PMC9905021_01
Timeline with relevant data of events and interventions before and with maintenance tryptophan immunoadsorption (Tr-IA) in two cases with severe NMOSD.. EDSS, expanded disability status scale; FSS, functional system score; Glat, glatirameracetate; IVIG, intravenous immunoglobulin; Mitox, mitoxantron; R, right eye; Ritux, rituximab; TCZ, Tocilizumab; Tr-IA, tryptophan immunoadsorption; VA, visual acuity; VF, visual function.
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At the age of 42 years (Figure 1(a), start of observation period), the patient had an ON attack in the right eye. EDSS was 5.0 consistently due to blindness of the left eye since the age of 22 years (Figure 1(a)). At the age of 43 years, a female, otherwise healthy, patient experienced her first attack of ON (Figure 1(b), start of observation period), which was fully responsive to high-dose steroids. During the course of the disease (Figure 1(b), observation period year 0-10), multiple high-dose steroid pulse therapies were performed to treat acute exacerbations, partly with good response, especially for episodes of ON. The EDSS improved from 6.0 to 5.0, the FSS ambulation improved from 7 to 1 (Figure 1(b)).
MRI imaging of a 52-year-old female patient with AQP4-IgG-seropositive NMOSD (case 1): (a) T2-weighted sagittal and axial spinal cord imaging revealing a new lesion at Th6 (arrow) before start of Tr-IA maintenance therapy and (b) after 2.5 years of Tr-IA therapy stable disease without new spinal lesion.
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Due to the increasing frequency of attacks and the detection of another inflammatory lesion in the thoracic spinal cord at T6 (Figure 2(a)), continued Tr-IA was considered to stabilize the disease course and to prevent further attacks. Approximately 2 weeks after her second vaccination, the patient experienced a mild ON in the right eye with deterioration of VA (cerebral and spinal MRI with stable findings, Figure 2(b)) and mild sensory deficits in the right leg.
MRI imaging of a 49-year-old woman with AQP4-IgG-seronegative NMOSD (case 2). (a) T2-weighted sagittal and axial spinal cord imaging revealing an LETM extending from T2 to T4 (arrow), associated with acute myelitis, 6 years after onset of disease. (b) Sagittal STIR- and axial T1-gadolinium-enhanced images 10 years after disease onset showing acute cervical myelitis and widespread lesions in the entire spinal cord. (c) Axial T2-weighted and T1-gadolinium-enhanced images 11 years after disease onset and before start of Tr-IA maintenance therapy. (d) After 2 years of maintenance therapy with Tr-IA, almost complete regression of spinal lesion load on T-weighted sagittal and axial images is seen.
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Six years after onset of disease at the age of 49 years, MRI showed for the first time longitudinally extensive transverse myelitis (LETM) from T2 to T4 (Figure 3(a)). MRI showed lesions in almost the complete spinal cord, with patchy gadolinium enhancement at several positions (Figure 3(b)). However, approximately 8 weeks after Tr-IA treatments, there was a worsening of clinical symptoms, partly associated with a progression of MRI findings, revealing new lesions and gadolinium-positive lesions (Figure 3(c)). During the remaining observation period of 4 years, no new disease attacks or MRI lesions (Figure 3(d)), based on yearly investigations occurred.