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Abstract Details

Describe the Clinical Course, Diagnostic Approach and Outcomes Following Thymectomy in Four Non-myasthenic Patients with Synaptic Antibody-mediated Autoimmune Neurologic Disease
Autoimmune Neurology
P4 - Poster Session 4 (11:45 AM-12:45 PM)
14-001
There is limited data on the therapeutic implications of thymectomy in non-myasthenia  autoimmune neurologic conditions, and prior case reports on autoimmune encephalitis have suggested that the presence of a thymoma can be linked with poor outcomes without surgical intervention.
Describe the clinical course, diagnostic approach and outcomes following thymectomy in four non-myasthenic patients with synaptic antibody-mediated autoimmune neurologic disease.
We queried the University of Utah Autoimmune Neurology Clinic patient database for any patients who underwent thymectomy for a non-myasthenic indication.

We identified four patients between the ages of 22-42, out of which two were female. Three out of the four patients had elevated CASPR2 antibody levels in the serum at time of diagnosis while the remaining patient had positive NMDAR antibodies. One of the CASPR2 antibody positive patients had been diagnosed with AMPA receptor encephalitis two years prior, with continuing episodic disease fluctuations despite immunotherapy. All patients underwent thymectomy due to concern for thymoma based on imaging, with one of them testing positive for ovarian teratoma as well requiring surgical intervention. Two patients were treated with maintenance immunotherapy with IVIG and/or rituximab while the other two only received a short course of steroids followed by surgical intervention. CSF profile and MRI brain were largely normal in all patients. All patients had significant disease stability and clinical improvement following thymectomy, with only one patient needing to continue immunotherapy long term.   

Thymectomy allowed for improvement and stabilization of all patients in our cohort, and cessation of immunotherapy in three out of the four. There are no trials or high-quality data to guide appropriateness of thymectomy for CNS autoimmune disorders, but it is often considered in synaptic-antibody mediated neurologic syndromes—especially when multiple autoantibodies are present that are otherwise requiring prolonged immunotherapy or where long-term immunotherapy is contraindicated or not desired by patients. 

 

Authors/Disclosures
Aditi Sharma, MBBS (University of Utah)
PRESENTER
Dr. Sharma has nothing to disclose.
Tammy L. Smith, MD, PhD (Imaging and Neurosciences Center) The institution of Dr. Smith has received research support from Alexion/AstraZeneca. Dr. Smith has a non-compensated relationship as a participant with Euroimmun Academy, receiving travel support to attend, that is relevant to AAN interests or activities.
Melissa A. Wright, MD (University of Utah) Dr. Wright has nothing to disclose.
Ka-Ho Wong (U of U Neurology Clinic) The institution of Mr. Wong has received research support from The Sumaira Foundation .
M. M. Paz Soldan, MD, PhD (University of Utah) Dr. Paz Soldan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Paz Soldan has received research support from National Institutes of Health. The institution of Dr. Paz Soldan has received research support from National Multiple Sclerosis Society. The institution of Dr. Paz Soldan has received research support from Western Institute for Biomedical Research. The institution of Dr. Paz Soldan has received research support from Biogen. The institution of Dr. Paz Soldan has received research support from Novartis. The institution of Dr. Paz Soldan has received research support from Clene Nanomedicine.
John W. Rose, MD, FAAN (Imaging and Neurosciences Center) The institution of Dr. Rose has received research support from National Multiple Sclerosis Society. The institution of Dr. Rose has received research support from Guthy Jackson Charitable Foundation. The institution of Dr. Rose has received research support from NIH . The institution of Dr. Rose has received research support from VA. The institution of Dr. Rose has received research support from Biogen. The institution of Dr. Rose has received research support from Friends of MS. Dr. Rose has received intellectual property interests from a discovery or technology relating to health care.
Jonathan R. Galli, MD (University of Utah) Dr. Galli has nothing to disclose.
Robert Kadish, MD Dr. Kadish has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. The institution of Dr. Kadish has received research support from Alexion Pharmaceuticals.
Stacey Clardy, MD, PhD, FAAN (University of Utah) Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy 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 Neurology/AAN Publications. The institution of Dr. Clardy has received research support from Sumaira Foundation for NMO. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory.