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Two patients with an anti-N-methyl-D-aspartate receptor antibody syndrome-like presentation and negative results of testing for autoantibodies.

Publication ,  Journal Article
Shah, R; Veerapandiyan, A; Winchester, S; Gallentine, W; Mikati, MA
Published in: Pediatr Neurol
December 2011

We describe two boys whose distinct and remarkable clinical pictures suggested the possibility of anti-N-methyl-d-aspartate receptor antibody encephalitis. Both patients responded to immunotherapy, but neither manifested that antibody. Patient 1 exhibited florid encephalopathy with psychotic manifestations including inappropriate affect, intermittent delirium, visual hallucinations, severe anorexia, agitation, paranoid ideation, and abnormal electroencephalogram results. He responded to intravenous immunoglobulin, with steady improvement over 3 months to almost complete remission for 1 year, followed by a relapse that again responded, more quickly, to intravenous immunoglobulin. A second relapse occurred 1 month later, and again responded to intravenous immunoglobulin. Patient 2 exhibited progressive, severely debilitating limb dystonia that worsened over 1.5 years, with milder psychiatric symptoms including mood instability, aggressiveness, impulsivity, and depression. When he developed thymic hyperplasia 1.5 years into his illness, he underwent a thymectomy, and improved significantly on a regimen of plasmapheresis and intravenous immunoglobulin. Patients presenting with symptoms suggestive of autoimmune encephalitis, but without antibodies, may still respond to immunotherapy.

Duke Scholars

Published In

Pediatr Neurol

DOI

EISSN

1873-5150

Publication Date

December 2011

Volume

45

Issue

6

Start / End Page

412 / 416

Location

United States

Related Subject Headings

  • Receptors, N-Methyl-D-Aspartate
  • Neurology & Neurosurgery
  • Male
  • Magnetic Resonance Imaging
  • Humans
  • Encephalitis
  • Electroencephalography
  • Child
  • Autoantibodies
  • Antibodies
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Shah, R., Veerapandiyan, A., Winchester, S., Gallentine, W., & Mikati, M. A. (2011). Two patients with an anti-N-methyl-D-aspartate receptor antibody syndrome-like presentation and negative results of testing for autoantibodies. Pediatr Neurol, 45(6), 412–416. https://doi.org/10.1016/j.pediatrneurol.2011.09.012
Shah, Rikin, Aravindhan Veerapandiyan, Sara Winchester, William Gallentine, and Mohamad A. Mikati. “Two patients with an anti-N-methyl-D-aspartate receptor antibody syndrome-like presentation and negative results of testing for autoantibodies.Pediatr Neurol 45, no. 6 (December 2011): 412–16. https://doi.org/10.1016/j.pediatrneurol.2011.09.012.
Shah R, Veerapandiyan A, Winchester S, Gallentine W, Mikati MA. Two patients with an anti-N-methyl-D-aspartate receptor antibody syndrome-like presentation and negative results of testing for autoantibodies. Pediatr Neurol. 2011 Dec;45(6):412–6.
Shah, Rikin, et al. “Two patients with an anti-N-methyl-D-aspartate receptor antibody syndrome-like presentation and negative results of testing for autoantibodies.Pediatr Neurol, vol. 45, no. 6, Dec. 2011, pp. 412–16. Pubmed, doi:10.1016/j.pediatrneurol.2011.09.012.
Shah R, Veerapandiyan A, Winchester S, Gallentine W, Mikati MA. Two patients with an anti-N-methyl-D-aspartate receptor antibody syndrome-like presentation and negative results of testing for autoantibodies. Pediatr Neurol. 2011 Dec;45(6):412–416.
Journal cover image

Published In

Pediatr Neurol

DOI

EISSN

1873-5150

Publication Date

December 2011

Volume

45

Issue

6

Start / End Page

412 / 416

Location

United States

Related Subject Headings

  • Receptors, N-Methyl-D-Aspartate
  • Neurology & Neurosurgery
  • Male
  • Magnetic Resonance Imaging
  • Humans
  • Encephalitis
  • Electroencephalography
  • Child
  • Autoantibodies
  • Antibodies