A case of myelin oligodendrocyte glycoprotein antibody-associated disease presenting with radiculopathies
Sunao Takahashi1, Takashi Irioka1, Ryo Iwase1,2
1Department of Neurology, Yokosuka Kyosai Hospital.
Abstract:
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a demyelinating disease of the central nervous system (CNS). Recent reports suggest the peripheral nervous system (PNS) could also be involved, although the pathophysiology remains unclear. We report a 50-year-old woman who presented with acute cauda equina syndrome as the initial neurological manifestation of MOGAD. MOG antibodies were detected only in the cerebrospinal fluid (CSF). MRI showed a peripherally located small intramedullary lesion in the ventral spinal cord at the twelfth thoracic level. Leptomeningeal lesions were also present over the epiconus and conus. Despite treatment with corticosteroids and azathioprine, she experienced four relapses over years. During the fourth relapse, she developed left trigeminal neuralgia, and the MRI showed a small lesion at the entry zone of the trigeminal nerve. This case illustrates that leptomeningeal lesions can cause peripheral neuropathies in MOGAD in the absence of overt CNS signs. Testing for MOG antibodies in the CSF should be considered when there is a strong clinical suspicion of MOGAD or combined CNS and PNS involvement, even if serum results are negative.
Insights
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can affect the peripheral nervous system, causing conditions like cauda equina syndrome. Cerebrospinal fluid MOG antibody testing is crucial for diagnosis, even with negative serum results.
Area of Science:
- Neurology
- Immunology
- Demyelinating Diseases
Background:
- Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is primarily known as a central nervous system (CNS) demyelinating disorder.
- Emerging evidence suggests potential involvement of the peripheral nervous system (PNS) in MOGAD, but the underlying mechanisms are not fully understood.
Purpose of the Study:
- To report a case of MOGAD presenting with peripheral nervous system involvement.
- To highlight the diagnostic significance of cerebrospinal fluid (CSF) MOG antibody testing in suspected MOGAD cases with combined CNS and PNS manifestations.
Main Methods:
- Case report of a 50-year-old woman with MOGAD.
- Analysis of neurological presentation, MOG antibody levels in CSF and serum, and MRI findings.
- Documentation of disease course, treatment response, and relapses over several years.
Main Results:
- The patient presented with acute cauda equina syndrome, a PNS manifestation, with MOG antibodies detected solely in the CSF.
- MRI revealed spinal cord and leptomeningeal lesions, and later a lesion at the trigeminal nerve entry zone during relapse.
- The patient experienced multiple relapses and developed trigeminal neuralgia, indicating recurrent PNS involvement.
Conclusions:
- Leptomeningeal lesions in MOGAD can lead to peripheral neuropathies even without apparent CNS signs.
- CSF MOG antibody testing is recommended for suspected MOGAD, especially with combined CNS/PNS involvement, regardless of serum antibody status.
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