Glucocorticoid-dependent multiple sclerosis overlapping anti-NMDA receptor encephalitis: a case report and literature
1Department of Center for Psychosomatic Medicine, Sichuan Provincial Center for Mental Health, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.
Background:
Previous studies suggest a relationship between central nervous system inflammatory demyelinating diseases and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. Also, the overlap between anti-NMDAR encephalitis and multiple sclerosis (MS) has been reported. However, the pathogenesis and clinical characteristics are still obscure.
Case Presentation:
A 33-year-old woman presented with diplopia and sensory ataxia at the onset. The cerebrospinal fluid (CSF) anti-NMDAR antibodies were positive (1:3.2), and nuclear magnetic resonance imaging (MRI) showed bilateral centrum ovale and lateral ventricle demyelinating lesions. Therefore, she was diagnosed with anti-NMDAR encephalitis. After administering intravenous immunoglobulin and oral prednisone, her lesions disappeared, and symptoms were relieved. The condition was maintained with a low dose of prednisone, but her lesions reappeared on MRI. Consequently, immunomodulatory therapy of mycophenolate mofetil was initiated. However, she developed dysarthria and right limb ataxia after 10 months with a positive CSF anti-NMDAR antibody (1:1) and positive oligoclonal band. The MRI showed symmetrical multiple demyelinating lesions. Considering the MS diagnosis, her neurological dysfunction again improved significantly after intravenous methylprednisolone. Unfortunately, her symptoms aggravated for the second time when teriflunomide was started. Finally, her condition was controlled again with oral prednisone.
Conclusions:
Consistent with previous cases of overlapping anti-NMDAR encephalitis and MS, patients often show atypical symptoms on MRIs and immunological tests. The overlap cannot be arbitrarily treated because of the recurrence of previous diseases. Long-term follow-up, dynamic antibody monitoring, and MRI examination are crucial for these patients. The special dependency of the patient on glucocorticoids in this study has been rarely reported, which may guide the treatment of insensitivity to disease-modifying therapy in recurrent overlapping anti-NMDAR encephalitis and MS.
Insights
This case highlights the complex overlap between anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis and multiple sclerosis (MS). Recurrent symptoms and atypical MRI findings underscore the need for long-term monitoring and individualized treatment strategies.
Area of Science:
- Neuroimmunology
- Neurology
- Demyelinating Diseases
Background:
- Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis and multiple sclerosis (MS) share overlapping features.
- The pathogenesis and clinical characteristics of this overlap remain incompletely understood.
Observation:
- A 33-year-old woman presented with diplopia and sensory ataxia, diagnosed with anti-NMDAR encephalitis based on positive CSF antibodies and demyelinating lesions on MRI.
- Despite initial treatment with immunotherapy, she experienced relapses with recurrent demyelinating lesions, positive oligoclonal bands, and symptoms suggestive of MS.
- The patient demonstrated a significant, albeit temporary, response to glucocorticoids, with disease recurrence upon initiation of disease-modifying therapies.
Findings:
- The patient exhibited atypical MRI findings and immunological markers characteristic of both anti-NMDAR encephalitis and MS.
- Recurrence of symptoms and lesions occurred despite standard immunomodulatory treatments, indicating disease insensitivity.
- A notable dependency on glucocorticoids for symptom control was observed.
Implications:
- This case underscores the diagnostic and therapeutic challenges in overlapping anti-NMDAR encephalitis and MS.
- Long-term follow-up, dynamic antibody monitoring, and serial MRI are essential for managing these complex cases.
- The patient's glucocorticoid dependency may offer insights into treating refractory cases of recurrent overlapping encephalitis and MS, potentially guiding alternative therapeutic strategies.
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