Double-negative NMOSD: from case report to a proposed diagnostic and therapeutic algorithm.
Giuseppe Romano1, Giacomo Lus1, Mario Cirillo2
1Second Division of Neurology, University of Campania "Luigi Vanvitelli", Naples, Italy.
BMC Neurology
|November 12, 2025
Summary
Double seronegative neuromyelitis optica spectrum disorder (DN-NMOSD) is a rare CNS autoimmune disease. Anti-IL-6 therapy showed promise for DN-NMOSD when anti-CD20 treatments failed, suggesting a potential therapeutic strategy.
Area of Science:
- Neurology
- Immunology
- Autoimmune Diseases
Background:
- Double seronegative neuromyelitis optica spectrum disorder (DN-NMOSD) is a rare autoimmune central nervous system disease.
- It is characterized by optic nerve and spinal cord involvement and negativity for anti-Aquaporin-4 (AQP4) and anti-Myelin Oligodendrocyte Glycoprotein (MOG) antibodies.
Purpose of the Study:
- To report a case of DN-NMOSD in a 35-year-old male with optic neuritis and myelitis.
- To evaluate the efficacy of Satralizumab (anti-IL-6 receptor) after failure of Rituximab (anti-CD20).
Main Methods:
- Diagnosis of DN-NMOSD based on clinical presentation and serological testing.
- Treatment initiated with Rituximab, followed by Satralizumab due to disease relapse.
- Clinical and radiological outcomes were monitored.
Main Results:
- The patient experienced temporary stability with Rituximab but relapsed.
- Switching to Satralizumab resulted in two years of clinical and radiological stability.
- This suggests Satralizumab's effectiveness in managing refractory DN-NMOSD.
Conclusions:
- Anti-IL-6 drugs may be a viable therapeutic option for DN-NMOSD, particularly when anti-CD20 therapies are ineffective.
- The case highlights the heterogeneity of DN-NMOSD and the need for better biomarkers (e.g., GFAP, tau, IL-6).
- A stepwise treatment approach, considering anti-IL-6 agents after anti-CD20 failure, could optimize patient outcomes.
Related Concept Videos
01:20Myasthenia Gravis: Overview and Treatment
3.7K
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
3.7K
01:27Open Angle Glaucoma: Treatment
1.1K
In open-angle glaucoma, the iridocorneal angle remains open, but the trabecular meshwork becomes stiff, slowing down the outflow of aqueous humor. This causes a buildup of aqueous humor in the anterior chamber, leading to a sudden increase in intraocular pressure. The treatment for open-angle glaucoma focuses on reducing the elevated intraocular pressure by either decreasing the secretion of aqueous humor or increasing its outflow.
Drugs such as carbonic anhydrase inhibitors, α2- and...
Drugs such as carbonic anhydrase inhibitors, α2- and...
1.1K
01:28Angle Closure Glaucoma: Treatment
1.7K
Angle-closure glaucoma, or closed-angle glaucoma, is an eye condition where the iris bulges out and blocks the iridocorneal angle, resulting in a buildup of aqueous humor and increased intraocular pressure. Immediate medical attention is necessary due to the sudden onset of symptoms. The treatment for angle-closure glaucoma includes short-term and long-term approaches. Short-term treatment involves using eye drops like pilocarpine to lower intraocular pressure by increasing aqueous humor...
1.7K
01:19Multiple Sclerosis l: Introduction
2
Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
2