Abstract Details

Title Therapeutic Plasma Exchange in the Management of Stiff Person Syndrome Spectrum Disorders: A Case Series and Review of the Literature

Topic Autoimmune Neurology

Presentation(s) S25 - Autoimmune Neurology 2: Clinical Trials and Treatment (4:54 PM-5:06 PM)

Poster/Presentation
Number 008

Background Stiff person syndrome spectrum disorders (SPSD) are a rare group of disabling neuroimmunological disorders. SPSD often require immune therapies especially in the setting of inadequate response to symptomatic treatments. The safety and efficacy of therapeutic plasma exchange (TPE) in SPSD is unclear.

Objective To describe the safety, tolerability, and response to TPE in patients with SPSD.

Design/Methods A retrospective review of medical records for patients with SPSD seen at Johns Hopkins Hospital from 1997 to 2021 was performed. Patient characteristics, exam findings, diagnostic studies, treatment response and TPE-related complications were recorded. Literature review of SPSD and TPE was also conducted.

Results

Thirty-six SPSD patients were treated with TPE; mean age was 48 years, 81% female, median modified Rankin Scale 3, and average anti-GAD65 antibody titer was 42352 U/mL (range, 0-309,902). Twenty-two patients had classic SPS, 10 had SPS-plus, 2 had progressive encephalomyelitis with rigidity and myoclonus, 1 had pure cerebellar ataxia, and 1 had stiff limb syndrome. Thirty-three patients were treated for acute exacerbations, and 3 were on maintenance TPE. There were 4 (11.1%) TPE-related complications (catheter infection, catheter thrombosis, hemorrhage), but no deaths or anaphylaxis. Twenty patients (55.6%) reported improvement in symptoms after TPE, 13 reported no change, and 3 reported worsening of symptoms. Of the 36 total patients who received TPE, 32 received TPE at Johns Hopkins Hospital for an acute exacerbation of their condition, with 16 improving after TPE in functional status allowing for discharge. Literature review identified 51 more patients; 63% of these patients reported an improvement in their condition.

Conclusions

We describe the safety and tolerability of TPE in patients with SPSD and show that TPE-related complications are uncommon and manageable. Additionally, many patients with SPSD derived improvement with TPE. Further studies could help inform clinicians when to use TPE in SPSD.

Authors/Disclosures
Shuvro Roy, MD (University of Washington) PRESENTER	Dr. Roy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon.
	No disclosure on file
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.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Scott D. Newsome, DO, FAAN (Johns Hopkins Hospital)	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 Biogen. 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 Bristol Myers Squibb. 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 The Stiff Person Syndrome Research Foundation. The institution of Dr. Newsome has received research support from Lundbeck. The institution of Dr. Newsome has received research support from Sanofi. 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.