Researchers in Italy reported the first case of a patient with double-positive neuromyelitis optica spectrum disorder (NMOSD) with the involvement of both the central and peripheral nervous systems.
Very rare cases of anti-aquaporin-4 (AQP4)- and anti-myelin oligodendrocytic glycoprotein (MOG)-positive NMOSD have been reported in the literature in the past. However, these cases only involved the spinal cord and optic nerve, Roberta Lanzillo, MD, PhD, of the Federico II University of Naples in Italy, and colleagues noted.
“Collection and presentation of double-positive AQP4 and MOG NMOSD cases can shed light on the immunopathogenesis of this condition,” the study authors wrote.
Read more about the pathophysiology of NMOSD
The paper was published in the International Journal of Molecular Sciences.
The team performed brain and spine MRIs, cerebrospinal fluid studies, and electrophysiological tests. They also assessed AQP4 and MOG positivity using a live cell-based assay.
The case the researchers presented is that of a 62-year-old female who presented with recurrent optic neuritis, myelitis, and radiculitis. She tested AQP4 and MOG antibodies. In addition to her visual impairment, she developed whole-body hypoesthesia and flaccid tetraplegia with urinary incontinence.
She was treated with rituximab. Six months after her first dose of treatment, her AQP4-Ig and MOG-Ig titers were reduced, and her condition improved.
NMOSD is a rare chronic inflammatory disease affecting the nervous system. Patients who are clinically diagnosed are usually grouped as either having AQP4-antibody-seropositive autoimmune astrocytopathic disease or MOG-antibody-seropositive inflammatory demyelinating disease.
Common symptoms include ocular pain, vision loss, numbness or weakness in the arms or legs, dizziness, loss of balance, abnormal sensations like pins and needles, and problems with the control of the bowels and bladder.
Spiezia AL, Carotenuto A, Iovino A, et al. AQP4-MOG double-positive neuromyelitis optica spectrum disorder: case report with central and peripheral nervous system involvement and review of the literature. Int J Mol Sci. Published online November 23, 2022. doi:10.3390/ijms232314559