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Relapsing CNS involvement in MGUS-an Occam's razor?
Naveed Iqbal1, Navraj Chattha2, Alisha Musthafa2
1Stroke Medicine, Maidstone Hospital, Maidstone, England, UK naveed.iqbal12@nhs.net.
Abstract:
Monoclonal gammopathy of undetermined significance (MGUS) is traditionally considered a benign precursor plasma cell disorder with minimal risk of progression to multiple myeloma or lymphoproliferative malignancies. However, emerging evidence suggests that MGUS may, in select cases, be associated with clinically significant organ dysfunction. Central nervous system (CNS) involvement remains exceedingly rare.We report a woman in her 70s who presented with subacute visual disturbances. Over 2 years, she developed relapsing, posterior-predominant CNS inflammatory lesions affecting the occipital, temporal and parietal lobes, later extending to involve the pontine and cerebellar regions. Isoelectric focusing and immunofixation of cerebrospinal fluid and serum revealed identical paraprotein bands, implicating a possible pathogenic link between MGUS and CNS pathology.Extensive evaluations did not reveal any alternative explanation for her symptoms, including conditions such as multiple sclerosis, CNS lymphoma, vasculitis or paraneoplastic syndromes. Despite multiple courses of intravenous and oral corticosteroids, she experienced relapses with only moderate clinical improvement.
Insights
Monoclonal gammopathy of undetermined significance (MGUS) can rarely cause central nervous system (CNS) inflammation. This case highlights a potential link between MGUS and rare CNS inflammatory lesions.
Area of Science:
- Neurology
- Hematology
- Immunology
Background:
- Monoclonal gammopathy of undetermined significance (MGUS) is typically a benign plasma cell disorder.
- Emerging evidence suggests potential links between MGUS and organ dysfunction.
- Central nervous system (CNS) involvement in MGUS is exceptionally rare.
Purpose of the Study:
- To report a rare case of CNS inflammatory lesions associated with MGUS.
- To investigate the potential pathogenic link between MGUS and CNS pathology.
Main Methods:
- Case report of a woman in her 70s with visual disturbances.
- Longitudinal monitoring of neurological symptoms and CNS imaging over 2 years.
- Analysis of serum and cerebrospinal fluid using isoelectric focusing and immunofixation.
Main Results:
- The patient developed relapsing, posterior-predominant CNS inflammatory lesions.
- Identical paraprotein bands were found in serum and cerebrospinal fluid.
- No alternative diagnoses like multiple sclerosis or CNS lymphoma were identified.
Conclusions:
- This case suggests a possible pathogenic association between MGUS and CNS inflammatory disease.
- MGUS should be considered in the differential diagnosis of unexplained CNS inflammatory lesions.
- Further research is needed to understand the mechanisms and implications of CNS involvement in MGUS.
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