Anti-dipeptidyl-peptidase-like protein 6 encephalitis, a rare cause of reversible rapid progressive dementia and insomnia

Qinming Zhou; Xiaodong Zhu; Huanyu Meng; Min Zhang; Sheng Chen

doi:10.1016/j.jneuroim.2019.577114

Anti-dipeptidyl-peptidase-like protein 6 encephalitis, a rare cause of reversible rapid progressive dementia and insomnia

J Neuroimmunol. 2020 Feb 15:339:577114. doi: 10.1016/j.jneuroim.2019.577114. Epub 2019 Nov 18.

Authors

Qinming Zhou¹, Xiaodong Zhu², Huanyu Meng¹, Min Zhang³, Sheng Chen⁴

Affiliations

¹ Department of Neurology, Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200025, PR China.
² Department of Neurology, JiaXing First Hospital, JiaXing 314000, PR China.
³ Department of Nuclear Medicine, Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200025, PR China.
⁴ Department of Neurology, Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200025, PR China. Electronic address: mztcs@163.com.

PMID: 31775073
DOI: 10.1016/j.jneuroim.2019.577114

Abstract

Anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis is a rare type of autoimmune encephalitis. We present a case of a 72-year-old male with anti-DPPX encephalitis who developed rapidly progressive cognitive decline, psychiatric and sleep problems, severe abdominal pain and diarrhea. Antibodies against DPPX were positive both in serum and cerebrospinal fluid. ¹⁸F-FDG PET-MR imaging indicated hypometabolism in the bilateral temporal lobes and thalamus. No related tumors were found, and the patient responded to immunotherapy without relapse at the 3-year follow-up. The present case enriches the understanding of the clinical, imaging manifestations and prognosis of anti-DPPX encephalitis.

Keywords: Autoimmune encephalitis; DPPX antibody; Neuroimmunology.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Aged
Autoantibodies / blood*
Dementia / blood*
Dementia / complications
Dementia / diagnostic imaging
Dipeptidyl-Peptidases and Tripeptidyl-Peptidases / blood*
Disease Progression*
Encephalitis / blood*
Encephalitis / complications
Encephalitis / diagnostic imaging
Humans
Male
Nerve Tissue Proteins / blood*
Potassium Channels / blood*
Remission Induction
Sleep Initiation and Maintenance Disorders / blood*
Sleep Initiation and Maintenance Disorders / complications
Sleep Initiation and Maintenance Disorders / diagnostic imaging

Substances

Autoantibodies
Nerve Tissue Proteins
Potassium Channels
DPP6 protein, human
Dipeptidyl-Peptidases and Tripeptidyl-Peptidases