Immune-mediated pediatric epilepsies.

Apart from Rasmussen encephalitis, other immune-mediated brain disorders have emerged that are frequently associated with recurrent epileptic seizures: (1) limbic encephalitis, (2) anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis, and (3) steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT, formerly called Hashimoto encephalopathy). Limbic encephalitis mainly affects the mediotemporal aspect of the cortex giving rise to temporal lobe seizures and memory and affective disturbances. It can occur as a paraneoplastic or, even more frequently, a nonparaneoplastic condition. Detection of specific autoantibodies may suggest underlying cancer (onconeural antibodies) and may give hints regarding the long-term prognosis. VGKC-complex or GAD antibodies have been identified in children. Anti-NMDAR encephalitis is a disorder mainly of female adolescents and young adults. Patients present with seizures in around one-third of cases, and 80% of patients develop recurrent, mainly extratemporal seizures at some stage of their disorder. The clinical course is that of a mostly monophasic, but sometimes multiphasic, severe cortico-subcortical encephalopathy. Ovarian teratomas underlie the disorder in 30-60% of adult cases but are rare in young children. Tumor removal and early intense immunotherapy are predictors for a favorable outcome. SREAT is not a disorder with definite pathogenesis but rather a "catch all" for encephalopathic immune-mediated brain diseases (often with seizures) which have in common thyroid antibodies and a good response to steroid therapy.

Bien CG ，Vincent A 《-》

[Autoimmune encephalitis as a cause of psychosis].

Suokas K ，Kampman O 《-》

Autoimmune encephalitis: recent updates and emerging challenges.

The knowledge of immune dysregulation and autoimmunity in neurological disorders has expanded considerably in recent times. Recognition of clinical syndromes, reliable methods of diagnosis, and early targeted immunotherapy can lead to a favourable outcome in acute and subacute neurological disorders that may be associated with significant morbidity and mortality if left untreated. This review focuses on the rapidly expanding field of autoimmune encephalitis. We describe the differences between limbic encephalitis associated with antibodies targeting intracellular antigens, and neuronal surface antibody syndromes (NSAS) where the antigens are primarily receptors or synaptic proteins located on the neuronal cell surface. We chronologically highlight important developments in NSAS by focusing on voltage gated potassium channel complex-associated antibody mediated encephalitis, anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, and anti-dopamine 2 receptor antibody-associated basal ganglia encephalitis. Contentious issues such as the complexities of using serum antibodies as biomarkers, the initiation of central nervous system autoimmunity, and possible pathogenic mechanisms of these antibodies will be reviewed. The therapeutic challenges that clinicians face such as the timing of therapy and the role of second-line therapy will be discussed, with crucial concepts highlighted in the form of clinical vignettes. Future directions will involve the identification of novel antigens and methods to establish their pathogenicity, as well as evaluation of the most efficacious therapeutic strategies in patients with established NSAS.

Ramanathan S ，Mohammad SS ，Brilot F ，Dale RC ... -  《-》

Diagnostic value of CSF findings in antibody-associated limbic and anti-NMDAR-encephalitis.

In people with suspected inflammatory CNS disease, cerebrospinal fluid (CSF) is commonly analyzed. Antibody-associated limbic encephalitis (ab-LE) and anti-NMDAR-encephalitis are recognized as two major syndromes of autoimmune epilepsies. Here, we investigated the diagnostic value of CSF findings in these two entities. We reviewed patients from our tertiary epilepsy centre with ab-LE and anti-NMDAR-encephalitis in whom CSF examination including oligoclonal bands (OCB) was performed. Ab-LE patients were subdivided according to antibodies (voltage-gated potassium channels, VGKC; glutamic acid decarboxylase, GAD) or presence of onconeural antibodies/presence of tumour into three groups: VGKC-LE, GAD-LE or paraneoplastic LE (PLE). As controls, patients with CSF investigations in whom autoimmune origin was initially assumed but not confirmed later on were included. In addition, a review of published ab-LE and anti-NMDAR-encephalitis cases with reported CSF data was performed. 55 ab-LE (23 VGKC-LE, 25 GAD-LE, 7 PLE) and 14 anti-NMDAR-encephalitis patients were identified at our centre. OCB were significantly more frequent in ab-LE and anti-NMDAR-encephalitis than in controls. Literature review identified 150 ab-LE and 95 NMDAR cases. Analysis of pooled data confirmed that presence of OCB was significantly more frequent in ab-LE and anti-NMDAR-encephalitis (especially in people with GAD-LE and anti-NMDAR encephalitis) as compared to controls. Sensitivity and specificity of OCB in the pooled ab-LE and anti-NMDAR-encephalitis patients was 34% and 96%, respectively. In patients with ab-LE and anti-NMDAR-encephalitis, the likelihood of OCB in CSF was 8.5-fold higher as compared to controls. Furthermore, in the pooled ab-LE and anti-NMDAR-encephalitis patients, cell counts in CSF were more frequently elevated (especially in those with anti-NMDAR encephalitis) than in controls, whereas protein content of CSF was not different between the groups. OCB, and to a lesser extent cell counts in CSF, appear to be helpful additional CSF markers in the diagnostic evaluation of people presenting with a constellation suggestive for GAD-LE, PLE and anti-NMDAR-encephalitis, prompting subsequent analysis of specific antibodies.

Malter MP ，Elger CE ，Surges R 《-》

[Anti-nMDA receptor encephalitis--clinical manifestations and pathophysiology].

Iizuka T ，Sakai F 《-》