PMC9773878_01
MRI scans obtained at the time of the first attack. (A) Cerebral MRI (sagittal T2 image) exhibiting disseminated lesions in corpus callosum. (B, C) Spinal MRI (sagittal T2 image) exhibiting longitudinally extensive lesion from C3 to T5.
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AQP4-IgG and longitudinally extensive transverse myelitis (LETM, continuous spinal cord lesions extending from cervical to thoracic segments) ( Figure 1 ). No other abnormalities were detected on additional specific tests for autoimmune disorders (serum myelin oligodendrocyte glycoprotein antibodies and glial fibrillary acidic protein antibodies), metagenomic next-generation sequencing of cerebrospinal fluid for microbial infection, hematological examination, serum angiotensin-converting enzyme, tumor markers, and immunoglobulin levels. After 5 cycles of plasma exchange and 5 infusions of intravenous immunoglobulin (IVIg), the patient showed marked alleviation of symptoms and regained the ability to walk (EDSS=3.5).
Schematic illustration of the disease course showing the temporal sequence of symptoms, disability, treatment details, CD19+B cell count, and serum IgM level. EDSS, Expanded Disability Status Scale; FC, finger count; FK506, tacrolimus; GC, glucocorticoids; I.V., intravenous; S.C., subcutaneous; MMF, mycophenolate mofetil; OD, right visual acuity; OS, left visual acuity; LLN, lower limits of normal; VA, visual acuity.
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Unfortunately, two months after RTX induction therapy, she suffered another severe attack with left optic neuritis (visual acuity=finger count) with insufficient depletion of CD19+B cells ( Figure 2 ). However, she experienced five additional episodes of relapse during the last 1.5 years, despite sufficient immunosuppression with oral steroids (prednisone, 10-60 mg per day), mycophenolate mofetil (1000 mg, twice daily), and tacrolimus (1.5 mg, twice daily) ( Figure 2 ). Serum IgM level remained stable ( Figure 2 ).