LGI1 antibody encephalopathy overlapping with sporadic Creutzfeldt-Jakob disease

B Kim; P Yoo; T Sutherland; A Boyd; C Stehmann; C McLean; S Collins

Journal article

LGI1 antibody encephalopathy overlapping with sporadic Creutzfeldt-Jakob disease

B Kim, P Yoo, T Sutherland, A Boyd, C Stehmann, C McLean, S Collins

Neurology Neuroimmunology and Neuroinflammation | LIPPINCOTT WILLIAMS & WILKINS | Published : 2016

DOI: 10.1212/NXI.0000000000000248

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Abstract

Objective: To report a rare case of leucine-rich, glioma inactivated 1 (LGI1) antibody–mediated autoimmune encephalopathy clinically overlapping with pathologically confirmed sporadic Creutzfeldt-Jakob disease (CJD). Methods: The patient was investigated with repeated brain MRI, EEG, CSF examination, whole-body fluorodeoxy-glucose positron emission tomography, genetic analysis of the prion protein gene (PRNP), and extensive serologic screening for paraneoplastic and autoimmune encephalopathy markers. Written informed consent was obtained from the patient’s next of kin for access to clinical files for research purposes and for publication. Results: The patient was a 77-year-old man who presen..

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University of Melbourne Researchers

Tom Sutherland Author

Catriona McLean Author

Steven Collins Author

Related Projects (1)

DETERMINING NORMAL PRION PROTEIN FUNCTION, EMPHASISING THE ROLE PLAYED BY PROTEIN CLEAVAGE, AND ALSO DEFINING THE RELATIONSHIP BETWEEN SURVEILLANCE INTENSITY AND ANNUAL SPORADIC CJD INCIDENCE

As a neurologist undertaking research into prion diseases over an extended period, I have been able to lead and participate in many projects..

Grants

Awarded by National Health and Medical Research Council

Funding Acknowledgements

The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) is funded by the Commonwealth Department of Health. S.C. was funded in part by NHMRC Practitioner Fellowship (APP1005816).