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Abstract Details

Long-term Rituximab Use Benefits Patients with Stiff Person Syndrome
Autoimmune Neurology
P2 - Poster Session 2 (5:30 PM-6:30 PM)
15-033

To describe the effects of chronic Rituximab therapy in Stiff Person Syndrome (SPS).

Rituximab is effective for various neuroimmunologic conditions. Multiple reports suggested efficacy in SPS but a recently published clinical trial was negative. This trial had several limitations including small sample size (12 on active arm), lack of sensitive outcome measures, and importantly it was short in duration.

Data was obtained from more than 150 SPS patients followed at the Johns Hopkins Hospital.Demographics, SPS symptoms/signs, pertinent laboratory tests, and timed 25-foot walk (T25FW) were recorded. Data was managed using REDCap and analyzed using Stata15.
Forty-one patients with SPS were exposed to Rituximab in our cohort. Twenty-three had the minimum information required for analysis. The majority were female(78%) and Caucasian(61%). Average age at first Rituximab infusion was 49 years (range;20-76 years). Mean follow up was 24 months (range;3-60 months). Twenty one patients were antiGAD65 antibody positive SPS, 1 seronegative SPS, and 1 antiGAD-associated pure cerebellar ataxia. Seventeen patients (74%) experienced improvement in SPS symptoms and physical findings. Five had no obvious efficacy and one experienced worsening. Eleven patients (68%) reported improvement in back spasms, 9(70%) in leg spams and 11 (64%) in axial rigidity. Examination showed resolution of axial rigidity in 4 patients (22%), paraspinal muscle spasm in 6 patients (27%) while 6 (25%) patients had an objective improvement in gait. Sufficient T25FW data was available for 12 patients. Mean T25FW at baseline was 9.7 seconds (SD:±7.3) which decreased in last visit to 7.4 seconds (± 4.2). Five patients (42%) had significant improvement in walking speed defined as >20%change from baseline.

Long-term Rituximab therapy appears to benefit many patients with SPS. This observed improvement may be related to frequency and/or consistency of treatment. Further studies are needed to better understand what factors are associated with Rituximab treatment responders versus non-responders
Authors/Disclosures
Salman Aljarallah, MD
PRESENTER 		Dr. Aljarallah has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Aljarallah has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Aljarallah has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Sanofi Genzyme.
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.
Thomas J. Shoemaker, MD (Rush University Medical Center) Dr. Shoemaker has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen. Dr. Shoemaker has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for AMGEN. Dr. Shoemaker has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for EMD Serono.
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.