Plasma exchange vs IVIG —> PE
Although no systematic comparisons of the three first-line
immunotherapy options have been performed, early initiation
of PE prior to IVIG may provide better outcomes. A long-term
follow-up conducted by Pham et al. showed that patients
treated with IVIG after PE had better outcomes than those
who received IVIG prior to PE (Pham et al., 2011). In
addition, PE can remove the autoantibodies quickly, resulting
in rapid symptomatic improvement in severe cases of anti-
NMDAR encephalitis. Because patients with severe anti-
NMDAR encephalitis may suffer from autonomic instability, it
is important to evaluate whether they can tolerate PE
procedures, and their vital signs must be closely monitored
during therapy. Recently, a study divided patients with severe
refractory anti-NMDAR encephalitis into a PE group and a non-
PE group. The PE group showed greater improvement in
clinical symptoms than the non-PE group after 1 month and
2 months following treatment (Zhang et al., 2019), suggesting
that early use of PE may be a better treatment option.
Agitasi
Agitation, atypical sedating antipsychotics (such as
olanzapine) are preferable because they induce fewer side
effects, such as NMS and extrapyramidal symptoms (EPS),
which can worsen agitation. Moreover, olanzapine has been
shown to prevent toxicity associated with NMDAR antagonists
in animal experiments. Clonidine may help reduce agitation
through regulation of the autonomic nervous system.
Gejala Lain
Typical and atypical antipsychotics have been administered
alone or in combination to treat hallucination and delusion
(Kuppuswamy et al., 2014). Lithium and valproic acid areprimarily used to control manic symptoms. Owing to its anti-
epileptic and mood stabilizing properties, valproic acid can be
used to effectively treat mood symptoms. Furthermore,
valproic acid can be used to treat choreiform movements and
dyskinesia, which are commonly observed in anti-NMDAR
encephalitis
Implikasi Klinis
Anti-NMDA receptor encephalitis is a relatively newly identified
and potentially treatable cause of psychiatric symptoms. To date,
the recommendation for screening has advised testing of those
patients, particularly females, with an atypical new-onset
presentation of psychosis with motor features. It is clear that early
identification and treatment may have serious prognostic
implications. Delay to treatment with immunosuppressive therapy
probably results in worsened outcomes, with evidence for
permanent hippocampal damage. Anti-NMDA receptor
encephalitis is a potentially treatable form of psychiatric illness
that is illuminating our understanding of the neuropathophysiology
involved in some individuals who present with symptoms of
psychosis.
Differential Diagnosis
Clinical symptoms of anti NMDAR encephalitis may mimic
schizophrenia and psychotic spectrum disorders. New onset
psychosis typically occurs in late teens to 30s. Females generally
present at a later age than males. There is often a family history of
mental illness, but this is not always the case.
Many patients with schizophrenia do not have insight into their
illness. For example, they are less likely to view their hallucination
as such and instead are likely to incorporate them into delusional
thoughts. In our experience, our patient had insight into her
cognitive decline. At times, she seemed to be pleading for helpwhile acknowledging her thoughts were disordered by nodding.
Abnormalities in CSF and autoantibodies can occur in individuals
with schizophrenia as well as those with antiNMDAR encephalitis.
Elevated inflammatory cytokines, immunoglobulins, and elevated
cell counts within the CSF have been noted in 40-70% of
individuals with schizophrenia and affective psychosis.
The differential diagnosis also includes other viral encephalitides
(cytomegalovirus [CMV], Epstein-Barr, herpes simplex virus [HSV],
varicella zoster virus [VZV], human immunodeficiency virus [HIV],
human herpesvirus 6 [HHV6]/ human herpesvirus 7 [HHV7],
arbovirus, rabies virus), other autoimmune causes (limbic
encephalitis, other paraneoplastic encephalitides, systemic lupus
erythematosus, antiphospholipid syndromes, Sjégren’s syndrome,
Graves’ disease, Hashimoto's encephalitis, vasculitis), and toxic/
metabolic disorders (drug ingestion, porphyria, mitochondrial
disorders). When antipsychotics have been used for initial
presenting symptoms, neuroleptic malignant syndrome enters the
differential. It may be difficult to differentiate this from the
autonomic symptoms seen in anti-NMDAR encephalitis. In our
experience, we found patients with anti-NMDAR encephalitis to be
less responsive to typical antipsychotic treatments.
NEOS
The score was strongly associated with the probability of poor
functional status at 1 year (3% for 0 or 1 point to 69% for 4 or 5
points).36 This may be of use to the provider when discussing
prognosis with family members, whereas, in the past, these
discussions may have been more nebulous. Of note, the authors
found that of patients in their cohort with poor functional status at
1 year, 35% of them recovered to good functional status at 2 years.