Abstract Details

Title Neurologic Manifestations of Sjogren’s Syndrome: A Case of Central Demyelination and Diffuse Small Fiber Neuropathy

Topic General Neurology

Presentation(s) P4 - Poster Session 4 (8:00 AM-9:00 AM)

Poster/Presentation
Number 7-004

Objective N/A

Background Sjogren’s syndrome is known to involve both peripheral and central nervous systems. Peripheral neuropathy, typically sensory or sensorimotor, is a common extra-glandular manifestation, while central nervous system (CNS) involvement may mimic demyelinating diseases such as multiple sclerosis. This case presents a rare combination of diffuse small fiber sensory neuropathy (SFSN) with concurrent CNS demyelinating lesions.

Design/Methods N/A

Results A 55-year-old male with history of mechanical heart valve replacement on Coumadin, hypertension, and chronic dry mouth requiring multiple dental extractions presented with right-sided facial and body numbness and imbalance immediately following valve surgery in May 2024. Initial symptoms were attributed to a perioperative stroke, but imaging was not obtained. Neurological exam revealed decreased pinprick sensation in the right lower extremity and diminished vibration sense in both feet (right > left). MRI brain in October 2024 showed T2 FLAIR hyperintensities in left paramedian posterior cingulate gyrus and adjacent posterior corpus callosum, with subtle DWI signal and ADC hyperintensity. Follow-up MRI demonstrated interval progression extending into the splenium of the corpus callosum, persistent DWI changes, and no contrast enhancement, raising concern for a subacute or chronic demyelinating process; cervical and thoracic spine MRIs were unremarkable. By May 2025, the patient developed progressive imbalance, whole-body burning sensations, and diaphoresis. Labs showed leukopenia (WBC 2700), positive ANA (1:320 speckled), elevated SSA-52 (121) and SSA-60 (118), increased kappa (54.45) and lambda (36.82) light chains, and negative SS-B, dsDNA, Smith, and scleroderma antibodies. Skin biopsy confirmed moderately severe, length-dependent SFSN. The patient failed multiple neuropathic medications, with some benefit from oxycodone, and is currently being initiated on IVIG.

Conclusions This case underscores the diverse neurological manifestations of Sjogren’s syndrome, including diffuse SFSN and CNS demyelinating lesions. Early recognition is critical, as presentations may mimic other neurologic disorders like multiple sclerosis or ischemia, and appropriate treatment depends on accurate diagnosis.

Authors/Disclosures
Marie Hashitani (University of Nevada Reno School of Medicine) PRESENTER	Ms. Hashitani has nothing to disclose.
Verena C. Samara, MD (Carson Tahoe Medical Group)	An immediate family member of Dr. Samara has received personal compensation for serving as an employee of Supernus. The institution of Dr. Samara has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Samara has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Argenx. The institution of Dr. Samara has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Samara has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Johnson and Johnson. An immediate family member of Dr. Samara has stock in Supernus.
Raja Godasi, MBBS (St Luke's Health System)	Dr. Godasi has nothing to disclose.
Neeharika Thottempudi, MD	Dr. Thottempudi has nothing to disclose.

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