by Fluoro-Jade B fluorescence stain at intervals of 4, 12, 24, and 48 h and 1 week after SE. Each interval was composed of four induced SE pups and a control. Fluoro-Jade B positive neurons in the dorsal striatum (DS) were screened and plotted on stereotaxic rat brain maps. The DS showed consistent neuronal damage in pups aged 18, 21, and 25 days. The peak of the detected damage was observed in pups aged 18 days, and the start of the morphological sequela was observed 12 h post SE. The neuronal damage in the DS was distributed around its periphery, extending medially. The damaged neurons showed intense Fluoro-Jade B staining at the intervals of 12 and 24 h post SE. SE neuronal damage was evidenced in the post-natal developing brain selectively in the DS and was age-dependent with differing morphological sequela.
- MeSH
- Corpus Striatum * pathology metabolism MeSH
- Epilepsy, Temporal Lobe * pathology chemically induced MeSH
- Rats MeSH
- Disease Models, Animal MeSH
- Neurons pathology metabolism MeSH
- Pilocarpine MeSH
- Rats, Wistar MeSH
- Status Epilepticus * pathology chemically induced MeSH
- Animals MeSH
- Check Tag
- Rats MeSH
- Male MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
Focal cortical dysplasia (FCD) represents the most common cause of drug-resistant epilepsy in adult and pediatric surgical series. However, genetic factors contributing to severe phenotypes of FCD remain unknown. We present a patient with an exceptionally rapid development of drug-resistant epilepsy evolving in super-refractory status epilepticus. We performed multiple clinical (serial EEG, MRI), biochemical (metabolic and immunological screening), genetic (WES from blood- and brain-derived DNA), and histopathological investigations. The patient presented 1 month after an uncomplicated varicella infection. MRI was negative, as well as other biochemical and immunological examinations. Whole-exome sequencing of blood-derived DNA detected a heterozygous paternally inherited variant NM_006267.4(RANBP2):c.5233A>G p.(Ile1745Val) (Chr2[GRCh37]:g.109382228A>G), a gene associated with a susceptibility to infection-induced acute necrotizing encephalopathy. No combination of anti-seizure medication led to a sustained seizure freedom and the patient warranted induction of propofol anesthesia with high-dose intravenous midazolam and continuous respiratory support that however failed to abort seizure activity. Brain biopsy revealed FCD type IIa; this finding led to the indication of an emergency right-sided hemispherotomy that rendered the patient temporarily seizure-free. Postsurgically, he remains on antiseizure medication and experiences rare nondisabling seizures. This report highlights a uniquely severe clinical course of FCD putatively modified by the RANBP2 variant. PLAIN LANGUAGE SUMMARY: We report a case summary of a patient who came to our attention for epilepsy that could not be controlled with medication. His clinical course progressed rapidly to life-threatening status epilepticus with other unusual neurological findings. Therefore, we decided to surgically remove a piece of brain tissue in order to clarify the diagnosis that showed features of a structural brain abnormality associated with severe epilepsy, the focal cortical dysplasia. Later, a genetic variant in a gene associated with another condition, was found, and we hypothesize that this genetic variant could have contributed to this severe clinical course of our patient.
- MeSH
- Child MeSH
- DNA MeSH
- Epilepsy * complications MeSH
- Focal Cortical Dysplasia * MeSH
- Nuclear Pore Complex Proteins * MeSH
- Humans MeSH
- Midazolam MeSH
- Molecular Chaperones * MeSH
- Brain Diseases * MeSH
- Child, Preschool MeSH
- Disease Progression MeSH
- Drug Resistant Epilepsy * genetics surgery MeSH
- Status Epilepticus * genetics surgery MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Male MeSH
- Child, Preschool MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
Křeče patří mezi akutní stavy, se kterými se u dětí setkáváme poměrně často. Jedná se o symptom, který může mít velmi rozmanitou etiologii. Z různých pohledů můžeme křeče rozdělit na epileptické a neepileptické, na křeče s teplotou a bez teploty, na křeče fokální a generalizované. K rozlišení nám pomůže důkladná anamnéza, fyzikální vyšetření pacienta, pomocné vyšetřovací metody. V přednemocniční a v časné nemocniční péči by měla být u křečujícího dítěte základní diferenciální diagnostika provedena rychle, včas by měla být zahájena adekvátní symptomatická a podpůrná léčba. Jen tak předejdeme trvalému poškození mozkových buněk, které u protrahovaných křečí hrozí.
Seizures are among the acute conditions that we encounter quite often in children. This is a symptom that can have a very diverse etiology. From different points of view, seizures can be divided into epileptic and non-epileptic, into convulsions with temperature and without temperature, into focal and generalized convulsions. A thorough anamnesis, physical examination of the patient, auxiliary examination methods will help us to make the distinction. In pre-hospital and early hospital care, the basic differential diagnosis of a convulsing child should be performed quickly, and adequate symptomatic and supportive treatment should be started in time. This is the only way to prevent permanent damage to brain cells, which is a risk in prolonged seizures.
- MeSH
- Electroencephalography MeSH
- Humans MeSH
- Status Epilepticus * diagnosis etiology pathology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Introductory Journal Article MeSH
Konvulzivní status epilepticus je tonicko-klonický záchvat trvající déle než 5 minut, nebo opakované tonicko-klonické záchvaty v tomto časovém období, mezi nimiž nedochází k návratu vědomí. Iniciálně je léčen benzodiazepiny - diazepamem i. v., midazolamem i. m. nebo podaným bukálně, nebo diazepamem podaným rektálně. V případě selhání terapie benzodiazepiny je indikováno intravenózní podání protizáchvatových léků - levetiracetamu v dávce 60 mg/kg (maximálně 4 500 mg), valproátu v dávce 40 mg/kg (maximálně 3 000 mg), nebo fenytoinu v dávce 20 mg/kg (maximálně 1 500 mg). V článku uvádíme udržovací dávky léků a věnujeme se jejich významným farmakologickým vlastnostem i možným nežádoucím účinkům. Závěrem zmiňujeme další léky potenciálně využitelné při léčbě konvulzivního status epilepticus, které jsou dostupné ve formě pro intravenózní podání - lakosamid a brivaracetam.
Convulsive status epilepticus is the form of a tonic-clonic seizure lasting longer than 5 minutes, or in the case of repeated tonic-clonic seizures within this time period. Initially is treated with benzodiazepines - diazepam i. v., midazolam i. m. or buccal, or diazepam administered rectally. If benzodiazepine therapy fails, intravenous administration of anti-seizure drugs is indicated - levetiracetam at 60 mg/kg (maximum 4500 mg), valproate at 40 mg/kg (maximum 3000 mg), or phenytoin at 20 mg/kg (maximum 1500 mg). In this article, we present maintenance doses of the drugs and discuss their important pharmacological properties and possible adverse effects. Finally, we mention other drugs potentially useful in the treatment of convulsive status epilepticus that are available in a form for intravenous administration - lacosamide and brivaracetam.
- MeSH
- Medication Adherence MeSH
- Anticonvulsants administration & dosage pharmacology classification adverse effects MeSH
- Benzodiazepines administration & dosage classification MeSH
- Humans MeSH
- Status Epilepticus * etiology drug therapy pathology MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Nekonvulzivní status epilepticus (NCSE) je významnou příčinou morbidity a mortality, zvláště vzniká‐li v souvislosti s akutním mozkovým inzultem u pacientů vyžadujících neurointenzivní péči. Jedná se o heterogenní skupinu stavů a klinické projevy, nález na EEG, léčba a prognóza se velmi liší podle klinického kontextu, ve kterém NCSE vzniká - věk, přítomnost epilepsie, komorbidity, komedikace, přítomnost akutní strukturální léze mozku nebo akutní systémové poruchy. Pro diagnostiku NCSE je nezbytné EEG, ale zejména u pacientů s akutním symptomatickým NCSE existuje pestrá paleta EEG nálezů, které neumožňují s jistotou určit, zda jsou způsobeny samotnou záchvatovou aktivitou, nebo vyvolávající lézí - tzv. iktálně interiktální kontinuum. Léčba NCSE může být náročná a vyžaduje úzkou spolupráci klinika s elektroencefalografistou. Zásadní je včasné odhalení NCSE a jeho etiologie a správně vedená léčba.
Non-convulsive status epilepticus (NCSE) is an important cause of morbidity and mortality, especially in patients with acute cerebral insults and those in need of neurointensive care. NCSE is a clinically heterogeneous group of conditions and the clinical manifestations, EEG findings, treatment and prognosis vary widely according to the clinical context in which NCSE occurs (age, presence of epilepsy, comorbidities, comedication, presence of an acute structural brain lesion or acute systemic disorder). EEG is essential for the diagnosis of NCSE. However, especially in patients with acute symptomatic NCSE, a wide variety of EEG findings occur that do not allow to determine with certainty whether they are caused by the epileptic activity itself or by the causative lesion - the so-called ictal-interictal continuum. Treatment of NCSE can be challenging and requires close collaboration between clinicians and EEG-readers. Critical is the early identification of NCSE and underlying etiology and properly managed treatment.
- MeSH
- Electroencephalography MeSH
- Humans MeSH
- Prognosis MeSH
- Status Epilepticus * diagnosis etiology drug therapy classification MeSH
- Check Tag
- Humans MeSH
- Publication type
- Case Reports MeSH
- Review MeSH
V tomto článku podáváme definici refrakterního a superrefrakterního status epilepticus (SE). Evidence‐based či konzensuální léčebné strategie neexistují ani pro refrakterní SE. U super‐refrakterního SE jsou léčebná doporučení ještě neurčitější. Z tohoto důvodu uvádíme i způsoby léčby, jejichž výsledky byly publikovány pouze na malých souborech pacientů nebo v kazuistikách. Tyto informace mohou přispět k vytvoření institucionálních léčebných protokolů.
In this article, we give definitions of refractory and super-refractory status epilepticus (SE). Evidence-based or consensual treatment strategies are not known even for refractory SE. In super-refractory SE, the treatment options are even more ambiguous. Therefore, we also present the results of methods published in small case series or case reports. Potentially, this information may contribute to the development of in‐house treatment protocols.
- MeSH
- Anesthetics administration & dosage adverse effects MeSH
- Drug Resistance MeSH
- Humans MeSH
- Status Epilepticus * epidemiology drug therapy therapy MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Nově vzniklý refrakterní status epilepticus (new‐onset refractory status epilepticus, NORSE) je vzácně se vyskytující závažný klinický syndrom s možnými fatálními důsledky, jehož etiologie často zůstává neobjasněna. NORSE je ve své podstatě popisný termín, který se vztahuje ke klinickému obrazu a absenci anamnézy epilepsie, nikoliv ke konkrétní etiologii stavu. Přestože stejně jako u jakéhokoliv jiného epileptického statu (SE) je naší prioritou zvládnutí záchvatové aktivity, pátrání po vyvolávající příčině má velký význam pro správné vedení terapie a pro prognostifikaci pacienta. V tomto článku přinášíme z literatury vyplývající doporučené diagnostické a terapeutické postupy v závislosti na předpokládané/potvrzené etiologii NORSE.
New-onset refraktory status epilepticus (NORSE) is a rare severe condition with possible fatal consequences. Etiology of the syndrome often remains unclear. The term NORSE describes the clinical condition according to patient's symptoms and history but has no connection with a specific etiology. Achievement of the control of seizures is our main priority in management of every status epilepticus (SE), however finding the etiology of SE has both therapeutic and prognostic implications. Aim of this article is to suggest diagnostic and therapeutic processes based on the considered/confirmed underlying cause of NORSE.
- MeSH
- Drug Resistance MeSH
- Humans MeSH
- Status Epilepticus * diagnosis etiology drug therapy therapy MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Status epilepticus (SE) v dětském věku se oproti dospělým vyznačuje vyšší incidencí, odlišnými příčinami a nižším rizikem úmrtí. Většina případů SE u dětí se objevuje bez předchozí historie epileptických záchvatů. Etiologie SE u dětí se liší, přičemž febrilní SE je u této věkové skupiny nejčastější. Specifické pro dětský věk jsou SE u některých epileptických syndromů, například u syndromu Dravetové a syndromu Lennox‐Gastautova. Léčba SE u dětí využívá podobné algoritmy jako u dospělých, ale s určitými specifiky, jako je např. použití vitaminu B6 a preference midazolamu nebo thiopentalu před propofolem. Mortalita spojená se SE je u dětí nižší (0-16 %) a přímo závisí na typu SE a jeho příčině.
In comparison to adults, status epilepticus (SE) in childhood is characterized by a higher incidence, different causes, and a lower mortality. Most cases of SE in children occur without a prior history of epileptic seizures. The etiology of SE in children is distinct, with febrile SE being the most common in this age group. Specific to childhood are SE cases in certain epileptic syndromes, such as Dravet syndrome and Lennox-Gastaut syndrome. The treatment of SE in children employs similar algorithms as in adults, but with certain specifics, such as the use of vitamin B6 and a preference for midazolam or thiopental over propofol in younger patients. The mortality associated with SE in children is lower (0-16%) and directly depends on the type and the etiology of SE.
- MeSH
- Child * MeSH
- Electroencephalography MeSH
- Epilepsy diagnostic imaging classification pathology MeSH
- Epileptic Syndromes diagnostic imaging classification pathology MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Brain diagnostic imaging pathology MeSH
- Prognosis MeSH
- Status Epilepticus * diagnostic imaging etiology MeSH
- Check Tag
- Child * MeSH
- Humans MeSH
- Publication type
- Review MeSH
