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Abstract Details

Immunotherapy in Stiff Person Syndrome, is Timing Really Important?
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
068
To describe the use of immunotherapies in a large cohort of patients with stiff person syndrome (SPS) and investigate whether an early vs. late treatment approach differentially affect outcomes in SPS.

Conventional treatment in SPS has focused on using an escalation treatment paradigm, starting with symptomatic therapies followed by immunotherapies if there is suspected disease progression. Data is lacking as to whether starting immunotherapies earlier in the SPS disease course is warranted in all patients and whether this is associated with better outcomes over time.
Retrospective review of medical records from 211 SPS patients seen at Johns Hopkins. Demographics, clinical profiles, pertinent laboratory tests and modified Rankin Scale (mRS) were assessed and patients with longer than 18 months follow-up were included. A cut off of 60 months was used to assess early(<60 months;Group A) vs. late immune treatment group(≥60 months;Group B).  The Mann–Whitney U test was used to evaluate difference in mRS between groups.
One-hundred and thirty-two SPS patients received some type of immunotherapy in our cohort. Most patients were Caucasian females and antiGAD65 antibody positive. Majority of patients received intravenous immunoglobulin(96.9%) as first-line immunotherapy agent. Second-line agent was used in 108(81.8%) patients; most commonly rituximab(37.1%) and/or plasmapheresis(33.3%). Complete data/follow-up was available in 89 patients; median follow-up of 44 months. Immune treatment was started early in 53(59.5%) patients and late in 36(40.5%).  There was a trend towards lower mRS at last visit in the early treatment group (Group A;median 3[IQR 2-3]) when compared to late treatment group (Group B;median 3[IQR 3-4])(p=0.0078). Subgroup analyses will be presented.

In this large cohort of SPS patients followed longitudinally, there is a hint of evidence by change in mRS that may support initiating immunotherapies earlier in the course of SPS. However, further analyses are needed.
Authors/Disclosures
Maria I. Reyes-Mantilla, MD (Johns Hopkins University, Neurology)
PRESENTER 		Dr. Reyes-Mantilla has nothing to disclose.
Yujie Wang, MD (UW Northwest) Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Wang has received research support from Genentech. The institution of Dr. Wang has received research support from uniQure. The institution of Dr. Wang has received research support from NIH/NINDS.
Grigorios Kalaitzidis, MD (Boston University Medical Center) Mr. Kalaitzidis has nothing to disclose.
Daniela A. Pimentel Maldonado, MD, MSCR (U.S. Food and Drug Administration) Dr. Pimentel Maldonado has nothing to disclose.
Jacqueline Koshorek, DO (HonorHealth Neurology) Dr. Koshorek has nothing to disclose.
No disclosure on file
Scott D. Newsome, DO, FAAN (Johns Hopkins Hospital) Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. The institution of Dr. Newsome has received research support from Biogen. The institution of Dr. Newsome has received research support from Genentech/Roche. The institution of Dr. Newsome has received research support from Department of Defense. The institution of Dr. Newsome has received research support from Patient Centered Outcomes Research Institute. The institution of Dr. Newsome has received research support from National MS Society. The institution of Dr. Newsome has received research support from Lundbeck. The institution of Dr. Newsome has received research support from Sanofi. The institution of Dr. Newsome has received research support from Kyverna Therapeutics. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving as a Lead PI for Clinical Trial with Roche.