BMJ Case Reports 2018; doi:10.1136/bcr-2017-224060
  • Findings that shed new light on the possible pathogenesis of a disease or an adverse effect

Primary lateral sclerosis-like picture in a patient with a remote history of anti-N-methyl-D- aspartate receptor (anti-NMDAR) antibody encephalitis

  1. Sam Salman2,3
  1. 1 Department of Neurology, Fiona Stanley Hospital, Perth, Western Australia, Australia
  2. 2 Department of Clinical Immunology, Fiona Stanley Hospital, Perth, Western Australia, Australia
  3. 3 Medical School, University of Western Australia, Perth, Western Australia, Australia
  1. Correspondence to Dr Mubeen Janmohamed, mubeen.janmohamed{at}
  • Accepted 28 May 2018
  • Published 10 June 2018


Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a well-recognised disorder, first fully characterised in 2007. The long-term sequelae reported thus far include relapses with typical, as well as partial aspects of the well-defined neuropsychiatric syndrome. Rarely, isolated atypical symptoms (diplopia, ataxia and tremor) have been reported as relapse phenomenon. We report a case of a patient with a remote history of likely anti-NMDAR encephalitis with the longest follow-up reported in the literature to date (22 years). The relapse presentation was of a purely upper motor neuron syndrome with a primary lateral sclerosis-like picture.


  • Contributors MJ reviewed archived medical records, was the author of the initial draft and was involved in patients care as the advanced trainee of the team. WK was the primary neurologist involved in patient’s current outpatient and inpatient evaluation/management and the main editor of the manuscript. He was also involved in her original care in 1994. MN was involved in patients follow-up, care and editing of the manuscript. SS was involved in refining and editing of manuscript, contribution to discussion from the neuroimmunology perspective and reviewed previous case series on the topic.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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