Autoimmune encephalopathies in children: diagnostic clues and therapeutic challenges

Abstract

Neuronal surface antibody syndromes (NSAS) encompass a variety of disorders associated with "neuronal surface antibodies". These share clinical and neuroradiological features that pose challenges related to their recognition and treatment. Recent epidemiological studies show a clear predominance for the glutamate-N-methyl-D-aspartate receptor encephalitis in both adults and pediatric population. Despite this, the overall NSAS's incidence remains underestimated, and diagnosis persists to be not always easy to achieve. Based on current literature data, in this paper the authors propose a diagnostic pathway to approach and treat pediatric NSAS. An autoimmune etiology can be suggested through the integration of clinical, immunological, electrophysiological and neuroradiological data. On that basis, a target treatment can be started, consisting of corticosteroids and intravenous immunoglobulin or plasma exchange as a first-line immunotherapy, followed by second-line drugs including rituximab, cyclophosphamide or mycophenolate mophetil, if the case. In children a prompt diagnosis and a targeted treatment may lead to a better clinical outcome. Nevertheless further studies are required to assess the need of more tailored treatments according to longterm outcome findings and prognostic factors in different NSAS.

The role of clinical diagnostic criteria for anti-N-methyl-D-aspartate receptor encephalitis in children: A case report

“…In the absence of a malignancy, most children with autoimmune encephalitis have good prognoses. [20][21][22] In conclusion, the clinical diagnostic criteria for anti-NMDAR encephalitis used in this study have high sensitivity and specificity and can be applied in limitedresource settings to identify patients with probable anti-NMDAR encephalitis and guide the initiation of immunotherapy before antibody testing results are available. Conversely, in patients who do not meet the criteria, other differential diagnoses, such as herpes simplex encephalitis, should be considered.…”

“…Reports have shown better results when immunotherapy is given as a combination of steroids and IVIG. 17,[19][20][21][22] Half of anti-NMDAR encephalitis patients respond well to steroids as the first-line treatment. 3.…”

The relation between lesions and localization of sources of slow biphasic complexes in encephalitis

“…Further work is suggested to deepen the promising results found in this pilot study on a few patients. In particular, using a high-density system for EEG acquisition could allow us to better locate the sources of SBCs, to be correlated with MRI results [30] . Moreover, the use of functional MRI in synchronous with EEG registration [31] could help in investigating better SBC sources.…”

A Rare Case of Anti-Ma2 Antibody-Mediated Autoimmune Encephalomyelitis in Childhood

“…Although no evidence-based treatment protocols are currently available for pediatric AE, the first-line treatment often consists of high-dose steroids, IVIG, and/or plasmapheresis. Clinicians can escalate to second-line immunotherapy, including treatment with rituximab and cyclophosphamide, when first-line therapies have failed [6]. Anti-Ma2 antibody is not directly pathogenic, but indicates a T-cell-mediated immune response against neurons.…”