Rossi, M;
Mead, S;
Collinge, J;
Rudge, P;
Vincent, A;
(2015)
Neuronal antibodies in patients with suspected or confirmed sporadic Creutzfeldt-Jakob Disease.
Journal of Neurology, Neurosurgery, and Psychiatry
, 86
(6)
pp. 692-694.
10.1136/jnnp-2014-308695.
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Abstract
Objectives There have been reports of patients with antibodies to neuronal antigens misdiagnosed as sCJD. Conversely, low levels of antibodies to neuronal proteins have been reported in patients with Creutzfeldt-Jakob disease (sCJD). However, the frequency of misdiagnoses, or of antibodies in patients with subsequently confirmed sCJD, is not clear. Methods We reviewed 256 consecutive cases of sCJD seen in the National Prion Clinic, of whom 150 had sera previously referred for selected antibody tests. 82 available samples were retested for antibodies to NMDAR, the glycine receptor (GlyR), VGKC-complex, and the associated proteins, leucine-rich glioma inactivated 1 (LGI1), and contactin associated protein 2 (CASPR2). Results Four of the initial 150 sera referred were positive; two had antibodies to NMDAR, and two to the VGKC-complex, one of which was also positive for GlyR antibodies. Of the 82 sCJD sera retested, one had VGKC-complex antibodies confirming the previous result, two had CASPR2 and GlyR antibodies, and one had CASPR2 and NMDAR antibodies; all antibodies were at low levels. Over the same period three patients with autoimmune encephalitis and high VGKC-complex antibodies were initially referred as sCJD. Conclusions This study indicates that <5% sCJD patients develop serum antibodies to these neuronal antigens and, when positive, only at low titres. By contrast, three patients referred with possible prion disease had a clinical picture in keeping with autoimmune encephalitis and very high VGKC-complex/LGI1 antibodies. Low titres of neuronal antibodies occur only rarely in suspected sCJD patients and when present should be interpreted with caution.
| Type: | Article |
|---|---|
| Title: | Neuronal antibodies in patients with suspected or confirmed sporadic Creutzfeldt-Jakob Disease |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1136/jnnp-2014-308695 |
| Publisher version: | http://dx.doi.org/10.1136/jnnp-2014-308695 |
| Language: | English |
| Additional information: | This is an Open Access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) license, which permits others to distribute, remix, adapt and build upon this work, for commercial use, provided the original work is properly cited. See: http://creativecommons.org/licenses/by/4.0/ |
| Keywords: | Prion, NMDAR, VGKC-complex, glycine receptor, autoantibody |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases > MRC Prion Unit at UCL |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/1447058 |
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