N‑metyl‑D‑aspartát receptorová (NMDAR) encefalitida je nedávno definované autoimunitní postižení centrálního nervového systému (CNS). Podstatou neurologických potíží je porucha synaptického přenosu v důsledku internalizace NMDA receptorů po navázání autoprotilátek. V následujícím sdělení přinášíme nejnovější informace o onemocnění a jeho diagnostice. Současně dokládáme klinický průběh, strategii léčby a sledování našich pěti pacientek., N-methyl-D-aspartate encephalitis is a recently defined autoimmune disorder of the central nervous system. Neurological symptoms are associated with antibody-dependent internalization of NMDA receptors that leads to synapse disruption. Information about the disease, therapy and diagnosis are followed by clinical experience with our group of five patients., and Z. Libá, J. Hanzalová, V. Sebroňová, V. Komárek
Spatiotemporal dynamics of event-related potentials (ERP) evoked by non-target stimuli in a visual oddball experiment and the presence of coherent oscillations in beta 2 frequency band of decomposed EEG records from peristimulus period were investigated by means of intracranial electrodes in humans. Twenty-one patients with medically intractable epilepsy participated in the study. The EEG signal was recorded using platinum electrodes implanted in several cortical and subcortical sites. Averaged 2 s EEG records were analyzed. Task-specific EEG changes were found in each patient, ERPs were derived from 92 electrodes used (96 % of possible cases). In the majority of analysed cases, ERPs were composed of several distinct components, and their duration was mostly longer than 1 s. The mean onset of the first ERP component was 158±132 ms after the stimulus (median 112 ms, minimum value 42 ms, maximum value 755 ms), and large variability of these onset times was found in all the investigated structures. Possible coherence between neural activities of remote brain sites was investigated by calculating running correlations between pairs of decomposed EEG records (alpha, beta 1, beta 2 frequency bands were used, total number of correlated pairs was 662 in each frequency band). The record pairs exhibiting highly correlated time segments represented 23 % of all the investigated pairs in alpha band, 7 % in beta 1 band, and 59 % in beta 2 band. In investigated 2 s record windows, such segments were distributed evenly, i.e. they were also found before the stimulus onset. In conclusion, the results have implicated the idea that a lot of recorded ERPs was more or less by-products of chance in spreading a signal within the neuronal network, and that their functional relevance was somewhat linked with the phenomenon of activity synchronization., M. Kukleta ... [et al.]., and Obsahuje seznam literatury
Úvod: Protilátky proti membránovým a synaptickým antigenům jsou přímo patogenní protilátky sdružené s pestrou paletou neurologických syndromů. Různorodost příznaků komplikuje stanovení diagnózy, a tím i včasné zahájení imunoterapie. Cíl: Cílem práce bylo popsat klinické charakteristiky pacientů s pozitivitou uvedených protilátek. Metodika a soubor: V období dvou let jsme zjišťovali přítomnost protilátek proti membránovým a synaptickým antigenům u 224 pacientů. Metodou nepřímé imunofluorescence byly vyšetřeny protilátky proti N‑metyl‑D‑aspartátovému receptoru (anti‑NMDAR), α‑amino‑3-hydroxy‑5-metyl‑4-izoxazolepropionátovému receptoru (anti‑AMPAR 1 a 2), B‑receptoru kyseliny γ‑aminomáselné (anti‑GABABR), proti leucin‑rich‑glioma‑inactivated proteinu‑1 (anti‑LGI1) a proti contactin‑associated proteinu‑2 (anti‑CASPR2). U pozitivních pacientů byly retrospektivně zhodnoceny klinické projevy, nálezy pomocných metod a vývoj onemocnění. Pacienti s pozitivitou anti‑NMDAR protilátek nebyli do hodnocení zahrnuti. Výsledky: Autoprotilátky byly prokázány v séru 11 pacientů (medián 58 let, sedm mužů) – u šesti pacientů anti‑LGI1, u čtyř anti‑CASPR2, u dvou anti‑AMPAR1 (u jednoho pacienta současně anti‑AMPAR1 a anti‑CASPR2). Příznaky zahrnovaly epilepsii (n = 5), subakutní encefalopatii (n = 5), doprovázenou ve čtyřech případech epileptickými záchvaty, a mozečkový syndrom s doprovodným kognitivním deficitem (n = 1). U dvou pacientů byly současně přítomny dobře charakterizované onkoneurální protilátky (anti‑Hu, anti‑Ma2). Nádor (malobuněčný karcinom plic) byl zjištěn u jedné pacientky (anti‑AMPAR1). U osmi pacientů došlo po podání imunosuprese k remisi nebo částečnému zlepšení klinického stavu. Závěr: Přítomnost protilátek proti membránovým antigenům je sdružena nejčastěji s limbickou encefalitidou nebo fokální epilepsií, vzácněji s jinými neurologickými syndromy. Efekt imunoterapie závisí na jejím časném podání., Background: Neuronal surface antibodies are associated with numerous neurological symptoms. Better knowledge of these symptoms may improve identification of potential candidates for immunotherapy. Aim: Characterize clinical signs in patients with neuronal surface antibodies positivity. Methods: We detected neuronal surface antibodies in 11/2011–12/2013 in 224 patients (224 in serum and 37 in cerebrospinal fluid). We investigated anti-NMDAR, anti-AMPAR1, anti-AMPAR2, anti-GABABR, anti-LGI1, anti-CASPR2 using cell-based assays for indirect immunofluorescence (Euroimmun AG). We retrospectively analyzed clinical characteristics of patients with positive neuronal surface antibodies in serum or cerebrospinal fluid other than anti-NMDAR positive patients. Results: Neuronal surface antibodies were detected in 11 patients (seven males, median age 58). Six patients had anti-LGI1, four anti-CASPR2 and two anti-AMPAR1 antibodies (one patient had both anti-CASPR2 and anti-AMPAR1 antibodies). Clinical symptoms included chronic epilepsy (n = 5), acute encephalopathy (n = 5) accompanied by epileptic seizures in four patients and one patient presented with cerebellar syndrome and cognitive deficit. Two patients had coincidence of paraneoplastic antibodies (anti-Hu, anti-Ma2). Tumor (small cell lung carcinoma) was diagnosed in one patient (anti-AMPAR1). Eight patients improved following immunotherapy (corticosteroids, IVIG). Early immunotherapy was associated with better outcome. Conclusion: NS-Abs were mostly associated with limbic encephalitis and chronic temporal lobe epilepsy. Immunotherapy had better effect when applied early in the disease course. Key words: autoimmune diseases – encephalitis – antibodies to cell-surface proteins – limbic encephalitis The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers., and M. Elišák, D. Krýsl, J. Hanzalová, I. Gažová, I. Doležalová, J. Slonková, P. Marusič
The saccadic eye movement related potentials (SEMRPs) enable to study brain mechanisms of the sensorimotor integration. SEMRPs provide insight into various cognitive mechanisms related to planning, programming, generation and execution of the saccadic eye movements. SEMRPs can be used to investigate pathophysiological mechanisms of several disorders of the central nervous system. Here we shortly summarize basic findings concerning the significance of SEMRP components, their relationship to the functional brain asymmetry and visual attention level as well as changes related to certain neuropsychological disorders., F. Jagla, M. Jergelová, I. Riečanský., and Obsahuje bibliografii a bibliografické odkazy
The purpose of this study was to investigate the possible relationship between Toxoplasma gondii (Nicolle et Manceaux, 1908) and stuttering. We investigated the seropositivity rate for anti-T. gondii IgG and antibodies by enzyme-linked immunosorbent assay (ELISA) in stuttering children to ascertain a possible relationship between T. gondii infection and stuttering. We selected 65 stuttering children and 65 control children (non-stutterers) to investigate the seropositivity rate of anti-T. gondii antibodies by ELISA. Cranial magnetic resonance imaging (MRI) and scalp electroencephalography (EEG) were also performed in stuttering children. The seropositivity rate of anti-T. gondii IgG antibodies among stuttering children (28%) was significantly higher than in control group (5%; p = 0.001). No abnormality was detected in cranial MRI's of stutttering children and their EEG recordings were also normal. There was no significant difference in seropositivity rate regarding age, genders and residence area. The association between seroprevalence of infection with T. gondii and stuttering may be due to hyperdopaminergic state in brains of patients who are T. gondii-seropositive. Thus, there might be a causal relationship between toxoplasmosis and stuttering., Tuncay Çelik, Cem Gökçen, Özgür Aytaş, Aysima Özçelik, Mustafa Çelik, Nurdan Çoban., and Obsahuje bibliografii