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  4. Anti‑ganglioside antibody positive neuromyelitis optica spectrum disorders with peripheral neuropathy: a case report

Anti‑ganglioside antibody positive neuromyelitis optica spectrum disorders with peripheral neuropathy: a case report

BMC Neurology, 2023 · DOI: https://doi.org/10.1186/s12883-023-03214-6 · Published: May 26, 2023

ImmunologyNeurology

Simple Explanation

Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune disorders affecting the central nervous system, particularly the optic nerve and spinal cord. This report describes a patient with NMOSD who also had peripheral nerve damage, undifferentiated connective tissue disease and tested positive for multiple anti-ganglioside antibodies. The patient improved after treatment with methylprednisolone, gamma globulin, plasma exchange, and rituximab.

Study Duration
Not specified
Participants
One 57-year-old female patient
Evidence Level
Case Report

Key Findings

  • 1
    The patient met the diagnostic criteria for AQP4-IgG positive NMOSD with undifferentiated connective tissue disease and multiple peripheral neuropathy.
  • 2
    The patient was positive for multiple anti-ganglioside antibodies (anti-GD1a IgG antibodies and anti-GD3 IgM antibodies) and anti-sulfatide IgG antibodies in serum and cerebrospinal fluid.
  • 3
    After treatment with methylprednisolone, gamma globulin, plasma exchange, and rituximab, the patient's condition improved.

Research Summary

This case report describes a 57-year-old female patient with AQP4-IgG positive NMOSD, undifferentiated connective tissue disease, and multiple peripheral neuropathy. The patient was also positive for multiple anti-ganglioside antibodies and anti-sulfatide IgG antibodies in serum and cerebrospinal fluid. The patient's condition improved after treatment with methylprednisolone, gamma globulin, plasma exchange, and rituximab.

Practical Implications

Clinical Awareness

Neurologists should be aware of the unusual association between NMOSD and immune-mediated peripheral neuropathy.

Pathophysiology Insights

Multiple antibody-mediated nerve damage may contribute to peripheral nerve damage in NMOSD patients.

Treatment Strategies

IVMP combined with IVIg or PLEX may be a better treatment option for NMOSD patients with peripheral neuropathy.

Study Limitations

  • 1
    Single case report limits generalizability.
  • 2
    Pathogenesis of peripheral neuropathy in NMOSD remains unclear.
  • 3
    Underlying mechanisms of antibody involvement require further investigation.

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