Detection of anti-glutamate receptor ε2 and anti-N-methyl-d-aspartate receptor antibodies in a patient with sporadic Creutzfeldt–Jakob disease

Anti-N-methyl-D-Aspartate Receptor Encephalitis Mimicking Sporadic Creutzfeldt–Jakob Disease

“…Mackay et al found that two definite sCJD cases had low levels of serum anti-NMDAR antibodies ( 3 ). In CSF, antibodies against NMDAR with low titers were detected in an sCJD patient, and the diagnosis was validated by positive real-time quaking-induced conversion (RT-QUIC) results ( 8 ). Grau-Rivera et al found that anti-NMDAR antibodies were detected in CSF of 1/346 (0.29%) patients with rapid neurologic deterioration suggestive of CJD, but this patient did not fulfill the diagnostic criteria for probable or possible CJD, whereas none of the 49 patients with definite CJD had anti-NMDAR antibodies in CSF ( 7 ).…”

Antibodies to N-methyl-D-aspartate glutamate receptors in Creutzfeldt–Jakob disease patients

“…We have hypothesized that autoantibodies are also produced and potentially contribute to neuropsychiatric symptoms in CJD. In fact, we reported a sporadic CJD patient with antibodies against the GluN2B molecule and native N-methyl-D-aspartate glutamate receptor (NMDAR) (Fujita et al, 2012a). Here, we explored further the association of CJD and antibodies recognizing NMDARs.…”

“Antibody of Unknown Significance” (AUS): The Issue of Interpreting Antibody Test Results

“…10 Neuronal autoantibodies have also been found when the final neurological diagnosis was not autoimmune. For example, GlyR antibodies were detected in Creutzfeldt-Jakob disease or genetic dystonia 11,12 ; NMDAR antibodies in patients with Creutzfeld-Jakob disease 13,14 or Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) 15 ; and GABA A R antibodies in genetically proven Huntington's disease. 16 The above examples are mostly because of methodological shortcomings (eg, type of tests used, lack of CSF testing, etc.…”