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Abstract Details

Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis: Clinical Presentation and Misdiagnosis
Autoimmune Neurology
S39 - Autoimmune and Paraneoplastic Neurological Disorders: Clinical Characteristics and Diagnosis (2:48 PM-3:00 PM)
010
To describe the spectrum of cases referred to Autoimmune Neurology at Mayo Clinic with suspected steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT).
SREAT, also known as Hashimoto encephalopathy, is defined by subacute onset encephalopathy, with thyroid antibodies, and objective neurological improvement after immunotherapy, in the absence of other neural autoantibodies.
Retrospective review of clinical, laboratory and radiologic findings in patients referred with suspected SREAT from 2006 to 2019.
143 patients were included in the study. 71% were female. Median age was 45 years. 68% had history of thyroid disease. 80% had received immunotherapy. After our evaluation, 38 patients (27%) were diagnosed with autoimmune encephalopathy. 26% of them had neural autoantibodies, including VGKC (n=3), GAD65 (n=3), VGCC (n=3), acetylcholine receptors (n=2), AMPAR (n=1), unclassified (n=1). 105 patients (73%) were given an alternative diagnosis including subjective cognitive complaints (n=26), conversion (n=20), and neurodegenerative (n=16) disorders amongst others. TPO antibody titer was not significantly different between the groups (median 168.5IU/mL). Patients with autoimmune encephalopathy were more likely to present with subacute symptoms (p<0.001), abnormal MRI (p=0.018), abnormal EEG (p=0.039), and inflammatory CSF (p=0.002). Aphasia (p=0.03), ataxia (p=0.022), and stroke-like episodes (p=0.011), were more frequent in these patients. Patients with an alternative diagnosis were more likely to present with depression (p=0.014), anxiety (p=0.004), and chronic pain (p=0.002).  Patients with inflammatory CSF were more likely to respond to a subsequent immunotherapy trial (p=0.011).

A significant number of patients with suspected SREAT had an alternative non-immune mediated disorder. SREAT diagnosis should not be made without objective evidence of encephalopathy, and should only be considered after exclusion of other causes. TPO antibody prevalence is high, and only reflects a predisposition to autoimmunity. It may also be detected in coexistence with other neural antibodies. Documentation of objective abnormalities at baseline and post-treatment comparison is essential to avoid misdiagnosis.
Authors/Disclosures
Cristina Valencia Sanchez, MD (Mayo Clinic Arizona)
PRESENTER 		Dr. Valencia Sanchez has a non-compensated relationship as a member of the medical advisory board with The MOG Project that is relevant to AAN interests or activities.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology) Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic) The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic) The institution of Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Flanagan 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. The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from Viela Bio. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities.
No disclosure on file
Nicholas L. Zalewski, MD (Mayo Clinic) Dr. Zalewski has nothing to disclose.
Anastasia Zekeridou, MD, PhD, FAAN (Neuroimmunology Laboratory, Mayo Clinic) The institution of Dr. Zekeridou has received research support from Roche/Genentech. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care.
Andrew McKeon, MD (Mayo Clinic) The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.