Autoimmune encephalitis—An update

Abstract

ZusammenfassungDer Nachweis von Autoantikörpern gegen Nerven- oder Gliazellen ermöglicht heute bei zahlreichen neurologischen und psychiatrischen Symptomkomplexen die frühe und spezifische Diagnose einer Autoimmunenzephalitis. Damit hat sich auch die Herangehensweise an die immuntherapeutische Behandlung dieser Krankheitsgruppe fundamental verändert, ebenso wie das Verständnis der zugrunde liegenden Pathophysiologie und der auslösenden Faktoren. Die noch immer wachsende Zahl neuer Autoantikörper erfordert ein regelmäßiges Update über den Stand der Antikörperdiagnostik, die Häufigkeit assoziierter Tumoren sowie das antikörperspezifische Spektrum klinischer Symptome, die von Wesensänderungen und kognitiven Störungen über epileptische Anfälle und Bewegungsstörungen bis hin zu vegetativen und Bewusstseinsstörungen führen. Der Beitrag fasst die aktuellen Neuerungen zusammen, die sich im klinischen Spektrum von Enzephalitiden, in der bildgebenden und Liquordiagnostik, in der Prognoseabschätzung, in der Etablierung innovativer Immuntherapien, in der Anwendung diagnostischer Pfade bereits vor dem Eintreffen des Antikörperbefundes und im Verständnis der Krankheitsentstehung ergeben.

Lethal Borna disease virus 1 (BoDV-1) infections of humans and animals – in-depth molecular epidemiology and phylogeography

“…This study raises the number of published laboratory-confirmed human BoDV-1 infections to 46 (Table 1), following the case definition of Eisermann et al 11 , which requires direct virus from the patient. Twenty-eight cases were confirmed by RT-qPCR during this study (including previously published cases without BoDV-1 sequence; 7,9,14,16,17,18,65,66,67 ). BoDV-1 sequences were available from public databases for further 16 previously published human cases (Table 1) 6,7,8,10,11,12,13,15 .…”

“…Our study increases the number of published laboratory-confirmed human BoDV-1-infections to 46 and provides a first comprehensive summary of metadata for all published cases 6,7,8,9,10,11,12,13,14,15,16,17,18 . The cases covered all age groups and encephalitis was diagnosed for 45 of the 46 patients, of -25 -whom 44 had died as a result of the disease, resulting in a known case-fatality rate of 97.8%.…”

“…In addition, brain samples from 29 archived or recent human BoDV-1 encephalitis cases were obtained from diagnostic centres and pathologists in Germany. These cases included unpublished cases as well as previously published cases without available BoDV-1 sequence 7,9,14,16,17,18 (Table 1). In addition, samples from seven BoDV-1-positive bicolored white-toothed shrews were obtained from an ongoing large-scale small mammal screening study (Haring et al, manuscript in preparation; Table 1).…”

“…Borna disease virus 1 (BoDV-1, species Orthobornavirus bornaense, family Bornaviridae) is the causative agent of Borna disease, a severe and often fatal neurologic disease of various domestic mammals, particularly horses, sheep, and New World camelids 1,2,3,4,5 . Recently, the virus received increased attention following confirmation of fatal BoDV-1-induced encephalitis in humans 6,7,8,9,10,11,12,13,14,15,16,17,18 . In domestic mammals and humans, BoDV-1 establishes a persistent and strictly neurotropic infection mainly of the central nervous system, with so far no evidence of shedding of infectious virus.…”

Milestones in the history of neurocritical care

“…Anti-NMDAR antibodies of the immunoglobulin subtypes IgA, IgG, or IgM were detected in 30% of patients during HSE, suggesting secondary autoimmune mechanisms. The presence of these antibodies is associated with classic autoimmune encephalitis in most patients, though notably some patients who developed anti-NMDAR antibodies after HSE did not clinically develop autoimmune encephalitis [ 118 ]. Typically, patients with later develop autoimmune encephalitis have symptoms within the first two months after HSV encephalitis, however, a delayed onset up to a year has been reported [ 118 ].…”