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[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis].
Rev Neurol. 2012 Apr 01; 54(7):420-4.RN

Abstract

INTRODUCTION

Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults.

CASE REPORT

We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment.

CONCLUSIONS

Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.

Authors+Show Affiliations

Hospital Universitario 12 de Octubre, Madrid, Espana. noenu_enamorado@yahoo.esNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article
Review

Language

spa

PubMed ID

22451129

Citation

Nunez-Enamorado, N, et al. "[Fast and Spectacular Clinical Response to Plasmapheresis in a Paediatric Case of anti-NMDA Encephalitis]." Revista De Neurologia, vol. 54, no. 7, 2012, pp. 420-4.
Nunez-Enamorado N, Camacho-Salas A, Belda-Hofheinz S, et al. [Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. Rev Neurol. 2012;54(7):420-4.
Nunez-Enamorado, N., Camacho-Salas, A., Belda-Hofheinz, S., Cordero-Castro, C., Simon-De Las Heras, R., Saiz-Diaz, R., Martinez-Sarries, F. J., Martinez-Menendez, B., & Graus, F. (2012). [Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. Revista De Neurologia, 54(7), 420-4.
Nunez-Enamorado N, et al. [Fast and Spectacular Clinical Response to Plasmapheresis in a Paediatric Case of anti-NMDA Encephalitis]. Rev Neurol. 2012 Apr 1;54(7):420-4. PubMed PMID: 22451129.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. AU - Nunez-Enamorado,N, AU - Camacho-Salas,A, AU - Belda-Hofheinz,S, AU - Cordero-Castro,C, AU - Simon-De Las Heras,R, AU - Saiz-Diaz,R, AU - Martinez-Sarries,F J, AU - Martinez-Menendez,B, AU - Graus,F, PY - 2012/3/28/entrez PY - 2012/3/28/pubmed PY - 2012/7/25/medline SP - 420 EP - 4 JF - Revista de neurologia JO - Rev Neurol VL - 54 IS - 7 N2 - INTRODUCTION: Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. CASE REPORT: We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. CONCLUSIONS: Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement. SN - 1576-6578 UR - https://www.unboundmedicine.com/medline/citation/22451129/[Fast_and_spectacular_clinical_response_to_plasmapheresis_in_a_paediatric_case_of_anti_NMDA_encephalitis]_ L2 - http://www.revneurol.com/LinkOut/formMedLine.asp?Refer=2012067&Revista=RevNeurol DB - PRIME DP - Unbound Medicine ER -