Table 2.

Studies on third-line therapy for refractory autoimmune encephalitis.

Name	Study Population	Result	Type	References
Bortezomib	A 22-year-old woman with refractory anti-NMDAR encephalitis	Clinical symptoms improved; autoantibody titers decreased from 1:1000 to 1:320 in serum	Case report	Schroeder Christoph et al. in (25).
	An 8-year-old girl with refractory anti-NMDAR encephalitis	Clinical symptoms improved; autoantibody titers decreased from 1:20 to 1:2 in CSF	Case report	Cordani R et al. in (26)
	5 cases of severe refractory anti-NMDA encephalitis	Three patients improved to a minimally conscious state; Two patients’ autoantibody titers decreased in CSF	Case series	Yong-Won Shin et al. in (27).
	A 26-year-old woman with refractory anti-NMDAR encephalitis	Clinical symptoms improved	Case report	Olafur Sveinsson et al. in (28).
	Two cases of severe refractory anti-NMDA encephalitis	Significant clinical improvement	Case series	S. Keddie et al. in (29).
Tocilizumab	A 64-year-old man with refractory Anti-CASPR2 Antibodies	Clinical and neuroradiological improved; autoantibody titers decreased from 1:1000 to 1:100 in serum	Case report	Maurizio benucci et al. in (30).
	Three children with refractory autoimmune encephalitis	Clinical symptoms improved	Case series	Randell R. et al. in (31)
	An 8-year-old girl with refractory anti-GAD encephalitis	Clinical symptoms improved; autoantibody levels declined from 303.1 U/ml to 30 U/ml in serum	Case report	Jaafar F. et al. in (32).
	30 patients with refractory autoimmune encephalitis	Higher frequencies of mRS improvement and favorable clinical responses compared with the control groups	Cohort study	Lee et al. in (33).
	52 patients with anti-NMDA encephalitis	Better efficacy than the control groups	Cohort study	Lee et al. in (34).
Daratumumab	A 60-year-old man with refractory anti-CASPR2 encephalitis	Clinical symptoms improved; autoantibody titers decreased from > 1:1.000 to 1:32 in CSF and > 1:10.000 to 1:10.000 in serum	Case report	Scheibe F et al. in (35).
	A 26-year-old woman with refractory anti-NMDAR encephalitis	Clinical symptoms improved	Case report	Ratuszny D et al. in (36).
Tofacitinib	8 patients with refractory autoimmune encephalitis	Two had good responses; three had partial responses; three showed no significant improvements	Cohort study	Lee et al. in (34).
Low-dose Interleukin 2	10 patients with refractory autoimmune encephalitis	Functional outcome improved in six patients	Cohort study	Lim JA et al. in (37).
Rapamycin	17 patients with anti-Hu encephalitis	Over half of the patients improved or stabilized functional disabilities	Cohort study	de Jongste AH et al. in (38).
	An 8-year-old girl with refractory anti-NMDAR encephalitis	Clinical symptoms improved	Case report	Cordani R et al. in (26)