Refractory Longitudinally Extensive Transverse Myelitis Responsive to Cyclophosphamide

Baxter, Laura J; Chen, Shuo; Couillard, Philippe; Scott, James N.; Doig, Christopher J.; Costello, Fiona; Girard, Louis-Philippe; Klassen, John S.; Burton, Jodie M.

doi:10.1017/cjn.2017.201

Cited by 5 publications

(3 citation statements)

References 13 publications

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“…Treatment regimens often involve intravenous pulse therapy with or without corticosteroids every 4–8 weeks ( 23 ). Several studies have demonstrated the effectiveness of Cy, either alone or in combination with other treatments, in managing NMOSD-related LETM ( 24 , 25 ). In this case, the patient’s NMOSD-associated myelitis symptoms were initially managed with IVMP combined with Cy.…”

Section: Discussionmentioning

confidence: 99%

A case report of AQP4-IgG-seropositive refractory neuromyelitis optica spectrum disorder patient with Sjögren’s syndrome and pancytopenia treated with inebilizumab

Li,

Gao,

et al. 2024

Front. Neurol.

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Patients with neuromyelitis optica spectrum disorder (NMOSD) coexisting with both Sjögren’s syndrome (SS) and pancytopenia are exceptionally rare. There is no study on the treatment of such patients. We presented a case of AQP4-IgG seropositive refractory NMOSD patient combined with SS and pancytopenia with significant response to inebilizumab. In 2017 the 49-year-old female patient was diagnosed with SS and pancytopenia without any treatment. In August 2022, she had a sudden onset of lower limbs weakness, manifested as inability to walk, accompanied by urinary incontinence. After receiving methylprednisolone and cyclophosphamide, she regained the ability to walk. In February 2023, she suffered from weakness of both lower limbs again and paralyzed in bed, accompanied by retention of urine and stool, and loss of vision in both eyes. After receiving methylprednisolone and three plasmapheresis, the condition did not further worsen, but there was no remission. In March 2023, the patient was admitted to our hospital and was formally diagnosed with AQP4-IgG seropositive NMOSD combined with SS and pancytopenia. After receiving two 300 mg injections of inebilizumab, not only the symptoms of NMOSD improved significantly, but also the symptoms of concurrent SS and pancytopenia. In the cases of AQP4-IgG seropositive NMOSD who have recurrent episodes and are comorbid with other autoimmune disorders, inebilizumab may be a good choice.

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Section: Discussionmentioning

confidence: 99%

A case report of AQP4-IgG-seropositive refractory neuromyelitis optica spectrum disorder patient with Sjögren’s syndrome and pancytopenia treated with inebilizumab

Li,

Gao,

et al. 2024

Front. Neurol.

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“…The goal is to remove any humoral factors that may be contributing to the underlying pathological process and is highly effective if initiated within the first two weeks after onset of symptoms [18]. If response is still suboptimal, some authors suggest the use of IV immunoglobulin, rituximab or cyclophosphamide; however, their effectiveness remain unvalidated by larger studies [19]. Even though early improvement and response to therapy predict better outcomes, it should be remembered that approximately one third of patients remain severely disabled and some remain with moderate degree of residual disability [1,20].…”

Section: Discussionmentioning

confidence: 99%

Transverse myelitis masquerading as cauda equina syndrome, stroke and cervical myelopathy

Chan¹,

Kaliya-Perumal²,

Yeo³

et al. 2020

BioMedicine

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Transverse myelitis is an uncommon but well-defined neurological syndrome. However, a high index of suspicion is needed to diagnose this condition, especially when it occurs in concomitance with preexisting spinal canal stenosis. We report our patient, a 48 year old male, who initially presented to our spine clinic with acute onset unilateral lower limb weakness associated with urinary retention, which was suspected to be cauda equina syndrome due to a prolapsed intervertebral disc. However, initial magnetic resonance (MR) imaging showed only mild spinal canal stenosis from L2-L5 and C3e C6 levels; thus, the possibility of cauda equina syndrome was ruled out. A few days later, patient developed ipsilateral upper limb weakness giving an impression of hemiparesis due to stroke. However, imaging of brain returned normal. There was still a dilemma whether symptoms could be due to cervical myelopathy as there was mild cervical cord compression with early myelomalacia changes, but the findings were subtle to come to a definite conclusion. Subsequently, patient desaturated and required ventilatory support. Repeat MR imaging of the cervical spine revealed T2 hyperintensities spanning multiple levels in the cervical cord which highlighted the possibility of transverse myelitis and the diagnosis was clinched after a CSF analysis. Despite the debilitating effects, patient responded well to corticosteroid therapy and gradually recovered. This case is reported to highlight the diagnostic dilemma and the rapid progression of transverse myelitis that demands timely medical intervention to avoid permanent disabilities.

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“…Yet, rituximab may be used if all other options have failed, although the evidence of its utility a rescue medication is unclear. For more refractory cases, use of other immunosuppressive agents may need to be considered, such as cyclophosphamide [ 1 , 21 ].…”

Section: Acute Relapse Managementmentioning

confidence: 99%

Contemporary management challenges in seropositive NMOSD

Costello

Burton

2022

J Neurol

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Neuromyelitis optica spectrum disorders (NMOSD) is an inflammatory disorder of the central nervous system that presents unique management challenges. Neurologic disability in NMOSD is directly linked to acute attacks, therefore, relapse prevention is an overarching goal of care. To this end, identifying effective biomarkers that predict relapse onset and severity is of critical importance. As treatment becomes more precision-based and patient-centred, clinicians will need to be familiar with managing circumstances of particular vulnerability for patients with NMOSD, including infection, pregnancy, and the post-partum phase. The discovery of the pathogenic aquaporin-4 Immunoglobulin G (AQP4 IgG) autoantibody almost 20 years ago ultimately distinguished NMOSD as an autoimmune astrocytopathy and helped spearhead recent therapeutic advancements. Targeted therapies, including eculizumab, satralizumab, and inebilizumab, approved for use in aquaporin-4 immunoglobulin G (AQP4 IgG) seropositive patients with NMOSD will likely improve outcomes, but there are formidable costs involved. Importantly, seronegative patients continue to have limited therapeutic options. Moving forward, areas of research exploration should include relapse prevention, restorative therapies, and initiatives that promote equitable access to approved therapies for all people living with NMOSD.

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Refractory Longitudinally Extensive Transverse Myelitis Responsive to Cyclophosphamide

Cited by 5 publications

References 13 publications

A case report of AQP4-IgG-seropositive refractory neuromyelitis optica spectrum disorder patient with Sjögren’s syndrome and pancytopenia treated with inebilizumab

A case report of AQP4-IgG-seropositive refractory neuromyelitis optica spectrum disorder patient with Sjögren’s syndrome and pancytopenia treated with inebilizumab

Transverse myelitis masquerading as cauda equina syndrome, stroke and cervical myelopathy

Contemporary management challenges in seropositive NMOSD

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