Abstract Details

Title Severe Lumbar Stenosis Masquerading as Progression in Stiff Person Syndrome

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 14-012

Objective To describe a case of severe lumbar stenosis occurring in the context of a person with stiff person syndrome (SPS) who has concomitant hyperlordosis.

Background SPS is a rare, autoimmune neurological condition that most often presents with intermittent spasms, stiffness, and ambulatory dysfunction. Over time, people with SPS can develop lumbar hyperlordosis which is a hallmark sign on musculoskeletal exam. Other musculoskeletal changes can occur resulting in acute or chronic back pain that can be mistaken as progression of SPS.

Design/Methods Case report.

Results A 63-year-old woman developed insidious onset of stiffness, falls, and incoordination. Her exam demonstrated spasticity in lower extremities, hyperreflexia, and limb dysmetria. Initial MRIs were unremarkable. She continued to worsen and eventually was diagnosed with SPS; testing showed anti-GAD65 antibody at 19580 IU/mL. Upon re-examination, the patient was noted to have moderate hyperlordosis along with her previously noted findings. Muscle relaxers and intravenous immunoglobulin therapy (IVIG) were initiated, and symptoms remained stable until 2010, when patient reported worsening low back pain and imbalance. Due to suspected progression in her SPS, IVIG was stopped and mycophenolate mofetil was started. In 2014, her symptoms again worsened, and she underwent plasma exchange therapy with improvement in symptoms outside of her back pain. The patient also noted increasing lumbar radiculopathy that was further assessed by MRI which demonstrated severe lumbar stenosis secondary to spondylolisthesis. She underwent lumbar spine surgery. Post-operative lumbar hyperlordosis was less severe and her severe back and radicular pain resolved. Serial MRIs will be shown pre-and-post surgery.

Conclusions We present a case in which an SPS patient developed worsening back pain and stiffness with increasing hyperlordosis in the setting of severe lumbar stenosis. Clinicians should be aware of non-SPS related structural changes that can masquerade as progression in SPS especially since surgical intervention may be needed versus escalation in immune-based treatment.

Authors/Disclosures
Ashley Miles (The Johns Hopkins Hospital) PRESENTER	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.

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