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

Piero Pavone
1  Unit of Pediatrics and Pediatric Emergency, University-Hospital “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy
,
Raffaele Falsaperla
1  Unit of Pediatrics and Pediatric Emergency, University-Hospital “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy
,
Martino Ruggieri
2  Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
,
Alberto Verrotti
3  Department of Pediatrics, University of Perugia, Perugia, Italy
,
Riccardo Lubrano
1  Unit of Pediatrics and Pediatric Emergency, University-Hospital “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy
,
Renata Rizzo
2  Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
,
Salomone Salomone
4  Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy
,
Andrea D. Praticò
2  Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
4  Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy
› Author Affiliations
Further Information

Publication History

19 November 2016

29 January 2017

Publication Date:
20 March 2017 (online)

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.