Antagonistic activity of brain networks likely plays a fundamental role in how the brain optimizes its performance by efficient allocation of computational resources. A prominent example involves externally/internally oriented attention tasks, implicating two anticorrelated, intrinsic brain networks: the default mode network (DMN) and the dorsal attention network (DAN). To elucidate electrophysiological underpinnings and causal interplay during attention switching, we recorded intracranial EEG (iEEG) from 25 epilepsy patients with electrode contacts localized in the DMN and DAN. We show antagonistic network dynamics of activation-related changes in high-frequency (> 50 Hz) and low-frequency (< 30 Hz) power. The temporal profile of information flow between the networks estimated by functional connectivity suggests that the activated network inhibits the other one, gating its activity by increasing the amplitude of the low-frequency oscillations. Insights about inter-network communication may have profound implications for various brain disorders in which these dynamics are compromised.
- MeSH
- dospělí MeSH
- elektroencefalografie MeSH
- elektrofyziologické jevy MeSH
- epilepsie patofyziologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mozek * fyziologie patofyziologie MeSH
- nervová síť * fyziologie MeSH
- pozornost * fyziologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- MeSH
- elektroencefalografie MeSH
- lidé MeSH
- status epilepticus * diagnóza etiologie patologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- úvodní články 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
- elektroencefalografie MeSH
- lidé MeSH
- prognóza MeSH
- status epilepticus * diagnóza etiologie farmakoterapie klasifikace MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- kazuistiky MeSH
- přehledy MeSH
OBJECTIVE: Refractory epilepsy may have an underlying autoimmune etiology. Our aim was to assess the prevalence of neural autoantibodies in a multicenter national prospective cohort of patients with drug-resistant epilepsy undergoing epilepsy surgery utilizing comprehensive clinical, serologic, and histopathological analyses. METHODS: We prospectively recruited patients undergoing epilepsy surgery for refractory focal epilepsy not caused by a brain tumor from epilepsy surgery centers in the Czech Republic. Perioperatively, we collected cerebrospinal fluid (CSF) and/or serum samples and performed comprehensive commercial and in-house assays for neural autoantibodies. Clinical data were obtained from the patients' medical records, and histopathological analysis of resected brain tissue was performed. RESULTS: Seventy-six patients were included, mostly magnetic resonance imaging (MRI)-lesional cases (74%). Mean time from diagnosis to surgery was 21 ± 13 years. Only one patient (1.3%) had antibodies in the CSF and serum (antibodies against glutamic acid decarboxylase 65) in relevant titers; histology revealed focal cortical dysplasia (FCD) III (FCD associated with hippocampal sclerosis [HS]). Five patients' samples displayed CSF-restricted oligoclonal bands (OCBs; 6.6%): three cases with FCD (one with FCD II and two with FCD I), one with HS, and one with negative histology. Importantly, eight patients (one of them with CSF-restricted OCBs) had findings on antibody testing in individual serum and/or CSF tests that could not be confirmed by complementary tests and were thus classified as nonspecific, yet could have been considered specific without confirmatory testing. Of these, two had FCD, two gliosis, and four HS. No inflammatory changes or lymphocyte cuffing was observed histopathologically in any of the 76 patients. SIGNIFICANCE: Neural autoantibodies are a rare finding in perioperatively collected serum and CSF of our cohort of mostly MRI-lesional epilepsy surgery patients. Confirmatory testing is essential to avoid overinterpretation of autoantibody-positive findings.
- MeSH
- autoprotilátky MeSH
- epilepsie * epidemiologie chirurgie komplikace MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- malformace mozkové kůry * komplikace MeSH
- prevalence MeSH
- prospektivní studie MeSH
- refrakterní epilepsie * diagnostické zobrazování chirurgie komplikace MeSH
- retrospektivní studie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
BACKGROUND AND OBJECTIVES: Autoimmune encephalitis (AE) refers to a heterogenous group of inflammatory CNS diseases. Subgroups with specified neural autoantibodies are more homogeneous in presentation, trigger factors, outcome, and response to therapy. However, a considerable fraction of patients has AE features but does not harbor detectable autoantibodies and is referred to as antibody-negative AE. Our aim was to describe clinical features, trigger factors, treatments, and outcome of a cohort of comprehensively tested antibody-negative AE patients. METHODS: This retrospective monocentric study recruited adult patients whose serum and/or CSF was sent to our tertiary center for neural antibody testing between 2011 and 2020, who entered the diagnostic algorithm as possible antibody-negative AE and had the following: (1) probable antibody-negative AE, definite antibody-negative acute disseminated encephalomyelitis (ADEM), or definite autoimmune limbic encephalitis (LE) according to diagnostic criteria; (2) available data on MRI of the brain, CSF, and EEG; and (3) stored serum and/or CSF samples. These samples were reanalyzed using a comprehensive combination of cell-based and tissue-based assays. RESULTS: Of 2,250 patients tested, 33 (1.5%) were classified as possible antibody-negative AE. Of these, 5 were found to have antibodies by comprehensive testing, 5 fulfilled the criteria of probable AE (3F:2M, median age 67, range 42-67), 4 of definite autoimmune LE (2F:2M, median age 45.5, range 27-60 years), one of definite antibody-negative ADEM, 2 of Hashimoto encephalopathy, one had no samples available for additional testing, and 15 had no further categorization. Of 10 probable/definite AE/LE/ADEM, one had a malignancy and none of them received an alternative diagnosis until the end of follow-up (median 18 months). In total, 80% (8/10) of patients received immunotherapy including corticosteroids, and 6/10 (60%) patients received rituximab, azathioprine, cyclophosphamide, plasma exchange, or IV immunoglobulins. Five (50%) patients improved, one (10%) stabilized, one (10%) worsened, and 3 (30%) died. All deaths were considered to be related to encephalitis. We did not observe differences of immunotherapy-treated patients in likelihood of improvement with or without nonsteroidal immunotherapy (with 2/6, without 1/2). DISCUSSION: Antibody-negative AE should be diagnosed only after comprehensive testing. Diagnostic effort is important because many patients benefit from immunotherapy and some have malignancies.
- MeSH
- autoimunitní nemoci nervového systému * diagnóza terapie MeSH
- autoprotilátky MeSH
- dospělí MeSH
- encefalitida * diagnóza terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory * MeSH
- retrospektivní studie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND AND PURPOSE: N-methyl-d-aspartate receptor (NMDAR) and leucine-rich glioma-inactivated protein 1 (LGI1) encephalitis are important types of autoimmune encephalitis (AE) with significant morbidity. In this study, we used a proteomic approach in search of novel clinically relevant biomarkers in these types of encephalitides. METHODS: Swedish and Czech tertiary neuroimmunology centers collaborated in this retrospective exploratory study. Fifty-eight cerebrospinal fluid (CSF) samples of 28 patients with AE (14 definite NMDAR, 14 with definite LGI1 encephalitis) and 30 controls were included. CSF samples were analyzed using proximity extension assay technology (Olink Target 96 Inflammation panel). For each CSF sample, 92 proteins were measured. Clinical variables were retrospectively collected, and correlations with protein levels were statistically analyzed. RESULTS: Patients and controls differed significantly in the following 18 biomarkers: TNFRSF9, TNFRSF12, TNFRSF14, TNFβ, TNFα, IL7, IL10, IL12B, IFNγ, CD5, CD6, CASP8, MMP1, CXCL8, CXCL10, CXCL11, IL20RA, and sirtuin 2 (SIRT2). In LGI1 encephalitis, no clinically useful association was found between biomarkers and clinical variables. In the NMDAR encephalitis group, SIRT2, TNFβ, and CD5 were significantly associated with ovarian teratoma. For SIRT2, this was true even for the first patients' CSF sample (SIRT2 without vs. with tumor, mean ± SD = 2.2 ± 0.29 vs. 2.88 ± 0.48; p = 0.007, 95% confidence interval = -1.15 to -0.22; r statistic in point-biserial correlation (rpb) = 0.66, p = 0.011). SIRT2 was positively correlated with age (rpb = 0.39, p = 0.018) and total hospital days (r = 0.55, p = <0.001). CONCLUSIONS: SIRT2 should be investigated as a biomarker of paraneoplastic etiology in NMDAR encephalitis.
- MeSH
- autoprotilátky MeSH
- biologické markery mozkomíšní mok MeSH
- encefalitida s protilátkami proti NMDA receptorům * mozkomíšní mok MeSH
- lidé MeSH
- novorozenec MeSH
- proteomika MeSH
- retrospektivní studie MeSH
- sirtuin 2 MeSH
- Check Tag
- lidé MeSH
- novorozenec MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Autoimunitní encefalitidy (AIE) jsou autoimunitně podmíněná onemocnění centrálního nervového systému s dominujícím postižením mozkové kůry. Prevalence AIE je srovnatelná s encefalitidami infekční etiologie. Mohou se vyskytovat jako paraneoplastické syndromy (u kterých je abnormální imunitní odpověď podmíněna přítomností periferního tumoru), nebo jako syndromy neparaneoplastické (kde zřejmě hraje roli kombinace virového triggeru a vrozené dispozice). Mezi nejčastější AIE patří encefalitida s protilátkami proti glutamátovým N‐methyl‐D-aspartátovým recetorům (NMDAR encefalitida), limbická encefalitida s protilátkami proti leucin‐rich glioma inactivated proteinu 1 (LGI1 encefalitida) a některé syndromy dříve označované jako "klasické paraneoplastické" - v dnešní terminologii "vysoce rizikové fenotypy". Jedná se o limbickou encefalitidu s pozitivitou anti‐Hu a anti‐CV2, rychle progredující cerebelární syndrom (většinou sdružený s protilátkami anti‐Yo) a stiff‐person syndrom (včetně jeho variant). V posledních letech bylo popsáno několik nových fenotypů, které si zaslouží pozornost, např. anti‐GFAP syndrom, anti‐GABAA receptorová encefalitida (relevantní v kontextu new‐onset refractory status epilepticus - NORSE) a syndrom s protilátkami anti‐IgLON5. Tento článek přináší základní přehled aktuálních informací ohledně AIE.
Autoimmune encephalitides (AIE) are autoimmune diseases of the central nervous system with predominant involvement of the cerebral cortex. The prevalence of AIE is comparable to that of encephalitis of infectious etiology. They can occur as paraneoplastic syndromes (in which the abnormal immune response is triggered by the presence of a peripheral tumor) or as non-paraneoplastic syndromes (where a combination of a viral trigger and an innate disposition seems to play a role). The most common AIEs include encephalitis with antibodies to glutamate N-methyl-D-aspartate receptors (NMDAR encephalitis), limbic encephalitis with antibodies to leucine-rich glioma inactivated protein 1 (LGI1 encephalitis) and syndromes formerly referred to as "classic paraneoplastic" - in today's terminology "high-risk phenotypes". These include limbic encephalitis with anti-Hu and anti-CV2 positivity, rapidly progressive cerebellar syndrome (usually associated with anti-Yo antibodies) and stiff-person syndrome (including its variants). In recent years, several new phenotypes have been described that deserve attention, such as anti-GFAP syndrome, anti-GABAA receptor encephalitis (relevant in the context of new-onset refractory status epilepticus - NORSE) and syndrome with anti-IgLON5 antibodies. This article provides a basic overview of current information regarding AIE.
- MeSH
- autoimunitní nemoci nervového systému * diagnostické zobrazování klasifikace komplikace mozkomíšní mok patofyziologie patologie terapie MeSH
- diferenciální diagnóza MeSH
- elektroencefalografie MeSH
- encefalitida s protilátkami proti NMDA receptorům diagnóza farmakoterapie patologie MeSH
- encefalitida * diagnostické zobrazování klasifikace mozkomíšní mok patofyziologie patologie terapie MeSH
- epilepsie temporálního laloku diagnóza etiologie komplikace MeSH
- epilepsie diagnóza etiologie komplikace MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- mozek diagnostické zobrazování MeSH
- nádory nervového systému diagnóza patologie MeSH
- paraneoplastické neurologické syndromy diagnóza klasifikace MeSH
- protilátky analýza MeSH
- rizikové faktory MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
Glial fibrillary acidic protein (GFAP) astrocytopathy, first described in 2016, belongs to the group of autoimmune diseases of the central nervous system. This disease typically manifests around 50 years of age, without sex predilection. Clinical picture is variable, but typically is that of meningoencephalitis. In approximately one fifth of cases, the patient suffers from another autoimmune disease and has a malignancy. Diagnosis is supported with typical findings on brain MRI - T2 hyperintense lesions and perivenular radial enhancement in contrast-enhanced T1 sequence. The disease responds well to immunotherapy, particularly corticosteroids. Relapses occur, mostly with fast detraction of corticotherapy. We present the first case of GFAP astrocytopathy in the Czech Republic.
Gliální fibrilární acidický protein (GFAP) astrocytopatie, poprvé popsaná v roce 2016, patří mezi autoimunitní onemocnění CNS. Toto onemocnění typicky postihuje pacienty kolem padesátého roku věku, podobné četnosti u mužů i žen. Klinický obraz může být různorodý, ale typicky se manifestuje jako meningoencefalitida. V pětině případů je asociovaná s tumorem a jiným autoimunitním onemocněním v anamnéze. Diagnózu podporuje typický nález na magnetické rezonanci mozku - T2 hyperintenzní léze a perivenulární radiální sycení v T1 obraze po podání gadolinia. Toto onemocnění dobře odpovídá na imunoterapii, a to zejména terapii kortikosteroidy. Relapsy jsou popisovány zejména při ryché detrakci kortikoterapie. Prezentujeme kazuistiku pacienta ve věku 45-49 let jako prvního zachyceného případu v České republice.
- MeSH
- cyklofosfamid aplikace a dávkování MeSH
- diferenciální diagnóza MeSH
- gadolinium terapeutické užití MeSH
- gliový fibrilární kyselý protein MeSH
- hormony kůry nadledvin aplikace a dávkování MeSH
- intravenózní imunoglobuliny aplikace a dávkování MeSH
- lidé středního věku MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- meningoencefalitida * diagnostické zobrazování farmakoterapie imunologie mozkomíšní mok patologie MeSH
- mozek diagnostické zobrazování patologie MeSH
- spinální punkce MeSH
- výsledek terapie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- Publikační typ
- kazuistiky MeSH
OBJECTIVE: Focal cortical dysplasia (FCD) is the most common malformation causing refractory focal epilepsy. Surgical removal of the entire dysplastic cortex is crucial for achieving a seizure-free outcome. Precise presurgical distinctions between FCD types by neuroimaging are difficult, mainly in patients with normal magnetic resonance imaging findings. However, the FCD type is important for planning the extent of surgical approach and counselling. METHODS: This study included patients with focal drug-resistant epilepsy and definite histopathological FCD type I or II diagnoses who underwent intracranial electroencephalography (iEEG). We detected interictal epileptiform discharges (IEDs) and their recruitment into repetitive discharges (RDs) to compare electrophysiological patterns characterizing FCD types. RESULTS: Patients with FCD type II had a significantly higher IED rate (p < 0.005), a shorter inter-discharge interval within RD episodes (p < 0.003), sleep influence on decreased RD periodicity (p < 0.036), and longer RD episode duration (p < 0.003) than patients with type I. A Bayesian classifier stratified FCD types with 82% accuracy. CONCLUSION: Temporal characteristics of IEDs and RDs reflect the histological findings of FCD subtypes and can differentiate FCD types I and II. SIGNIFICANCE: Presurgical prediction of FCD type can help to plan a more tailored surgical approach in patients with normal magnetic resonance findings.
- MeSH
- Bayesova věta MeSH
- elektroencefalografie škodlivé účinky MeSH
- elektrokortikografie škodlivé účinky MeSH
- epilepsie * chirurgie MeSH
- fokální kortikální dysplazie * MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- malformace mozkové kůry * diagnostické zobrazování chirurgie komplikace MeSH
- refrakterní epilepsie * diagnostické zobrazování chirurgie etiologie MeSH
- retrospektivní studie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
