Anti NMDAR encephalitis In 1997 a syndrome of Ovarian teratoma-associated limbic encephalitis (OTLE) was reported in 1 Japanese girl and 1 woman, both of whom improved following tumor resection.In 2004 Vitaliani et al described a treatment-responsive paraneoplastic encephalitis in relatively young women associated with ovarian teratoma and antibodies against the cell membrane of hippocampal neurons .
The target autoantigens were identified as heteromers containing NR1 and NR2 subunits of the N-methyl-D-aspartate receptor (NMDAR). In 2005,Dalmau et al first demonstrated antibodies to novel neuronal cell membrane antigens in 4 women with Ovarian teratoma-associated limbic encephalitis (OTLE) in a non-permeabilized culture of hippocampal neurons. Subsequently the same group reported a severe but treatment-responsive encephalitis associated with antibodies to NR2B and NR2A containing heteromers of the NMDAR and referred to the condition as Paraneoplastic Anti–N-methyl-D-aspartate Receptor Encephalitis.
Anti-NMDAR encephalitis are associated with tumours (commonly teratomas) in about 60% cases. Recent studies have however shown that this disorder can occur even in the absence of teratomas and is increasingly recognized in adolescents and children. Read More...