Encefalomielitis asociada a anticuerpos del receptor NMDA
DOI:
https://doi.org/10.36958/sep.v8i2.380Keywords:
anti-NMDAr, NMDA, EncephalomyelitisAbstract
NMDA receptor antibody–associated encephalomyelitis may present without psychiatric manifestations, with phenotypes dominated by spinal cord and brainstem involvement.
CASE REPORT: A 60-year-old male developed 48-hour paresthesias that progressed to asymmetric paraparesis with a T5–T6 sensory level, urinary retention, and a right Babinski sign. Studies: CSF showed mononuclear pleocytosis and elevated protein with a negative infectious panel; MRI demonstrated a short cervical lesion and longitudinally extensive thoracic myelitis (focal enhancement at T6–T7) plus multifocal FLAIR lesions in the medulla, pons, and midbrain. Serum NMDAR was positive; oncologic screening (CT and tumor markers) was unremarkable, with functional improvement after methylprednisolone 1 gram every 24 hours.
CONCLUSION: In encephalomyelitis with inflammatory CSF and exclusion of major differentials, NMDAR seropositivity warrants early immunotherapy and close follow-up.
KEYWORDS: encephalomyelitis; NMDAR antibodies; NMDA receptor; longitudinally extensive transverse myelitis; brainstem; cerebrospinal fluid; AQP4; MOG; steroids.
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Ayvacıoğlu Çağan, C., Göçmen, R., Acar Özen, N. P., & Tuncer, A. (2022). Life after tetra hit: Anti-NMDAR encephalitis after HSV encephalitis in a NMOSD coexistent with Sjögren’s syndrome. Archives of Neuropsychiatry, 59(2), 161–163. https://doi.org/10.29399/npa.27507
2. Bastiaansen, A. E. M., de Bruijn, M. A. A. M., Schuller, S. L., Martinez-Hernandez, E., Brenner, J., Paunovic, M., Crijnen, Y. S., Mulder, M. J. H. L., Schreurs, M. W. J., de Graaff, E., Eil, J., Neuteboom, R. F., de Vries, J. M., Sillevis Smitt, P. A. E., & Titulaer, M. J. (2022). Anti-NMDAR encephalitis in the Netherlands, focusing on late-onset patients and antibody test accuracy in serum and CSF. Neurology: Neuroimmunology & Neuroinflammation, 9(1), e1127. https://doi.org/10.1212/NXI.0000000000001127
3. Blackman, G., Lim, M. F., Pollak, T., Al-Diwani, A., Symmonds, M., Mazumder, A., Carter, B., Irani, S., & David, A. (2022). The clinical relevance of serum versus CSF NMDAR autoantibodies associated exclusively with psychiatric features. Journal of Neurology, 269(10), 5302–5311. https://doi.org/10.1007/s00415-022-11224-6
4. Dalmau, J., Lancaster, E., Martinez-Hernandez, E., Rosenfeld, M. R., & Balice-Gordon, R. (2011). Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. The Lancet Neurology, 10(1), 63–74. https://doi.org/10.1016/S1474-4422(10)70253-2
5. Dubey, D., Pittock, S. J., & McKeon, A. (2019). Antibody Prevalence in Epilepsy and Encephalopathy score: Increased specificity and applicability. Epilepsia, 60(2), 367–369. https://doi.org/10.1111/epi.14649
6. Graus, F., Titulaer, M. J., Balu, R., Benseler, S., Bien, C. G., Cellucci, T., Cortese, I., Dale, R. C., Gelfand, J. M., Geschwind, M., Glaser, C. A., Honnorat, J., Höftberger, R., Iizuka, T., Irani, S. R., Lancaster, E., Leypoldt, F., Prüss, H., Rae-Grant, A., … Dalmau, J. (2016). A clinical approach to diagnosis of autoimmune encephalitis. The Lancet Neurology, 15(4), 391–404. https://doi.org/10.1016/S1474-4422(15)00401-9
7. Hümmert, M. W., Jendretzky, K. F., Fricke, K., Gingele, M., Ratuszny, D., Möhn, N., Trebst, C., Skripuletz, T., Gingele, S., & Sühs, K.-W. (2023). The relevance of NMDA receptor antibody-specific index for diagnosis and prognosis in patients with anti-NMDA receptor encephalitis. Scientific Reports, 13, 12696. https://doi.org/10.1038/s41598-023-38462-6
8. Nguyen, L., & Wang, C. (2023). Anti-NMDA receptor autoimmune encephalitis: Diagnosis and management strategies. International Journal of General Medicine, 16, 7–21. https://doi.org/10.2147/IJGM.S397429
9. Orozco, I. J., Dubey, D., McKeon, A., McKeon, S., Zekeridou, A., & Flanagan, E. P. (2023). Autoimmune encephalitis criteria in clinical practice. Neurology: Clinical Practice, 13(4), e200187. https://doi.org/10.1212/CPJ.0000000000200187
10. Outteryck, O., Baille, G., Hodel, J., Giroux, M., Lacour, A., Honnorat, J., Zéphir, H., & Vermersch, P. (2013). Extensive myelitis associated with anti-NMDA receptor antibodies. BMC Neurology, 13, 211. https://doi.org/10.1186/1471-2377-13-211
11. Pollak, T. A., Kempton, M. J., Iyegbe, C., Vincent, A., Irani, S. R., Coutinho, E., Menassa, D. A., Jacobson, L., de Haan, L., Ruhrmann, S., Sachs, G., Riecher-Rössler, A., Krebs, M.-O., Amminger, P., Glenthøj, B., Barrantes-Vidal, N., van Os, J., Rutten, B. P. F., Bressan, R. A., … McGuire, P. (2021). Clinical, cognitive and neuroanatomical associations of serum NMDAR autoantibodies in people at clinical high risk for psychosis. Molecular Psychiatry, 26, 2590–2604. https://doi.org/10.1038/s41380-020-00899-w
12. Titulaer, M. J., Höftberger, R., Iizuka, T., Leypoldt, F., McCracken, L., Cellucci, T., Benson, L. A., Shu, H., Irioka, T., Hirano, M., Singh, G., Cobo Calvo, Á., Kaida, K., Morales, P. S., Wirtz, P. W., Yamamoto, T., Reindl, M., Rosenfeld, M. R., Graus, F., … Dalmau, J. (2014). Overlapping demyelinating syndromes and anti-NMDA receptor encephalitis. Annals of Neurology, 75(3), 411–428. https://doi.org/10.1002/ana.24117
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