Page 92 - Read Online

periodic screening for at least 2 years is recommended, PREGNANCY AND DISEASE
[11]
even for patients who are in recovery. Younger age (<
12 years), older age (> 40 years), and male gender are There is concern that the anti‑NMDAR antibody
associated with a reduced risk of tumor. [14] (subtypes IgG1 and IgG3), which can cross the placenta
beginning at 14th week of gestation and up to the time
TREATMENT, CLINICAL RESPONSE AND of delivery, may affect the developing brain of the
[35]
RECOVERY fetus. Factors that may determine outcomes are the
gestational age of disease onset, antibody titer of the
A large observational cohort study showed that maternal serum, timing of treatment to deplete maternal
immunotherapy (high‑dose steroid plus either pathologic antibody, and the fetal BBB. A few case
plasmapheresis or intravenous immunoglobulin) reports of anti‑NMDAR encephalitis during pregnancy
and tumor removal, if applicable, yielded beneficial showed a positive outcome. [36,37] However, one case
[14]
neurological outcomes. Patients in whom a tumor report showed evidence for the transplacental transfer
was detected and removed within 4 months of disease of NMDAR antibody. The antibody titer in this infant
[38]
onset experienced a more complete and rapid recovery was the same as in the mother at 2 days after delivery
[13]
than those without tumor. In patients who did not and declined until a negative titer was determined at
respond to first‑line treatment, second‑line therapy the age of 1 year. The infant showed a delay in global
(cyclophosphamide or rituximab) resulted in improved
outcomes and fewer relapses. Two independent development and had cortical dysplasia. It remains
predictors of good outcomes were a lower severity of the unknown whether these abnormalities resulted from
disease and an earlier time to appropriate treatment. the effect of the transplacental anti‑NMDAR antibody
These determining factors are maintained irrespective or were an indirect effect of the maternal illness.
of the age of disease onset. [14,33] The recovery process
is usually the reverse of the clinical presentation. [11] OVERLAPPING ANTI‑NMDAR ENCEPHALITIS
Autonomic and abnormal movement are usually AND DEMYELINATING DISEASE
recovered before other symptoms. Psychiatric features Many case reports have described the co‑existence
[13]
may persist up to several months. The anti‑NMDAR of anti‑NMDAR antibody and demyelinating disease
titer in the CSF usually decreases in parallel to the (AQP4‑IgG, MOG‑IgG). [39] The encephalopathy may
clinical response, but the presence of antibody may precede, follow, or occu simultaneously with a clinical
persist for a long period (up to 15 years) despite normal episode of demyelinating disease. Most patients
[34]
clinical features. Clinical history and neurological respond to immunotherapy but tend to require more
examination, including a neuropsychiatric test, is the
[31]
most useful indicator of the treatment response, and intensive treatment and display more residual deficits.
thus, the periodic detection of the anti‑NMDAR titer is These findings should prompt the awareness of
not necessary in the context of stable clinical status. physicians that patients with the demyelinating disease
who develop atypical symptoms such as abnormal
ANTI‑NMDAR ENCEPHALITIS IN OLDER AGE movements or vice versa should be investigated for
other conditions. [39]
The onset of anti‑NMDAR encephalitis at an age
of > 45 years has some characteristic features that CONCLUSION
differ from those of young patients. There is a greater
frequency of males compared to females (1:1.2), a Anti‑NMDAR encephalitis is more common than
reduced frequency of tumor association, fewer seizure expected and may be the most common cause of
episodes, and a greater tendency to present with antibody‑mediated encephalopathy. This disease
[33]
memory deficit in the late‑onset group. If tumors are should be suspected in children or young adults with
found, then carcinoma is more likely than teratoma. acute behavioral problems, seizure, and abnormal
However, within 4 weeks of onset, the patients develop movements. Late‑onset disease (patients > 45 years
symptoms that are typical of the disease in young old) may present with memory problems. Investigations
adults. The delay in diagnosis and treatment is longer of anti‑NMDAR antibody or other autoantibodies that
in the late‑onset group. This observation may be due to are present in the CSF and serum are recommended.
the wide differential diagnosis in clinical presentation. Patient outcomes depend on the severity of the disease
However, other prognostic factors, including an at the time of onset, early immunotherapy, and
earlier time to treatment, the use of second‑line adequate second‑line drugs if the response to first‑line
immunotherapy in the case of first‑line drug failure, therapy fails. Long‑term surveillance of ovarian
and younger age are associated with improved teratoma in young female patients is prudent if the
outcomes. initial workup is negative.

Neuroimmunol Neuroinflammation | Volume 3 | March 28, 2016 83