Clinical Course of N-Methyl-D-Aspartate Receptor Encephalitis and the Effectiveness of Cyclophosphamide Treatment

Piero Pavone; Raffaele Falsaperla; Martino Ruggieri; Alberto Verrotti; Riccardo Lubrano; Renata Rizzo; Salomone Salomone; Andrea D. Praticò

doi:10.1055/s-0037-1599832

Journal of Pediatric Neurology, Table of Contents

Journal of Pediatric Neurology 2017; 15(02): 084-089
DOI: 10.1055/s-0037-1599832

Case Report

Georg Thieme Verlag KG Stuttgart · New York

Clinical Course of N-Methyl-D-Aspartate Receptor Encephalitis and the Effectiveness of Cyclophosphamide Treatment

Authors

Piero Pavone

¹Unit of Pediatrics and Pediatric Emergency, University-Hospital “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy
Raffaele Falsaperla

¹Unit of Pediatrics and Pediatric Emergency, University-Hospital “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy
Martino Ruggieri

²Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
Alberto Verrotti

³Department of Pediatrics, University of Perugia, Perugia, Italy
Riccardo Lubrano

¹Unit of Pediatrics and Pediatric Emergency, University-Hospital “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy
Renata Rizzo

²Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
Salomone Salomone

⁴Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy
Andrea D. Praticò

²Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy

⁴Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy

Abstract

We describe the cases of two unrelated girls, aged 5 and 7, respectively, affected by N-methyl-D-aspartate receptor (NMDAr) encephalitis. The clinical records of both patients were reviewed retrospectively including family and personal history, clinical findings, laboratory, and neuroradiological examinations, electroencephalogram, and treatment performed during admissions to the unit. In both patients, the clinical course was slow and progressive. Both showed anti-NMDAr antibodies in serum and cerebrospinal fluid. Treatment with intravenous immunoglobulin and methylprednisolone was not efficacious in the long term, with several relapses occurring in both patients. Second-line treatment with cyclophosphamide (1 g/m² once a month) resulted in improvement of symptoms and disappearance of clinical signs that were sustained at 24 months follow-up. Side effects included neutropenia in one patient.

Keywords

NMDAr - encephalitis - intravenous immunoglobulins - steroids - cyclophosphamide

Full Text

References

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