Schnitzler's syndrome associated with sensorimotor neuropathy

Area of Science:

• Immunology
• Neurology
• Dermatology

Background:

• Schnitzler's syndrome is a rare autoinflammatory disorder characterized by the triad of chronic urticaria, monoclonal IgM gammopathy, and a bone disease (though not always present).
• The association between monoclonal gammopathies and neurological complications is increasingly recognized, but specific patterns in Schnitzler's syndrome are less defined.

Observation:

• A case study detailing a patient with established Schnitzler's syndrome who developed sensorimotor neuropathy seven years after the initial onset of chronic urticaria.
• Detection of anti-myelin-associated glycoprotein (MAG) antibodies in the patient's serum, suggesting an immune-mediated mechanism for the neuropathy.

Findings:

• High-dose corticosteroids provided temporary relief for the cutaneous symptoms of urticaria but did not halt the progression of the sensorimotor neuropathy.
• Intravenous immunoglobulin therapy administered for six months proved ineffective in treating the neurological deficit.

Implications:

• This case highlights the potential for delayed neurological complications in patients with Schnitzler's syndrome, specifically anti-MAG neuropathy.
• The findings underscore the limited efficacy of current standard treatments like corticosteroids and immunoglobulins for established neuropathy in this context.
• Further research is warranted to explore alternative therapeutic strategies for managing neurological involvement in Schnitzler's syndrome.