Related Experiment Video
Updated: Apr 28, 2026
09:42
A Chronic Autoimmune Dry Eye Rat Model with Increase in Effector Memory T Cells in Eyeball Tissue
Published on: June 7, 2017
11.5K
EVOLUTION OF AUTOIMMUNE RETINOPATHY IN STIFF PERSON SYNDROME: A CASE REPORT
Grace E Nipp1,2, Richmond Woodward1, Andrew Gross1
1Department of Ophthalmology, Duke University, Durham, North Carolina.
Retinal Cases & Brief Reports
|April 3, 2024
Summary
Autoimmune retinopathy preceded stiff person syndrome (SPS) by five years in a patient with vision loss. Early diagnosis of SPS is crucial even without systemic symptoms, especially when ruling out paraneoplastic causes.
Area of Science:
- Neuro-ophthalmology
- Autoimmune diseases
Background:
- Autoimmune retinopathy (AIR) is a rare autoimmune disorder affecting the retina.
- Stiff person syndrome (SPS) is a rare neurological disorder characterized by muscle rigidity and spasms.
Purpose of the Study:
- To report a case of autoimmune retinopathy as the initial manifestation of stiff person syndrome.
- To analyze the clinical evolution of this association.
Main Methods:
- Case report detailing a 35-year-old male patient.
- Ophthalmic examinations including visual acuity, Humphrey visual field, optical coherence tomography (OCT), and electroretinography (ERG).
- Serum testing for autoantibodies and systemic workup for malignancy.
Main Results:
- The patient presented with progressive vision loss and maculopathy years before developing muscle spasms.
- High-titer anti-glutamic acid decarboxylase 65-kDa isoform (GAD65) antibodies confirmed AIR and SPS.
- OCT showed progressive ellipsoid zone loss, and ERG revealed severe bilateral maculopathy.
Conclusions:
- Autoimmune retinopathy can precede the onset of stiff person syndrome by several years.
- SPS should be considered in unexplained retinopathy cases, particularly after ruling out paraneoplastic syndromes.
- Treatment with IV immunoglobulin and rituximab improved systemic symptoms.
