Detection of anti-glutamate receptor ε2 and anti-N-methyl-d-aspartate receptor antibodies in a patient with sporadic Creutzfeldt–Jakob disease
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Creutzfeldt–Jakob disease (CJD) is the most common type of human prion disease. CJD, particularly sporadic CJD (sCJD), is not completely related to immunological responses. However, immune abnormalities such as elevated pro- and anti-inflammatory cytokines have been reported in patients with sCJD [1, 2], warranting investigation of the immunological aspects of this disease. Here we present an sCJD case with autoantibodies against the N-methyl-d-aspartate (NMDA)-type glutamate receptor.
A 70-year-old right-handed woman developed a hand tremor, right-predominant rigidity in the upper limbs, and Myerson’s sign. Two months later, she developed left hemineglect. Her memory, insight, and judgment were normal at that time, but dressing apraxia, acalculia, and Balint’s syndrome were observed. She developed idiopathic hyponatremia, but her cognitive impairment progressed even after treatment of the hyponatremia. She underwent tracheostomy (without mechanical ventilation) 6 months...
KeywordsGlial Fibrillary Acidic Protein Prion Disease Bovine Spongiform Encephalopathy Limbic Encephalitis sCJD Patient
We thank Tetsuyuki Kitamoto, Tohoku University School of Medicine, for the prion protein gene analysis; Katsuya Satoh, Nagasaki University, for evaluating the 14-3-3 protein, total tau protein, and RT-QUIC; Osamu Watanabe, Kagoshima University, for the anti-voltage-gated potassium channel complex antibody assay. This work was supported by Grants-in-Aid from the Research Committee of Prion Disease and Slow Virus Infection, the Ministry of Health, Labour, and Welfare of Japan, and Health and Labour Sciences Research Grants for Research on Psychiatry and Neurological Diseases and Mental Health (H20-021).
Conflict of interest
The authors declare that they have no conflict of interest.
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