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Abstract Details

MRI hallmarks of LGI1- and CASPR2-antibody encephalitis
Autoimmune Neurology
P7 - Poster Session 7 (8:00 AM-9:00 AM)
6-032

To identify common imaging characteristics in LGI1- and CASPR2-antibody mediated disorders, and how these differ from non-autoimmune disorders such as infectious encephalitis and Creutzfeld-Jakob Disease; CJD.

 

LGI1- and CASPR2-antibody encephalitis can present with acute to subacute onset confusion, amnesia and seizures. Early recognition and treatment help optimise patient outcomes and salient MRI features can aid diagnosis. Two disorders commonly confused with these syndromes are viral encephalitis and CJD. Here, we asked whether patients with LGI1- and CASPR2-Antibody mediated encephalitis showed characteristic findings on MRI brain to help expedite diagnosis and distinguish the illness from these differentials.

This was a retrospective cross-sectional analysis of patients referred to the Oxford Autoimmune Neurology Group. MRI brains from 97 patients were reviewed blinded and independently by two neuroradiologists. Patients included those with antibodies against LGI1 (N=44), CASPR2 (N=17), LGI1 and CASPR2 (N=4),patients with proven viral encephalitis (N=22) and CJD (N=10). Statistical analyses (e.g. Chi-square test) were performed to identify differences between and within groups, and correlate imaging findings with clinical syndromes.

T2 hyperintensity of the mesial temporal lobe was common in LGI1- and CASPR2-Antibody mediated disease (43/65, 66%). Compared to viral encephalitis, this is more likely to be bilateral (24/43; 56% vs 5/18; 28%. P=0.046), and less likely to extend to other structures (8/43; 19% vs 17/18; 94%. P<0.001), or be associated with oedema (12/65; 19% vs 13/22; 59%, P<0.001). Notably, true diffusion restriction was not seen in any antibody-mediated cases (0/60 vs 16/22; 73%. P<0.001) and contrast enhancement was rare (1/22; 5% vs 7/17; 41%. P=0.013).

In the correct clinical context, highly characteristic imaging findings may be used to assist the diagnosis of LGI1- and CASPR2-Ab mediated encephalitis. In particular, the absence of diffusion restriction and contrast enhancement supports these diagnoses and differentiates them from other common differentials.

Authors/Disclosures
Mark J. Kelly, MBBS (Royal College of Surgeons in Ireland)
PRESENTER
The institution of Dr. Kelly has received research support from The Health Research Board / Wellcome Trust.
Eleanor Grant No disclosure on file
Andrew Murchison No disclosure on file
Christopher E. Uy, MD (Vancouver Coastal Health) Dr. Uy has received personal compensation in the range of $500-$4,999 for serving as a IVIG for CNS Disease Task Force Member with Provincial Blood Coordinating Office (British Columbia).
Sophie Binks, MD, MBBS, PhD The institution of Dr. Binks has received research support from Wellcome Trust. The institution of Dr. Binks has received research support from PetSavers. The institution of Dr. Binks has received research support from PetPlan. The institution of Dr. Binks has received research support from NIHR. The institution of Dr. Binks has received research support from Morris Animal Foundation. Dr. Binks has received personal compensation in the range of $0-$499 for serving as a Speaker with ECTRIMS. Dr. Binks has received personal compensation in the range of $0-$499 for serving as a Speaker with Vetmeduni Wien. Dr. Binks has received personal compensation in the range of $0-$499 for serving as a Speaker with ANA. Dr. Binks has a non-compensated relationship as a Speaker with Encephalitis Society UK that is relevant to AAN interests or activities. Dr. Binks has a non-compensated relationship as a Scientific Panel Member with Encephalitis International that is relevant to AAN interests or activities. Dr. Binks has a non-compensated relationship as a Editorial Fellow with JAMA Neurology that is relevant to AAN interests or activities.
No disclosure on file
Fintan Sheerin No disclosure on file
Sarosh R. Irani, MD, PhD, FRCP, FEAN (Mayo Clinic) Dr. Irani has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for AZ, Roche, Cerebral therapeutics, Biogen, Amgen, Argenex, Clarivate, IQVIA, BioHaven therapeutics.. Dr. Irani has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Brain. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care. Dr. Irani has received intellectual property interests from a discovery or technology relating to health care.