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Abstract Details

Analysis of Antineuronal Antibodies in Patients with Suspected and Pathologically Proven Creutzfeldt-Jakob Disease
Aging and Dementia
P04 - (-)
218
BACKGROUND: Previous studies have described the clinical overlap between CJD and immune-mediated encephalitis, and NMDA-R and VGKC-complex antibodies in serum of patients with definite CJD have been recently described.
DESIGN/METHODS: ANA-ab were analyzed by immunohistochemistry on frozen sections of rat brain fixed with paraformaldehyde in two groups of CSF samples: 243 sent during 2012 to our laboratory for 14-3-3 testing and 48 pathologically proven CJD cases. Positive cases in the first group were further studied for antigen specificity and all 48 CSF samples of CJD patients were also tested for the presence of NMDAR-ab using a highly specific NR1/2 cell-based assay.
RESULTS: ANA-ab were detected in 6 (2.4%) of the samples. The target antigens corresponded to CASPR2, GAD, NMDA-R, NMO, Tr (DNER) and one non-identified antigen. Five patients presented with rapidly progressive cognitive decline, and the patient with Tr-ab had a subacute cerebellar syndrome. Only the CSF with Tr-ab was also 14-3-3 positive. MRI features suggesting limbic encephalitis were observed in one patient and CSF pleocytosis in two. A small cell lung cancer was diagnosed in the patient with the unidentified antibody. All patients improved (three received immunotherapy, two symptomatic treatment, and one tumor therapy). The CSF of 3/48 CJD patients showed atypical reactivity with rat brain; none of the 48 cases had NMDAR or VGKC-complex antibodies.
CONCLUSIONS: A low but clinically relevant number of patients with suspected CJD have other potentially treatable disorders in association with ANA-ab. This finding reinforces the importance of including ANA-ab analysis in the diagnostic work-up of patients with suspected CJD. Patients with definite CJD do not have VGKC-complex or NMDAR-ab; our findings stress the need to examine CSF to avoid false positive antibody testing.
Authors/Disclosures

PRESENTER 		No disclosure on file
Raquel Sanchez Del Valle Diaz, MD No disclosure on file
Andrea L. Gropman, MD, FAAN (St Jude Children's Research Hospital) Dr. Gropman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Baylor Mirica. Dr. Gropman has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Immedica. Dr. Gropman has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for various legal firms. The institution of Dr. Gropman has received research support from National Institutes of Health.
Stanley L. Cohan, MD, PhD, FAAN Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AbbVie. Dr. Cohan has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Cohan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche Genentech. Dr. Cohan has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bristol Myers Squibb (Celgene). Dr. Cohan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Icometrix. Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for EMD Serono. Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for sanofi genzyme. Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Novartis. Dr. Cohan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Roche Genentech. Dr. Cohan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Bristol Myers Squibb. Dr. Cohan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for EMD Serono. Dr. Cohan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Icometrix.
Albert Saiz (Hospital Clinico De Barcelona) Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janseen. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for novartis. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.
Jose L. Molinuevo Guix, MD No disclosure on file
Josep O. Dalmau, MD, PhD, FAAN Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas Research Institute of America. Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Research & Development . Dr. Dalmau has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for 好色先生. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Dalmau has received research support from Sage Therapeutics. The institution of Dr. Dalmau has received research support from Edmond J.Safra Foundation . The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Euroimmun, Inc. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care.
Francesc R. Graus, MD (IDIBAPS) Dr. Graus has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for MedLink. Dr. Graus has received intellectual property interests from a discovery or technology relating to health care.