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Atypical presentation of anti-N-methyl-D-aspartate receptor encephalitis: two case reports.
J Med Case Rep. 2017 Aug 16; 11(1):225.JM

Abstract

BACKGROUND

Anti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis. The disease is often secondary to a neoplastic lesion, seldom diagnosed years later. Psychiatric symptoms are prevalent in adults; neurologic symptoms are more evident in children, who typically present primarily with neurological symptoms. To the best of our knowledge, the association with juvenile idiopathic arthritis has not been described.

CASE PRESENTATION

We report the cases of two caucasian girls with an atypical presentation. The first patient was an 8-year-old girl with normal psychomotor development. Over a 4-month period she developed behavioral problems, speech impairment, and deterioration in academic skills. Within 8 months from the onset of symptoms, choreic movements gradually appeared. Hematological, neuroradiological, and neurophysiological examinations were negative; however, her symptoms worsened and treatment with prednisone was started. Although her choreic movements improved within 1 month, her neuropsychological and behavioral symptoms continued. Anti-N-methyl-D-aspartate receptor antibodies in cerebrospinal fluid and in blood were detected. Therapy with intravenously administered immunoglobulins was administered, without improvement of symptoms. After 2 months of steroid treatment, she suddenly started to pronounce some words with a progressive improvement in language and behavior. The second patient was a 14-year-old girl with classic anti-N-methyl-D-aspartate receptor encephalitis, treated successfully with intravenously administered immunoglobulins and methylprednisolone, followed by orally administered prednisone, who developed chronic arthritis of the hip. The arthritis was confirmed by magnetic resonance imaging and associated to antinuclear antigen antibody positivity. One year after the encephalitis presentation, an ovarian cystic mass was identified as a teratoma. The surgical resection of the mass was followed by the resolution of the psychotic spectrum and arthritis.

CONCLUSIONS

Anti-N-methyl-D-aspartate receptor encephalitis in pediatric patients can present initially with neuropsychological and behavioral symptoms. In the literature, the association of anti-N-methyl-D-aspartate receptor encephalitis with juvenile idiopathic arthritis is not yet described: to the best of our knowledge, this is the first case reported. The link to a neoplastic lesion can explain the favorable course of encephalitis and arthritis, after the surgical resection of the mass. Early diagnosis and treatment can improve the patient's outcome.

Authors+Show Affiliations

Department Pro.Sa:M.I. "G. D'Alessandro" ARNAS, University of Palermo, Palermo, Italy. mariacristina.maggio@unipa.it.Department of Rheumatology, Meyer Children's Hospital, Florence, Italy.Department of Advanced Diagnostic and Clinical Trials, Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.Department of Advanced Diagnostic and Clinical Trials, Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.Department of Advanced Diagnostic and Clinical Trials, Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste, Italy.Unit of Paediatric Neuropsychiatry, Children Hospital "G. Di Cristina", ARNAS, Palermo, Italy.Unit of Paediatric Neuropsychiatry, Children Hospital "G. Di Cristina", ARNAS, Palermo, Italy.Department Pro.Sa:M.I. "G. D'Alessandro" ARNAS, University of Palermo, Palermo, Italy.Department of Rheumatology, Meyer Children's Hospital, Florence, Italy.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

28810906

Citation

Maggio, Maria Cristina, et al. "Atypical Presentation of anti-N-methyl-D-aspartate Receptor Encephalitis: Two Case Reports." Journal of Medical Case Reports, vol. 11, no. 1, 2017, p. 225.
Maggio MC, Mastrangelo G, Skabar A, et al. Atypical presentation of anti-N-methyl-D-aspartate receptor encephalitis: two case reports. J Med Case Rep. 2017;11(1):225.
Maggio, M. C., Mastrangelo, G., Skabar, A., Ventura, A., Carrozzi, M., Santangelo, G., Vanadia, F., Corsello, G., & Cimaz, R. (2017). Atypical presentation of anti-N-methyl-D-aspartate receptor encephalitis: two case reports. Journal of Medical Case Reports, 11(1), 225. https://doi.org/10.1186/s13256-017-1388-y
Maggio MC, et al. Atypical Presentation of anti-N-methyl-D-aspartate Receptor Encephalitis: Two Case Reports. J Med Case Rep. 2017 Aug 16;11(1):225. PubMed PMID: 28810906.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Atypical presentation of anti-N-methyl-D-aspartate receptor encephalitis: two case reports. AU - Maggio,Maria Cristina, AU - Mastrangelo,Greta, AU - Skabar,Aldo, AU - Ventura,Alessandro, AU - Carrozzi,Marco, AU - Santangelo,Giuseppe, AU - Vanadia,Francesca, AU - Corsello,Giovanni, AU - Cimaz,Rolando, Y1 - 2017/08/16/ PY - 2017/04/26/received PY - 2017/07/13/accepted PY - 2017/8/17/entrez PY - 2017/8/16/pubmed PY - 2018/7/11/medline KW - Anti-N-methyl-D-aspartate receptor encephalitis KW - Chorea KW - Juvenile idiopathic arthritis KW - Psychiatric symptoms KW - Speech disorders KW - Teratoma SP - 225 EP - 225 JF - Journal of medical case reports JO - J Med Case Rep VL - 11 IS - 1 N2 - BACKGROUND: Anti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis. The disease is often secondary to a neoplastic lesion, seldom diagnosed years later. Psychiatric symptoms are prevalent in adults; neurologic symptoms are more evident in children, who typically present primarily with neurological symptoms. To the best of our knowledge, the association with juvenile idiopathic arthritis has not been described. CASE PRESENTATION: We report the cases of two caucasian girls with an atypical presentation. The first patient was an 8-year-old girl with normal psychomotor development. Over a 4-month period she developed behavioral problems, speech impairment, and deterioration in academic skills. Within 8 months from the onset of symptoms, choreic movements gradually appeared. Hematological, neuroradiological, and neurophysiological examinations were negative; however, her symptoms worsened and treatment with prednisone was started. Although her choreic movements improved within 1 month, her neuropsychological and behavioral symptoms continued. Anti-N-methyl-D-aspartate receptor antibodies in cerebrospinal fluid and in blood were detected. Therapy with intravenously administered immunoglobulins was administered, without improvement of symptoms. After 2 months of steroid treatment, she suddenly started to pronounce some words with a progressive improvement in language and behavior. The second patient was a 14-year-old girl with classic anti-N-methyl-D-aspartate receptor encephalitis, treated successfully with intravenously administered immunoglobulins and methylprednisolone, followed by orally administered prednisone, who developed chronic arthritis of the hip. The arthritis was confirmed by magnetic resonance imaging and associated to antinuclear antigen antibody positivity. One year after the encephalitis presentation, an ovarian cystic mass was identified as a teratoma. The surgical resection of the mass was followed by the resolution of the psychotic spectrum and arthritis. CONCLUSIONS: Anti-N-methyl-D-aspartate receptor encephalitis in pediatric patients can present initially with neuropsychological and behavioral symptoms. In the literature, the association of anti-N-methyl-D-aspartate receptor encephalitis with juvenile idiopathic arthritis is not yet described: to the best of our knowledge, this is the first case reported. The link to a neoplastic lesion can explain the favorable course of encephalitis and arthritis, after the surgical resection of the mass. Early diagnosis and treatment can improve the patient's outcome. SN - 1752-1947 UR - https://www.unboundmedicine.com/medline/citation/28810906/Atypical_presentation_of_anti_N_methyl_D_aspartate_receptor_encephalitis:_two_case_reports_ L2 - https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-017-1388-y DB - PRIME DP - Unbound Medicine ER -