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Agrypnia excitata.

Agrypnia (from the Greek: to chase sleep) excitata (AE) is a syndrome characterized by loss of sleep and permanent motor and autonomic hyperactivation (excitata). Disruption of the sleep-wake rhythm consists in the disappearance of spindle-delta activities, and the persistence of stage 1 non-rapid eye movement (NREM) sleep. Rapid eye movement (REM) sleep persists but fails to stabilize, appearing in short recurrent episodes, isolated, or mixed with stage 1 NREM sleep. Diurnal and nocturnal motor, autonomic and hormonal overactivity is the second hallmark of AE. Of particular interest is the fi

Преступность у пациентов с аутоиммунным энцефалитом: серия дел.

Несмотря на то, что это неврологический синдром, реагирующий на иммунную терапию, у пациентов с аутоиммунным энцефалитом (АЭ) часто проявляются остаточные нейробиозные особенности. Здесь мы сообщаем о преступном поведении как о серьезном и новом постсенспелитическом сообществе. Это ретроспективное когортное исследование охватывало 301 пациента АВ. Пятеро из тех, кто совершил преступления, прошли прямую экспертизу и анализ документации наряду с исследованиями, посвященными автоантибиологии. РЕЗУЛЬТАТЫ: Пять из 301 пациента (1,7%) с AE проявили преступное поведение, включая просмотр детской порн

Encephalitis Associated to Metabotropic Glutamate Receptor 5 (mGluR5) Antibodies in Cerebrospinal Fluid.

A 68-years-old Hispanic man, complained of night sweats, low grade fewer, unexplained weight loss, and memory problems over 3 months. Abdomi … oantibodies was negative. The patient fulfilled the Gultekin diagnostic criteria for paraneoplastic limbic encephalitis and methylprednisolone IV 1g/d for 5 days was given. He recovered rapidly, with progressive improvement in memory and psychomotor agitation. After treatment commenced, results for antibodies to mGluR5 in CSF taken prior to treatment were returned as …

Seizure characteristics and outcomes in patients with neurological conditions related to high-risk paraneoplastic antibodies.

OBJECTIVE: Seizures are a common manifestation of paraneoplastic neurologic syndromes. The objective of this study was to describe the seizu … t least daily (p = .0002), seizures on electroencephalogram (EEG; p = .03), and imaging evidence of limbic encephalitis (LE; p = .03). Death occurred in 48% throughout the course of follow-up, with a higher mortality in patients with LE than in those without LE (p = .04). Of 31 surviving patients at last follow-up, 55% continued to have intermittent seizures. SIGNIF …

Autoimmune encephalitis in the elderly: who to test and what to test for.

The awareness and understanding of autoimmune encephalitis are blossoming in neurology, and patients are being diagnosed and successfully treated with immunotherapy. The diverse symptomatology associated with autoimmune encephalitis means that patients may present initially to mental health services, which are, as yet, less well equipped to identify and investigate such phenomena. Older adult mental health services are used to managing complexity, but the range of pathologies presenting with unusual symptoms that may mimic autoimmune encephalitis is wide and there is no clear guidance as to wh

Two Cases of Pediatric Leucine-Rich Glioma-Inactivated Protein-1 Encephalitis: Clinical Course, Challenges, and Implications.

BACKGROUND: Leucine-rich glioma-inactivated protein 1 (LGI-1) encephalitis is a rare form of autoimmune limbic encephalitis. Although relatively well documented in adults, pediatric cases are rare and remain poorly understood. METHODS: We reviewed two pediatric cases of LGI-1 encephalitis from a single tertiary care facility retrospectively. The detailed analysis included assessment of the initial presentation, clinical progression, diagnostic challenges, treatments, and outcome. To contextualize the differences between pediatric and adult manifestations of disease, we compared these findings…

Anti-LGI1 Энцефалит и совместное существование МОГ-ИГГ: доклад о конкретных случаях и обзор литературы.

Противоположность богатому люцином glioma-inactivated-1 (LGI1) энцефалит — аутоиммунное расстройство, характеризующееся антителами, направленными против LGI1.LGI1-IgG). Она, как правило, имеет когнитивные расстройства, психические расстройства и фасиобрахиальные припадки (FBDS). Myelin oligodendrocyte glycoprotein антитела (MOGAD) в настоящее время признается демиелинизирующим заболеванием центральной нервной системы (CNS) при посредничестве антител против mielin oligodendrocyte glycoprotein (MOG-IgG). Совмещение anti-LGI1 Энцефалит и MOG-IgG - это редкое явление. anti-LGI1 Энцефалит антител в

Совпадение в аннотации оригинала
BackgroundAnti-leucine-rich glioma-inactivated-1 (LGI1) encephalitis is an autoimmune disorder characterized by antibodies that target LGI1 … arch was conducted using the PubMed and Embase databases. We utilized the following search terms: ("Limbic Encephalitis"[MeSH Terms] OR ("autoimmune encephalitis"[Title/Abstract] OR "AE"[Title/Abstract])) AND ("Myelin-Oligodendrocyte Glycoprotein"[MeSH Terms] OR "demyelinating autoimmune diseases, cns"[MeSH Terms] OR ("MOG-IgG"[Title/Abstract] OR "MOGAD"[Title/Abstra …

Cognitive outcomes in anti-LGI-1 encephalitis.

OBJECTIVE: Cognitive impairment is one of the most common symptoms of anti-leucine rich glioma inactivated 1 (anti-LGI-1) encephalitis, but little is known about the cognitive profile of these patients. This study characterized the cognitive profile of patients with anti-LGI-1 encephalitis and compared patterns of impairment to healthy controls and other patient groups with known temporal lobe/limbic involvement. METHODS: A retrospective analysis of adult patients with anti-LGI-1 encephalitis who underwent neuropsychological assessment was conducted. Performance patterns of anti-LGI-1 patient

Seizure semiology and predictors of outcomes in Chinese patients with glutamic acid decarboxylase antibody-associated neurological syndrome.

BACKGROUND: In the current study, seizure semiology and potential predictive factors of seizure outcomes in glutamic acid decarboxylase anti … ted with epilepsy alone. Concomitant neurological syndromes were observed in 22 patients, including limbic encephalitis (n = 20), stiff-person syndrome (SPS, n = 1), and cerebellar ataxia (n = 1). Bilateral tonic-clonic seizures were observed in 21 patients (65.6%). Focal seizures occurred in 27 patients (84.4%); 17 had focal motor seizures and 18 focal non-motor sei …

Evaluation of warning strategies for paraneoplastic neurological syndromes associated with PD-1/PD-L1 inhibitors.

BACKGROUND: The suppressive effects of immune checkpoint inhibitors (ICIs) on anti-tumor immunity have been well documented. However, ICIs c … group, and 61 in the antibody-negative group. The risk-antibody group showed a higher incidence of limbic encephalitis, subacute cerebellar degeneration, and subacute sensory neuronopathy. The prognosis was worse in the risk-antibody group, with a mortality rate of 29%, significantly higher than 17% in the unknown-risk group and 10% in the antibody-negative group (P …

MRI findings in autoimmune encephalitis.

Autoimmune encephalitis encompasses a spectrum of conditions characterized by distinct clinical features and magnetic resonance imaging (MRI … ted protein-like 2 (CASPR2)- or glutamic acid decarboxylase (GAD)- antibodies typically presents as limbic encephalitis (LE) with unilateral or bilateral T2/fluid attenuated inversion recovery (FLAIR) hyperintensities in the medial temporal lobe that can progress to hippocampal atrophy. Gamma aminobutyric acid-B (GABA-B) receptor encephalitis also often shows such me …

Stiff Person Syndrome and Encephalitis with GAD Antibodies with Severe Anterograde Amnesia in an Adolescent: A Case Study and Literature Review.

Antiglutamic acid decarboxylase (GAD65) encephalitis is rare and few pediatric cases have been reported, with variable clinical presentation … AD65 antibodies are associated with various neurological syndromes, and this presentation combining limbic encephalitis and stiff person syndrome is the first pediatric form published to date; there are also few cases described in adults.

[LGI1 ENCEPHALITIS: THE FIRST HUNGARIAN PATIENT].

In the recent years, it has been increasingly recognised that in a group of limbic encephalitis antibodies are directed against the scaffolding protein LGI1 (Leucine-rich glioma inactivated 1), which is part of the voltage gated potassium channel (VGKC) complex on neural synapses. Patients present with seizures and subacute history of neuropsychiatric symptoms, including psychosis and changes in memory, cognition, behaviour. Faciobrachial dystonic seizures can be observed, which are highly characteristic for LGI1 encephalitis. MRI shows medial temporal abnormalities in more than half of the ca…

Drug-resistant glutamic acid decarboxylase 65-associated epilepsy: Pre-surgical evaluation and comparison to temporal lobe epilepsy of other etiology.

High-titer glutamic acid decarboxylase 65 (GAD65) antibodies (>20 nmol/L) are linked to chronic focal epilepsy, usually of temporal regional … ge 4-44). All had temporal regional-onset epilepsy; 53% had bitemporal seizures. Three patients had limbic encephalitis, and three developed mesial temporal sclerosis. Those with GAD65 antibody-associated epilepsy were more likely to have depression and/or anxiety and less likely to undergo resection after evaluation than those in the control group. Although seizure …

Status epilepticus associated with pregnancy: A cohort study.

BACKGROUND: Status epilepticus (SE) is a neurological emergency associated with a high mortality rate and long-term cognitive sequelae. Stat … history of epilepsy-related SE due to withdrawal of antiepileptic medication. In terms of etiology, limbic encephalitis and cerebral venous sinus thrombosis were the two main etiologies of de novo SE associated with pregnancy. The overall mortality rate was 28.5% at discharge, and poor outcomes were especially noted in the patients with limbic encephalitis compared t …

Autoimmune limbic encephalopathy and anti-Hu antibodies in children without cancer.

OBJECTIVE: The aim of this study was to describe the clinical presentation of children and adolescents with anti-Hu antibodies (Hu-Abs). ME … d neuroblastoma and opsoclonus-myoclonus. The other 6 children (5 female and 1 male) presented with limbic encephalitis (progressive personality changes, memory loss, and seizure) and were free of cancer (mean follow-up time: 50 months; range: 34-72 months). Brain MRI scans were abnormal in 4 of the 6 patients, with left, right, or bitemporal T2/fluid-attenuated inve …

[Antibodies to the glutamate receptor].

Glutamate receptors (GluRs) are classified into metabotropic GluRs and ionotropic GluRs. Ionotropic GluRs include the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) -type, kainate (KA)-type, and N-methyl-D-aspartate (NMDA)-type GluRs (NRs). Antibodies to the NRs have been detected using immunoblot, cell-based assays, and enzyme linked immunosorbent assay (ELISA). In patient with non-paraneoplastic, non-herpetic acute limbic encephalitis (NHALE), antibodies against GluN2B (GluRε2) and GluN1 (Gluζ1) are detected in the sera and CSF. In addition to the antibodies to NRs detected …

Understanding and managing acute encephalitis.

Encephalitis is an important cause of morbidity, mortality, and permanent neurologic sequelae  globally. Causes are diverse and include vira … and death. Autoimmune encephalitis (AIE)  includes two major categories: (i) classic paraneoplastic limbic encephalitis (LE) with autoantibodies against intracellular neuronal antigens (Eg: Hu and Ma2) and (ii) new-type AIE with autoantibodies to neuronal surface or synaptic antigens (Eg: anti-N-methyl-D-aspartate receptor). AIE has prominent psychiatric manifestatio …

Infectious and autoantibody-associated encephalitis: clinical features and long-term outcome.

BACKGROUND AND OBJECTIVES: Pediatric encephalitis has a wide range of etiologies, clinical presentations, and outcomes. This study seeks to … rs, psychiatric symptoms, agitation, speech dysfunction, cerebrospinal fluid oligoclonal bands, MRI limbic encephalitis, and clinical relapse were more common in patients with autoantibodies. An abnormal outcome occurred in 49% of patients after a median follow-up of 5.8 years. Herpes simplex virus and unknown forms had the worst outcomes. According to our multivaria …