Nationwide Survey of Acute Juvenile Female Non-Herpetic Encephalitis in Japan: Relationship to Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Abstract

Objective To study the incidence and clinical features of acute juvenile female non-herpetic encephalitis (AJFNHE)

Anaesthesia for a patient with paraneoplastic limbic encephalitis with ovarian teratoma: relationship to anti‐N‐methyl‐d‐aspartate receptor antibodies

“…In Japan, young women with acute non-herpetic limbic encephalitis have been studied as acute juvenile female non-herpetic encephalitis (AJFNHE). Although antibodies against GluRe2 have been frequently found in the sera and CSF of patients with AJFNHE [2], they have been also detected in patients with Rasmussen's encephalitis and epilepsia partialis continua [7]. Of these diagnoses, AJFNHE fits well with the presentation of our patient.…”

“…A unique limbic encephalitis that predominantly affects young women and results in a characteristic syndrome that presents with prominent psychiatric symptoms, central hypoventilation, seizures, involuntary movements and autonomic dysfunction has been noted [1,2]. Recently, a causal relationship between such encephalitis and ovarian teratoma has been proposed [3][4][5], and in patients with this disorder, antibodies binding to NR1 ⁄ NR2 heteromers of the N-methyl-D-aspartate receptor (NMDAR) have been identified [1].…”

Identification of the <i>N</i>-Methyl-D-Aspartate Receptor (NMDAR)-Related Epitope, NR2B, in the Normal Human Ovary: Implication for the Pathogenesis of Anti-NMDAR Encephalitis

“…Histopathological studies have shown that ovarian teratomas removed from patients with this encephalitis certainly contained many neurons having immunoreactivities for anti-NMDAR antibodies, mainly for an anti-NR2B antibody Seki et al 2008;Tüzün E et al 2009). However, recent studies on the basis of large numbers of patients with this disease have disclosed that ovarian teratomas were detectable in only about half of the affected females Kamei et al 2009;Irani et al 2010;Titulaer et al 2013), and added a significant number of male patients without tumors (Novillo-Lopez et al 2008;Wong-Kisiel et al 2010;Tojo et al 2011;Titulaer et al 2013). Another feature of this disease is that the vast majority of the patients are of reproductive age (Dalmau et al 2011;Titulaer et al 2013).…”

“…The disease was, therefore, considered to be a type of paraneoplastic encephalitis (Vitaliani et al 2005;Dalmau et al 2007), but in recent information about the rapidly increased number of patients with this encephalitis (Prüss et al 2010;Dalmau et al 2011), it is known that babies and children are also affected by anti-NMDAR encephalitis (Florance et al 2009;Niehusmann et al 2009;Titulaer et al 2013). However, about half of diseased females were not associated with ovarian teratoma Kamei et al 2009;Irani et al 2010). Additionally, a multi-institutional cohort observation study has revealed that 11% of 577 patients enrolled were male and that the vast majority of these male patients were found to have no underlying neoplasm (Eker et al 2008;NovilloLopez et al 2008;Wong-Kisiel et al 2010;Tojo et al 2011;Titulaer et al 2013).…”

Clinical Aspects of Anti-NMDA Receptor Encephalitis

“…Analysis of a worldwide one hundred anti-NMDA receptor encephalitis case series revealed that about 60% of them had associated tumors such as ovarian teratomas . Meanwhile, Kamei et al proposed 'acute juvenile female non-herpetic encephalitis (AJFNHE)' (Kamei et al, 2009). The clinical symptoms and course of AJFNHE are similar to those of anti-NMDA receptor encephalitis.…”