Anti-NMDAR encephalitis with simultaneous hypertrophic pachymeningitis in a 68-year-old male: a rare case report

BMC Neurol. 2019 Aug 31;19(1):215. doi: 10.1186/s12883-019-1444-x.

Abstract

Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most frequent types of autoimmune encephalitis. However, the instigating mechanisms are as yet not fully ascertained. Cardinal clinical manifestations of anti-NMDAR encephalitis include acute behavioural change, psychosis, and catatonia. As the level of diagnosis increases, encephalitis becomes more common, but there are never been published in patients with anti-NMDAR encephalitis and simultaneous hypertrophic pachymeningitis.

Case presentation: A sixty-eight-year-old man who presented with mental, behavioral abnormalities, unstable walking, headaches, and erratic hand movements. The neuropsychiatric symptoms and cerebrospinal fluid examination was consistent with the diagnosis criteria of anti-NMDAR encephalitis. Magnetic resonance imaging of the brain showed a thickening of dura mater localized at the left tentorium cerebelli, left cerebral hemisphere, and cerebral falx; the thickening dura mater was characterized by an intense contrast enhancement after the administration of gadolinium. High doses of intravenous methylprednisolone were administrated during his hospitalization. After 5 days, the patient's condition improved.

Conclusions: We herein describe a rare case of a 68-year-old man with anti-NMDAR encephalitis presenting with concomitant hypertrophic pachymeningitis. We systematically expounded anti-NMDAR encephalitis and hypertrophic pachymeningitis, and made bold conjectures on the etiology and pathogenesis of these two diseases, hoping to stimulate new ideas from clinicians and basic medical researchers.

Keywords: Anti-NMDA receptor encephalitis; Autoimmune encephalitis; Dural hypertrophy; Hypertrophic pachymeningitis (HP); NMDAR.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Anti-N-Methyl-D-Aspartate Receptor Encephalitis / diagnosis*
  • Brain / diagnostic imaging
  • Dura Mater / pathology
  • Headache / etiology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Meningitis / complications
  • Meningitis / diagnosis*
  • Methylprednisolone / therapeutic use
  • Psychotic Disorders / etiology

Substances

  • Methylprednisolone