Type Of Autoimmune Encephalitis Research Articles

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis.

The aim of this study was to assess the seizure manifestations and risk of epilepsy in encephalitis associated with antibodies against neuronal cell-surface (autoimmune encephalitis) or myelin-associated antigens, and to review several chronic epileptic disorders, including Rasmussen's encephalitis, fever-induced refractory epileptic syndromes (FIRES) and new-onset refractory status epilepticus (NORSE). Seizures are a frequent manifestation of autoimmune encephalitis. Some autoimmune encephalitis may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), electroencephalogram extreme delta brush (anti-NMDAR) or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic and basal ganglia dysfunction results in different types of seizures. Autoimmune encephalitis or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). Rasmussen's encephalitis or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Although seizures are frequent in all types of autoimmune encephalitis, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (Rasmussen's encephalitis, FIRES, NORSE), the prognosis remains poor.

Serial 18F-FDG PET/CT Findings in a Patient With IgLON5 Encephalopathy

We presented the serial FDG PET/CT brain scans of a 64-year-old woman with IgLON5 encephalopathy, which is a novel syndrome in association with antibodies to a neuronal cell adhesion protein named IgLON5, and FDG PET findings have not been characterized previously. For our case, the relatively hypermetabolism in primary sensorimotor cortices, basal ganglia, and cerebrum comparing to other cortical regions on the pretreatment FDG PET/CT was partially recovered on the follow-up FDG PET/CT scan after immunotherapy, corresponding with the alleviation of clinical syndromes. The metabolic change pattern was not similar as other types of autoimmune encephalitis.

Clinical, imaging, and follow-up observations of patients with anti-GABAB receptor encephalitis

Purpose: Anti-gamma-aminobutyric acid B (anti-GABAB) receptor encephalitis is a newly described type of autoimmune encephalitis. We report a case series of patients diagnosed with anti-GABAB receptor encephalitis in China, focusing on their presentations, laboratory and imaging results, and outcomes, as well as the treatment strategies which were employed. Methods: Data from patients diagnosed with anti-GABAB receptor encephalitis in the Second Affiliated Hospital, School of Medicine, Zhejiang University, from January 2014 to June 2015 were retrospectively collected and analyzed. Based on specific diagnostic criteria, seven cases were included. Results: Six of the seven patients were males, and a median age at presentation of 56 years (range: 4–71 years). Seizures were the most common initial symptom, and all patients developed symptoms of typical limbic encephalitis during their disease course. Additional types of autoantibodies were identified in four patients. After presentation, three patients were found to have small cell lung cancer and one patient was eventually diagnosed with thymoma. All patients accepted first-line immune therapy, but only one chose tumor treatment. The three tumor-free patients had a good outcome, whereas those with tumors had a poor one. Finally, there were no relapses during follow-up. Conclusion: Anti-GABAB receptor encephalitis is a rare, unique autoimmune disease, and is often associated with tumors. It should be considered in the differential diagnosis for middle and senior-aged patients who present with predominantly limbic encephalitis symptoms. Importantly, earlier recognition of this potentially treatable condition could improve its overall prognosis.

Anti-N-methyl-D-aspartate receptor encephalitis: analysis of three cases

Objective To study clinical features, diagnosis, therapy response and prognosis of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Methods Three cases with anti-NMDAR encephalitis were reported. The clinical features, laboratory examinations, imaging, EEG and therapy response of 3 cases were retrospectively analyzed, and also related literatures were reviewed. Results Two patients were young male and one patient was old female. Main symptoms included psychiatric symptoms in 3 cases (mania in 2 male patients and stupor in the female patient), epilepsy in 2 cases and respiratory failure in one case. The results of MRI examination revealed normal, while EEG examination showed abnormal in all cases. No tumor was detected in any of these patients. Lumbar puncture revealed normal cerebrospinal fluid (CSF) pressure (3 cases), elevated white blood cell (WBC, 3 cases) and protein quantification (one case). All cases were confirmed to have the disease by detection of anti-NMDAR antibodies in serum and CSF. One male patient got better after receiving immunotherapy with methylprednisolone and intravenous immunoglobulin (IVIg), but psychiatric symptoms were left over. Another male patient had no response to the above treatment. But the female patient was improved without immunotherapy. All 3 cases were followed up for one year after being discharged. One male patient died by accident because of mental disorders. Another male patient showed no sign of relief. The female patient got mild personality and memory change. Conclusions Anti-NMDAR encephalitis is a new type of autoimmune encephalitis. It is characterized by fever, memory deficits, seizures, disturbance of consciousness, and autonomic dysfunction in males and females of all ages. This type of encephalitis is often associated with teratoma, and has a good response to immunotherapy. There is a certain correlation between progression and prognosis. DOI: 10.3969/j.issn.1672-6731.2015.07.013

Defining and treating leucine-rich glioma inactivated 1 antibody associated autoimmunity

The identification of subtypes of autoimmune encephalitis, associated with autoantibodies to cell surface components of CNS ion channels and related proteins, has had great impact on the practice of clinical neurology in the last decade. The new classifications based on mechanism-related autoantibodies herald a new era which bridges neurology, psychiatry, immunology and synaptic biology. Leucine-rich glioma-inactivated (LGI) proteins 1–4 have roles in synaptic transmission and myelination (Kegel et al. , 2013), and LGI1 is one of the novel autoantigens in autoimmune encephalitis. The LGI1 gene on chromosome 10 was identified in high grade gliomas in 1998 and found to be mutated in autosomal dominant partial epilepsy with auditory features (ADPEAF) in 2002. LGI1 protein is notable for being secreted, and contains a leucine-rich (LRR) domain and an epilepsy-associated or epitempin (EPTP) domain. LGI1 may regulate synaptic strength at excitatory synapses through interaction with postsynaptic ADAM22, PSD95 and presynaptic ADAM23; and LGI +/− and LGI −/− mice are proving to be useful animal models of focal epilepsy (Chabrol et al. , 2010). One type of autoimmune encephalitis, notable for variable combinations of seizures, amnesia, psychosis, hyponatraemia, low prevalence of remote tumour and high response rate to immunotherapy was identified in 2001 by Angela Vincent and colleagues. The cases had serum autoantibodies directed to voltage gated potassium channel complexes (VGKC) measured by radioimmunoprecipitation assay of whole rabbit-brain homogenates using 125I-labelled dendrotoxin (Vincent et al. , 2004). These are the same circulating antibodies, though at higher levels, as those studied by the late John Newsom-Davis and the late Ian Hart in peripheral nerve hyperexcitability and Morvan’s syndrome (Hart et al. , 2002). VGKC-complex antibodies are probably pathogenic. They tick a few of the boxes to satisfy Witebsky’s postulates of autoimmune disease: the antibodies are highly specific, though …

Anti-NMDAR-encephalitis in children

Anti-N-methyl-D-aspartate receptor encephalitis (Anti-NMDAR-encephalitis) in children is a type of autoimmune encephalitis associated with NMDA receptor.Its etiology and clinical manifestations are specific in children.This disease is associated with teratoma more in adult than in children.However,its etiology is more associated with infection and heredity in children.The pathogenesis is that the anti-NMDAR-antibody can combine with NMDAR of neurons selectively and reversibly.The clinical manifestations include psychiatric syndrome,language,motor,sleep and autonomic nervous disorders.Tumor resection and immtmotherapy are chief treatments.This disorder is often misdiagnosed as it is rarely reported in children.It is valuable to make early diagnosis and treatment.This paper reviews the mechanism,clinical features,diagnosis and treatment of antiNMDAR-encephalitis of children. Key words: Anti-NMDAR-encephalitis; Children; Clinical manifestation; Treatment

Voltage gated potassium channel antibodies positive autoimmune encephalopathy in a child: A case report and literature review of an under-recognized condition

Autoimmune limbic encephalitis (LE) associated with voltage gated potassium channel antibodies (VGKC-Abs) in children is more common than previously thought and is not always paraneoplastic. Non-neoplastic, autoimmune LE associated with VGKC-Abs has been described recently. However, only few case reports in children as the disease is predominantly described in the adult population. It is likely that this type of autoimmune encephalitis is currently under-diagnosed and hence, under-treated, especially in children. We present a 13-year-old previously fit and healthy African girl diagnosed with LE and we reviewed the literature for its current management.

Guest editorial.

Since the identification of anti-N-methyl-D-aspartate (NMDA) receptor antibodies about 15 years ago, many patients with rapidly progressing psychiatric symptoms, abnormal movements, seizures or unexplained coma, have been diagnosed with autoimmune encephalitis (AE). The symptom onset is often unspecific and might mimic psychiatric disease, but the later course is frequently characterized by severe disease, often requiring intensive care. Clinical and immunological criteria are helpful in identifying the patients, but no biomarkers exist to guide the clinician in therapy or predict outcome. While persons of all ages can be affected by AE, some types of AE affect more children and young adults and are more prevalent in women. This review will focus on encephalitides associated with neuronal cell-surface or synaptic antibodies, which can result in characteristic syndromes, and are often recognizable on clinical grounds. AE subtypes associated with antibodies against extracellular epitopes can occur with or without tumours. Because the antibodies bind and alter the function of the antigen, the effects are often reversible if immunotherapy is initiated, and the prognosis is favourable in most instances. The first part of this series will introduce the topic, provide an overview of current neuronal surface antibodies and how they present, describe the most common subtype, anti-NMDA receptor encephalitis, and discuss the difficulties in recognizing patients with underlying AE amongst patients with new onset psychiatric disorders.