Journal of Pediatric Neurology 2019; 17(02): 077-084
DOI: 10.1055/s-0038-1636994
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Pediatric Relapsing Anti-NMDAR Encephalitis Crossing with Tumefactive Demyelinating Lesion

Sudarat Charoensanti
1   Department of Pediatrics, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand
,
Sutatip Pongcharoen
2   Division of Immunology, Department of Medicine, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand
3   Research Center for Academic Excellence in Petroleum, Petrochemical and Advanced Materials, Faculty of Science, Naresuan University, Phitsanulok, Thailand
4   Center of Excellence in Molecular Biotechnology, Faculty of Medical Science, Naresuan University, Phitsanulok, Thailand
,
Metha Apiwattanakul
5   Department of Neurology, Prasat Neurological Institute, Bangkok, Thailand
› Author Affiliations
Further Information

Publication History

18 November 2017

04 February 2018

Publication Date:
16 March 2018 (online)

Abstract

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalopathy characterized by neuropsychiatric symptoms, autonomic instability, and abnormal movements. Most children who undergo tumor removal and receive appropriate immunosuppressants have substantial neurological improvement. We report a 14-year-old girl with relapsing anti-NMDAR encephalitis who presented with paroxysmal tingling and weakness of the right lower limb. Despite long-term prophylaxis with immunotherapy (azathioprine), tumefactive demyelinating lesion and multifocal subcortical white matter lesions were identified on magnetic resonance imaging, which improved after high doses of steroid therapy. There may be overlap between the clinical manifestations of anti-NMDAR encephalitis and acquired demyelination syndromes in children.

 
  • References

  • 1 Dalmau J, Gleichman AJ, Hughes EG. , et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; 7 (12) 1091-1098
  • 2 Gleichman AJ, Spruce LA, Dalmau J, Seeholzer SH, Lynch DR. Anti-NMDA receptor encephalitis antibody binding is dependent on amino acid identity of a small region within the GluN1 amino terminal domain. J Neurosci 2012; 32 (32) 11082-11094
  • 3 Lin JJ, Lin KL, Hsia SH. , et al; Children with Encephalitis and/or Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE) Study Group. Anti-N-methyl-D-aspartate receptor encephalitis in Taiwan--a comparison between children and adults. Pediatr Neurol 2014; 50 (06) 574-580
  • 4 Bamford A, Crowe BHA, Hacohen Y. , et al. Pediatric herpes simplex virus encephalitis complicated by N-methyl-D-aspartate receptor antibody encephalitis. J Pediatric Infect Dis Soc 2015; 4 (02) e17-e21
  • 5 Yushvayev-Cavalier Y, Nichter C, Ramirez-Zamora A. Possible autoimmune association between herpes simplex virus infection and subsequent anti-N-methyl-d-aspartate receptor encephalitis: a pediatric patient with abnormal movements. Pediatr Neurol 2015; 52 (04) 454-456
  • 6 Armangue T, Titulaer MJ, Málaga I. , et al; Spanish Anti-N-methyl-D-Aspartate Receptor (NMDAR) Encephalitis Work Group. Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatr 2013; 162 (04) 850-856.e2
  • 7 Scheer S, John RM. Anti-N-methyl-D-aspartate receptor encephalitis in children and adolescents. J Pediatr Health Care 2016; 30 (04) 347-358
  • 8 Di Capua D, García-Ptacek S, García-García ME, Abarrategui B, Porta-Etessam J, García-Morales I. Extreme delta brush in a patient with anti-NMDAR encephalitis. Epileptic Disord 2013; 15 (04) 461-464
  • 9 Dalmau J, Gleichman AJ, Hughes EG. , et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008; 7 (12) 1091-1098
  • 10 Hacochen Y, Absound M, Hemingway C. , et al. NMDA receptor antibodies associated with distinct white matter syndromes. Neurol Neuroinflamation 2014; 1: 2
  • 11 Gahr M, Lauda F, Wigand ME. , et al. Periventricular white matter lesion and incomplete MRZ reaction in a male patient with anti-N-methyl-D-aspartate receptor encephalitis presenting with dysphoric mania. BMJ Case Rep 2015; 2015: 201
  • 12 Qin K, Wu W, Huang Y. , et al. Anti-N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis presents in atypical types and coexists with neuromyelitis optica spectrum disorder or neurosyphilis. BMC Neurol 2017; 17 (01) 1
  • 13 Luo JJ, Lv H, Sun W. , et al. Anti-N-methyl-d-aspartate receptor encephalitis in a patient with neuromyelitis optica spectrum disorders. Mult Scler Relat Disord 2016; 8: 74-77
  • 14 Titulaer MJ, Höftberger R, Iizuka T. , et al. Overlapping demyelinating syndromes and anti–N-methyl-D-aspartate receptor encephalitis. Ann Neurol 2014; 75 (03) 411-428
  • 15 Waschbisch A, Kallmünzer B, Schwab S. , et al. Demyelinating disease and anti-N-methyl-D-aspartate receptor immunoglobulin G antibodies: a case report. BMC Res Notes 2014; 7: 948
  • 16 Titulaer MJ, McCracken L, Gabilondo I. , et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol 2013; 12 (02) 157-165
  • 17 Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011; 10 (01) 63-74
  • 18 Gresa-Arribas N, Titulaer MJ, Torrents A. , et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol 2014; 13 (02) 167-177
  • 19 Alexopoulos H, Kosmidis ML, Dalmau J, Dalakas MC. Paraneoplastic anti-NMDAR encephalitis: long term follow-up reveals persistent serum antibodies. J Neurol 2011; 258 (08) 1568-1570
  • 20 Hansen HC, Klingbeil C, Dalmau J, Li W, Weissbrich B, Wandinger KP. Persistent intrathecal antibody synthesis 15 years after recovering from anti-N-methyl-D-aspartate receptor encephalitis. JAMA Neurol 2013; 70 (01) 117-119
  • 21 Gabilondo I, Saiz A, Galán L. , et al. Analysis of relapses in anti-NMDAR encephalitis. Neurology 2011; 77 (10) 996-999
  • 22 Given II CA, Stevens BS, Lee C. The MRI appearance of tumefactive demyelinating lesions. Am J Roentgenol 2004; 182 (01) 195-199
  • 23 Kiriyama T, Kataoka H, Taoka T. , et al. Characteristic neuroimaging in patients with tumefactive demyelinating lesions exceeding 30 mm. J Neuroimaging 2011; 21 (02) e69-e77
  • 24 Kobayashi M, Shimizu Y, Shibata N, Uchiyama S. Gadolinium enhancement patterns of tumefactive demyelinating lesions: correlations with brain biopsy findings and pathophysiology. J Neurol 2014; 261 (10) 1902-1910
  • 25 Barton J, Hardy TA, Riminton S. , et al. Tumefactive demyelination following treatment for relapsing multiple sclerosis with alemtuzumab. Neurology 2017; 88 (10) 1004-1006
  • 26 Kilic AK, Kurne AT, Oguz KK, Soylemezoglu F, Karabudak R. Mass lesions in the brain: tumor or multiple sclerosis? Clinical and imaging characteristics and course from a single reference center. Turk Neurosurg 2013; 23 (06) 728-735
  • 27 Jeong IH, Kim SH, Hyun JW, Joung A, Cho HJ, Kim HJ. Tumefactive demyelinating lesions as a first clinical event: clinical, imaging, and follow-up observations. J Neurol Sci 2015; 358 (01–02): 118-124
  • 28 Altintas A, Petek B, Isik N. , et al. Clinical and radiological characteristics of tumefactive demyelinating lesions: follow-up study. Mult Scler 2012; 18 (10) 1448-1453
  • 29 Polman CH, Reingold SC, Banwell B. , et al. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol 2011; 69 (02) 292-302
  • 30 Hardy TA, Chataway J. Tumefactive demyelination: an approach to diagnosis and management. J Neurol Neurosurg Psychiatry 2013; 84 (09) 1047-1053
  • 31 Kruer MC, Koch TK, Bourdette DN. , et al. NMDA receptor encephalitis mimicking seronegative neuromyelitis optica. Neurology 2010; 74 (18) 1473-1475
  • 32 Zoccarato M, Saddi MV, Serra G. , et al. Aquaporin-4 antibody neuromyelitis optica following anti-NMDA receptor encephalitis. J Neurol 2013; 260 (12) 3185-3187
  • 33 Ran Y, Wang L, Zhang F, Ao R, Dong Z, Yu S. Anti-NMDAR encephalitis followed by seropositive neuromyelitis optica spectrum disorder: a case report and literature review. Clin Neurol Neurosurg 2017; 155: 75-82
  • 34 Ishikawa N, Tajima G, Hyodo S, Takahashi Y, Kobayashi M. Detection of autoantibodies against NMDA-type glutamate receptor in a patient with recurrent optic neuritis and transient cerebral lesions. Neuropediatrics 2007; 38 (05) 257-260
  • 35 Lekoubou A, Viaccoz A, Didelot A. , et al. Anti-N-methyl-D-aspartate receptor encephalitis with acute disseminated encephalomyelitis-like MRI features. Eur J Neurol 2012; 19 (02) e16-e17
  • 36 Wang RJ, Chen BD, Qi D. Anti-N-methyl-D-aspartate receptor encephalitis concomitant with multifocal subcortical white matter lesions on magnetic resonance imaging: a case report and review of the literature. BMC Neurol 2015; 15: 107
  • 37 Pennington C, Livingstone S, Santosh C, Razvi S. N-methyl D-aspartate receptor antibody encephalitis associated with myelitis. J Neurol Sci 2012; 317 (01–02): 151-153
  • 38 Outteryck O, Baille G, Hodel J. , et al. Extensive myelitis associated with anti-NMDA receptor antibodies. BMC Neurol 2013; 13: 211
  • 39 Baheerathan A, Brownlee WJ, Chard DT, Shields K, Gregory R, Trip SA. Antecedent anti-NMDA receptor encephalitis in two patients with multiple sclerosis. Mult Scler Relat Disord 2017; 12: 20-22
  • 40 Ramberger M, Bsteh G, Schanda K. , et al. NMDA receptor antibodies: a rare association in inflammatory demyelinating diseases. Neurol Neuroimmunol Neuroinflamm 2015; 2 (05) e141
  • 41 KInzel S, Weber MS. The role of peripheral CNS-directed antibodies in promoting inflammatory CNS demyelination. Brain Sci 2017; 7: 70
  • 42 Hamid SHM, Whittam D, Saviour M. , et al. Seizures and encephalitis in myelin oligodendrocyte glycoprotein IgG disease vs aquaporin 4 IgG disease. JAMA Neurol 2018; 75 (01) 65-71
  • 43 Jarius S, Ruprecht K, Kleiter I. , et al; in cooperation with the Neuromyelitis Optica Study Group (NEMOS). MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation 2016; 13 (01) 280