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undergo a complete recovery spontaneously, this is not benzodiazepines seem to be the most effective, while
frequent and it is not possible to identify the patients antipsychotic drugs are less efficacious and often
with a favorable outcome. Taking time before treatment associated with adverse events.
[56]
waiting for immunological results or tumoral screening
in anti-NMDAR cases is not recommended, not only Finally, during the remission and stabilization phases a
because of the severity of the clinical findings but also gammaglobuline total check should be repeated in order
because patients not promptly treated may be at higher to detect rituximab induced hypogammaglobinemia,
risk for relapse. [12,53] that can eventually be treated with a replacement of an
extra dose of exogenous IvIg. [10,57]
To date no consensus has been achieved on the
treatment scheme to be used, and the available Regarding relapse risk prevention, no data are available
protocols are heterogeneous. so far on the preventive value of chronic long term IvIg
administration, but encouraging results come from
The first-line therapy usually includes a short the chance of monitoring the CD19+ and CD27+
course of high-dose steroids (methylprednisolone lymphocytes value every 2 months, re-administering
MP; 30 mg/kg/day i.v. per 3-5 days) followed by or rituximab in case of their further increase. [58]
combined with intravenous immunoglobulin (IvIg)
administration (0.4 g/kg/day per 5 days). DISCUSSION

Steroids are then tapered using 1-2 mg/kg/day orally, The early recognition of an immune mechanism
on average for another 12 weeks, adjusting the dose underlying a neurologic disorder provides a chance to
according to patient tolerability or possible side effects. start early treatment and to achieve a better outcome.
If no benefit is noticed during steroid treatment, plasma
exchange (PE), 3-5 cycles, should be considered. Guidelines for NSAS in children have been recently
developed, extrapolated from a previous study by
[10]
In case the first-line treatment is unsatisfactory, a Zuliani referred to adults, and mainly differing from
[1]
second-line immunotherapy should be started. It it since focused on the higher epilepsy occurrence
usually consists of rituximab 375 mg/mq per week among pediatric symptoms. [10,59] As in Zuliani’s, the
every other week for 4 weeks, [54,55] cyclophosphamide role given to immunotherapy response becomes a
(Cyc) 750 mg/m , 3 times or mycophenolate mophetil retrospective feature that helps with the classification
2
600 mg/m , alone or in combination. [1,13] itself. This points out that, whenever a specific antibody
2
is detected, the diagnosis of NSAS is easily achieved;
The immunotherapy’s effectiveness can be checked
with HIC on frozen rat brain tissue to assess the lack of conversely, the hypothesis lies in a shady area. In
this paper, based on a review of the literature and the
immunostaining.
experience, the authors provide a simplified pathway
In the meanwhile, antiepileptic treatment is usually that may facilitate the identification and the early
continued, even though its real impact in modifying treatment of these forms. Concerning the diagnostic
the epileptic course remains uncertain as long as the algorithm many questions remain unanswered.
immune mechanism starts to decrease itself. The
decision whether to withdraw antiepileptic drugs A field that requires further work is the differential
or not should be made according to the patient, the diagnosis among the individual forms of NSAS but
specific disorder and EEG findings in the follow-up. this was beyond the aim of the paper. The spectrum
of signs and symptoms is wide and it is often
Psychiatric symptoms and involuntary movements, difficult to achieve a specific diagnosis on clinical
when present, can be treated symptomatically, ground only because of the overlapping of clinical
and medications with a broad effect on multiple signs. The recognition of some highly characteristic
symptoms are usually recommended. Long acting clinical features is sometimes possible and further
benzodiazepines, sedatives such as clonidine, and work using an integrated approach combining EEG,
anticonvulsivant drugs may be helpful in improving neuroimaging and early identification of the underlying
abnormal movements and mood instability. The immunological mechanism is highly recommendable,
management of psychiatric symptoms is more as it can lead to an early appropriate treatment and to
challenging: sedative and sleep medications other than the possibility of a perceivable clinical improvement.
152 Neuroimmunol Neuroinflammation | Volume 3 | July 8, 2016