Page 160 - Read Online

According to Suleiman classification, in order to help must be taken for autoantibody testing, and a Ig total
in the diagnosis 5 different autoimmune epilepsy level as well as a study of lymphocytic profile should
categories are identifiable, on the basis both of the be performed, in order to get a value to compare later
data obtained and of the immunotherapy response. on with [Figure 2].
Treatment may lead to remission, but the response
depends on the specific form and possible association If NSAbs are detected, the diagnosis is usually easily
with malignancy. [7,45] In some forms, such as NMDA-R achieved. On a practical level, the expanding NSAbs
encephalitis, a complete recovery occurs in about spectrum could make it difficult to choose which
80% with a very low mortality rate. [13,14,46-48] When a antibody to check first. In order to direct the diagnostic
complete remission does not occur, sequelae ranging pathway, indirect immunohistochemistry on rat
from drug-resistant epilepsy associated with cognitive brain tissue or immunocytochemistry in primary
decline, to milder cognitive impairment are easily rat neuronal culture can highlight staining patterns
detectable. [1,12,13,47] evocative for surface (e.g. neuropil) or intracellular
antigens.
Following the treatment phase, EEG interictal activities
slowly improve and longitudinal MRI studies can This preliminary screening must be confirmed by more
reveal a trend toward complete resolution, especially in specific tecniques, such as cell-based assay, ELISA or
[51]
some specific NSAS and when a rapid immunotherapy radioimmunoassay. A blended approach reduces the
[52]
is administered. A more severe course towards a false positive rate, providing an efficient diagnostic
global atrophy predominantly affecting hippocampus, tool for pediatric NSAS.
frontal and parietal regions usually characterizes the
remaining cases. An EEG monitoring including A paraneoplastic cause is much less probable in
[43]
awakeness and sleep recordings (e.g. 40 min cad) as children, so that testing for onconeural antibodies (Hu,
well as a neuroradiological surveillance are thereby Ma2, CV2/CRMP5, Ri, amphiphysin) can be not strictly
suggested. necessary, at first instance at least. Nevertheless, if a
paraneoplastic clinical picture shows-up, a commercial
Ideally, CSF and autoimmune responses should be immunoblotting assay specifically designed can be
investigated as soon as there is suspicion of autoimmune properly used to manage a differential diagnosis.
encephalitis.
TUMOR SURVEILLANCE
CSF FINDINGS
Because of the low prevalence of malignancy in
As in adult, CSF usually shows a mild to moderate pediatric NSAS, many authors consider tumor
lymphocytic pleocytosis (< 100 white blood cells/μL), surveillance not strictly necessary in the first
increased protein concentration (< 150 mg/dL), normal instance, with the only exception related to NMDA-R
glucose level and frequently elevated IgG index; encephalitis. Nevertheless it may become increasingly
oligoclonal bands result detectable in about 50% of relevant in patients who are older at the time of clinical
cases. onset. [13]

In children, elevated CSF neopterin can be used as an TREATMENT
additional marker of CNS inflammation. [10,49]
Due to antibodies pathogenicity, treatment is focused
A recent study underlies the role of the CXCL13 on reducing the serum antibodies titer. There is no
chemokine as a potential CSF biomarker of clinical consensus on the immunotherapy approach to carry
outcome in anti-NMDAR encephalitis, its prolonged or out, but it has become increasingly clear that starting
secondary elevation suggesting a limited response to treatment as early as possible is crucial to achieve a
immunotherapy, an higher risk of relapses, and thereby better clinical outcome. [12,48]
the need to a more aggressive therapeutic approach. [50]
At seizure onset, antiepileptic therapy, with few
IMMUNOLOGICAL FINDINGS exceptions, usually results ineffective. [9]

As soon as an autoimmune condition is suspected During the acute phase, as the diagnostic work out
(at prodromal or early acute stage) and before may take time, starting immunotherapy empirically
immunotherapy is started, a serum and/or CSF sample is highly recommended. Although some patients
Neuroimmunol Neuroinflammation | Volume 3 | July 8, 2016 151