Fatal Autoimmune Anti-NMDA-Receptor Encephalitis with Poor Prognostication Score in a Young Kenyan Female

Abstract

Auto-immune N-methyl-D-aspartate receptor encephalitis (NMDARE) is a relatively recently described cause of acute encephalopathy with very few reports from sub-Saharan Africa (SSA). We report a case of NMDARE in a young Kenyan female who was transferred to our facility with headaches, insomnia, behaviour changes and latterly pathognomonic orofacial dyskinesias. We comprehensively ruled out infectious and other inflammatory/auto-immune causes. She was diagnosed with NMDARE by positive antibody testing in serum and cerebrospinal fluid and changes on brain magnetic resonance imaging. She was immunosuppressed with high-dose steroids, intravenous immunoglobulins, plasma exchange and rituximab, and showed signs of neurological improvement clinically and radiologically. Unfortunately, she succumbed to septic shock from prolonged intensive care. This is the first report of NMDARE in an indigenous patient from the eastern SSA. The majority (>80%) of patients are either left with mild disability or make a full recovery after NMDARE, but some factors – which comprise the NMDARE One-Year Functional Status (NEOS) prognostication score – can adversely affect outcome, as was the case in our patient.

Keywords:

• auto-immune encephalitis
• anti-NMDA receptor encephalitis
• sub-Saharan Africa

Data Sharing Statement

The clinical history and imaging data used to support the findings of this study are included within the article.

Research Ethics and Consent

This study was conducted in accordance with the principles stated in the Declaration of Helsinki. The study is exempted from our formal institutional ethics review given it is a case report. Informed and written consent for the case details to be published was obtained from the registered next of kin (the father) of the patient after the patient’s demise, and the consent form is filed in patient’s medical case file. The authors have removed all patient identifiable information from the manuscript.

Acknowledgments

The authors would like to acknowledge the help of Dr. Sheila Waa (consultant neuro-radiologist) for providing the neuro-imaging pictures, and Dr. Juzar Hooker, Dr. Peter Mativo and Dr. Sylvia Mbugua (consultant neurologists) for their clinical input with the case, all at the Aga Khan University Hospital, Nairobi.

Author Contributions

All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.

Disclosure

The authors report no conflicts of interest in this work and that there is no conflict of interest regarding the publication of this paper.

Additional information

Funding

This case series did not receive any specific funding. The cases have been compiled as part of authors’ current employment under the Aga Khan University Medical College of East Africa, Faculty of Health Sciences.