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Table 1: Contd...
Patient Sex Age Teratoma Surgery Glucocorticoids IVIg Serum Rituximab Anti‑ Prognosis Author
exchange virus
34 Female 47 Yes No Yes No - - - Favorable and Our case
no recurrence
35 Female 17 Yes Yes - Yes - - - Favorable and Our case
no recurrence
IVIg: Intravenous immunoglobulin; N/A: Not applicable; “-”: Not mentioned by author

our investigation, the mean price of an antibody test
is about 60 dollars for the reported patients in China.

Treatment
Regarding to the treatment for anti-NMDAR encephalitis,
Dalmau et al. [18] provided a treatment proposal for this
disease in 2011 [Figure 1]. They prefer concurrent
intravenous immunoglobulin (IVIg) (0.4 g/kg/day for
5 days) and methylprednisolone (1 g/day for 5 days) to
plasma exchange. As for the second-line therapy, they
often use rituximab combined with cyclophosphamide
in adults. And in children, they often use only one of
these drugs – mostly rituximab. In China, anti-NMDAR
encephalitis as a new disease, is often confused with
viral encephalitis, and is treated with acyclovir or/and Figure 1: Proposed algorithm for the treatment of anti-N-methyl-D-aspartate receptors
virazole. When the diagnosis was uncertain, some encephalitis
doctors gave IVIg as an alternative to the patients who
did not respond to anti-viral treatment. Rituximab was OTHER ISSUES RELATED TO ANTI-NMDA
seldom used for anti-NMDAR encephalitis patients due RECEPTOR ENCEPHALITIS
to its high cost, and lack of doctor’s experience with the
drug [Table 1]. Pregnancy
Majority of the patients are females, and the issue of
Prognosis pregnancy is unavoidable. Pregnant patients could
Gresa-Arribas et al. [41] conducted a 5-year study deliver a healthy baby if they have no NMDAR antibody
with 501 patients. Their findings include: (1) 81% in their serum. The curative effect increases significantly
of anti-NMDAR encephalitis patients had favorable after giving birth or after termination of pregnancy.
outcomes from immunotherapy, and factors affecting
these outcomes include early diagnosis and nonintensive Synaptic autoimmune encephalopathy
care unit treatment; (2) risk of recurrence is about 12% Some types of autoimmune encephalitis, such as
within 2 years, of which 67% is less harmful as compared anti-NMDAR encephalitis, anti-AMPAR encephalitis,
with the first outbreak; (3) normally, the second-line anti-GABABR encephalitis, and anti-LGI1 encephalitis,
immunotherapy was effective when the first-line therapy can be distinguished by the antibodies against the
had failed. [41] Based on the long-term follow-up, the receptors anchored in synapses. There are some
higher titer of antibody in patients’ serum or CSF, worse common features of these diseases: high incidence
was the prognosis. There was significant association in females always associated with tumor, psychiatric
between CSF antibody titer and the risk of recurrence. [42] disorders, behavioral changes, and refractory seizures.
Importantly, these diseases are reversible and curable
Among the 35 patients with anti-NMDAR encephalitis, with immunotherapy and removal of possible tumors,
one patient was in a continuous stupor-like state, one if they are diagnosed at an early stage.
patient died 4 days after the tumor removal, one patient
had an unfavorable prognosis, one patient recurred The term of synaptic autoimmune encephalopathy
but improved after IVIg again, and 30 patients (86%) is recommended for labeling these disease, thereby
had favorable prognosis without recurrence or hinting at their favorable prognosis and the necessity
sequel [Table 1]. for early immunotherapy.

Neuroimmunol Neuroinflammation | Volume 1 | Issue 1 | June 2014 21