Journal of Neurology

, Volume 259, Issue 9, pp 1979–1981 | Cite as

NMDA receptor autoantibodies in sporadic Creutzfeldt-Jakob disease

  • Graham Mackay
  • Kate Ahmad
  • Jon Stone
  • Cathie Sudlow
  • David Summers
  • Richard Knight
  • Robert Will
  • Sarosh R. Irani
  • Angela Vincent
  • Paul Maddison
Letter to the Editors

Dear Sirs,

Sporadic Creutzfeldt-Jakob disease (sCJD) and N-methyl- d-aspartate receptor antibody (NMDAR-Ab) encephalitis [ 1], share similar clinical features including prominent psychiatric symptoms, memory problems, and seizures (Table  1). Previously, other forms of autoimmune encephalitis have been misdiagnosed as CJD, including Hashimoto’s and voltage-gated potassium channel complex antibody-associated encephalopathies [ 2, 3]. Here, conversely, we describe two patients presenting with progressive cognitive impairment and NMDAR-Abs with confirmed sCJD at post mortem.
Table 1

Key clinical and investigation features


NMDA encephalitis

Sporadic CJD

Variant CJD

Age (range)

Median 23 years (range 5–76) [5, 12]

Median 66 years (range 15–94) [13]

Median 27 (range 12–74) [13]

Sex ratio

80–90 % female [5, 12] (n = 44, 100)

50 % female [13]

41.5 % female [13]

Psychiatric symptoms at onset

77 % [5, 12] (n = 44, 100)

15 % [14] (n = 88)

92 % [19] (n = 106)

Akinetic mutism

45–88 % [5, 12]...


Encephalitis Prion Disease S100b Level Progressive Cognitive Impairment Spongiform Degeneration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



The National CJD research and surveillance unit is funded by the Department of Health and the Scottish Government. We are grateful for a remarkable level of cooperation from clinicians across the UK. We are grateful to the relatives of these patients for their permission to publish their cases reports. SRI was supported by the National Institute for Health Research (NIHR), Department of Health, UK, and AV receives funding from the NIHR Oxford Biomedical Research Centre.

Conflicts of interest


Supplementary material

415_2012_6489_MOESM1_ESM.docx (161 kb)
Supplementary material 1 (DOCX 161 kb)


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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Graham Mackay
    • 1
  • Kate Ahmad
    • 2
  • Jon Stone
    • 2
  • Cathie Sudlow
    • 2
  • David Summers
    • 3
  • Richard Knight
    • 1
  • Robert Will
    • 1
  • Sarosh R. Irani
    • 4
  • Angela Vincent
    • 4
  • Paul Maddison
    • 5
  1. 1.National CJD Research and Surveillance UnitEdinburghUK
  2. 2.Department of NeurologyWestern General HospitalEdinburghUK
  3. 3.Department of NeuroradiologyWestern General HospitalEdinburghUK
  4. 4.Nuffield Department of Clinical NeurosciencesJohn Radcliffe HospitalOxfordUK
  5. 5.Nottingham University Hospitals NHS TrustNottinghamUK

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