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Arch Pediatr. 2014 Jun;21(6):620-3. doi: 10.1016/j.arcped.2014.03.005. Epub 2014 May 13.

[N-methyl-D-aspartate receptor antibody encephalitis: value of immunomodulatory therapy].

[Article in French]

Author information

1
Service de neuropédiatrie, unité Inserm U930, hôpital d'Enfants Gatien de Clocheville, université de Tours, CHU de Tours, 49, boulevard Béranger, 37044 Tours cedex 01, France.
2
Laboratoire d'immunologie, Labex Mab Improve, CHRU de Tours, 37000 Tours, France.
3
Laboratoire d'immunologie, Labex Mab Improve, CHRU de Tours, 37000 Tours, France; Université François-Rabelais de Tours, 37044 Tours, France.
4
Unité de néphrologie pédiatrique, hôpital Clocheville, CHRU de Tours, 37000 Tours, France.
5
Service de neuropédiatrie, Centre de référence des maladies inflammatoires du cerveau, AP-HP, 94275 Le Kremlin-Bicêtre, France.
6
Service de neuropédiatrie, unité Inserm U930, hôpital d'Enfants Gatien de Clocheville, université de Tours, CHU de Tours, 49, boulevard Béranger, 37044 Tours cedex 01, France; Université François-Rabelais de Tours, 37044 Tours, France; UMR Inserm U930, CNRS FRE 2448, 37044 Tours, France. Electronic address: castelnau@med.univ-tours.fr.

Abstract

INTRODUCTION:

Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is little known to pediatricians and likely underdiagnosed. The child's vital and cognitive prognosis is at stake. The use of immunomodulatory drugs, such as rituximab has led to spectacular results, but many questions remain about its mode of action in this type of pathology.

CASE REPORT:

We report the case of a 6-year-old girl with no medical history, admitted for status epilepticus preceded by behavior symptoms and sleep disorders. Gradually, the child became bedridden, mute, and animated by predominantly orofacial dyskinesia. Examinations were normal (cerebrospinal fluid [CSF] analysis, brain MRI). The diagnosis was established by the presence of NMDA-R antibodies in the CSF. After exclusion of a tumor-associated syndrome, treatment was started initially by intravenous immunoglobulins, then by plasma exchange, and finally rituximab. The patient was cured with rituximab despite an unusually early recovery of the B-cell pool.

DISCUSSION:

Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is a severe but potentially reversible neurologic disorder only recently described, even in childhood. It may be reversible without sequelae if diagnosed and treated early. The use of immunomodulatory therapy, such as rituximab seemingly improves the outcome. Immunological monitoring is needed to better understand its mechanism of action in autoimmune diseases of the nervous system in childhood.

PMID:
24832126
DOI:
10.1016/j.arcped.2014.03.005
[Indexed for MEDLINE]

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