Gerstmann-Sträussler-Scheinker disease mimicking primary progressive multiple sclerosis: A case with positive
Miwa Ito1, Hiroki Masuda1, Marie Morooka2
1Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Background:
Certain prion diseases have been observed to exhibit positive oligoclonal bands (OCBs), a key marker in diagnosing multiple sclerosis (MS).
Methods:
We describe a case of Gerstmann-Sträussler-Scheinker (GSS) disease presenting with positive OCBs, no sustained abnormalities in the cortex or basal ganglia, and limited brain atrophy progression, mimicking the clinical profile of primary progressive MS (PPMS).
Conclusion:
Atypical white matter findings and warning signs should prompt caution in diagnosing MS. GSS should be recognized as a potential differential diagnosis for PPMS.
Insights
Gerstmann-Sträussler-Scheinker (GSS) disease can mimic multiple sclerosis (MS) by presenting with positive oligoclonal bands (OCBs). Clinicians should consider GSS as a differential diagnosis for primary progressive MS (PPMS) due to atypical findings.
Area of Science:
- Neurology
- Neuroimmunology
- Prion Diseases
Background:
- Prion diseases can present with positive oligoclonal bands (OCBs), a marker typically associated with multiple sclerosis (MS).
- This overlap can complicate differential diagnoses, particularly in cases mimicking MS phenotypes.


