Investigating paraneoplastic aquaporin‐4‐IgG‐seropositive neuromyelitis optica spectrum disorder through a data‐driven approach

Dinoto, Alessandro; Borin, Giovanni Umberto; Campana, Giulia; Carta, Sara; Ferrari, Sergio; Mariotto, Sara

doi:10.1111/ene.15479

Cited by 9 publications

(2 citation statements)

References 23 publications

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“…The motor outcome is usually poor and most patients become non-ambulatory and wheelchair-dependent ( 13 ). To note, cases of paraneoplastic AQP4+NMOSD have been reported, ( 59 – 61 ) especially in the elderly or in middle-aged males ( 62 , 63 ). Therefore, screening for neoplasms should be performed in the AQP4+NMOSD patients at disease onset meeting these demographic red flags.…”

Section: Spinal Cord Mri Protocol and Lesion Featuresmentioning

confidence: 99%

Neuroimaging features in inflammatory myelopathies: A review

et al. 2022

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Spinal cord involvement can be observed in the course of immune-mediated disorders. Although multiple sclerosis (MS) represents the leading cause of inflammatory myelopathy, an increasing number of alternative etiologies must be now considered in the diagnostic work-up of patients presenting with myelitis. These include antibody-mediated disorders and cytotoxic T cell-mediated diseases targeting central nervous system (CNS) antigens, and systemic autoimmune conditions with secondary CNS involvement. Even though clinical features are helpful to orient the diagnostic suspicion (e.g., timing and severity of myelopathy symptoms), the differential diagnosis of inflammatory myelopathies is often challenging due to overlapping features. Moreover, noninflammatory etiologies can sometimes mimic an inflammatory process. In this setting, magnetic resonance imaging (MRI) is becoming a fundamental tool for the characterization of spinal cord damage, revealing a pictorial scenario which is wider than the clinical manifestations. The characterization of spinal cord lesions in terms of longitudinal extension, location on axial plane, involvement of the white matter and/or gray matter, and specific patterns of contrast enhancement, often allows a proper differentiation of these diseases. For instance, besides classical features, such as the presence of longitudinally extensive spinal cord lesions in patients with aquaporin-4-IgG positive neuromyelitis optica spectrum disorder (AQP4+NMOSD), novel radiological signs (e.g., H sign, trident sign) have been recently proposed and successfully applied for the differential diagnosis of inflammatory myelopathies. In this review article, we will discuss the radiological features of spinal cord involvement in autoimmune disorders such as MS, AQP4+NMOSD, myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and other recently characterized immune-mediated diseases. The identification of imaging pitfalls and mimics that can lead to misdiagnosis will also be examined. Since spinal cord damage is a major cause of irreversible clinical disability, the recognition of these radiological aspects will help clinicians achieve a correct and prompt diagnosis, treat early with disease-specific treatment and improve patient outcomes.

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Section: Spinal Cord Mri Protocol and Lesion Featuresmentioning

confidence: 99%

Neuroimaging features in inflammatory myelopathies: A review

et al. 2022

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“…These syndromes can affect various organ systems and are independent of tumor size or extent of the tumor. 2 Paraneoplastic NMO, although rarely reported, has been associated with various malignancies. 3 Herein, we present a rare case of possible paraneoplastic NMO with respiratory failure in a patient with a history of triple-negative infiltrating ductal carcinoma.…”

Section: Introductionmentioning

confidence: 99%

Breast cancer-associated paraneoplastic neuromyelitis optica with cervical cord compression and spondylosis requiring laminectomy: A case report

Srichawla,

Sivakumar,

Cheraghi

et al. 2023

SAGE Open Medical Case Reports

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Neuromyelitis optica, an autoimmune inflammatory disorder affecting the central nervous system, can occur in a paraneoplastic context, although rare. We report an intriguing case of a 71-year-old woman with a history of triple-negative infiltrating ductal breast carcinoma, manifesting with paraneoplastic neuromyelitis optica that led to significant respiratory failure and required a cervical laminectomy. The patient presented with pain in the left breast, weakness in the lower extremities, and neck pain. The neurological evaluation showed 2/5 muscle strength in all extremities, diffuse hyperreflexia, and loss of multimodal sensation below the shoulder. She developed acute respiratory failure that required mechanical ventilation. Magnetic resonance imaging highlighted a diffuse abnormal increase in T2 signal intensity throughout the posterior and central portion of the cervical and thoracic spinal cord consistent with longitudinally extensive transverse myelitis, and significant cervical cord compression at C3–C4. Magnetic resonance imaging of the brain showed non-enhancing T2/fluid-attenuated inversion recovery (FLAIR) white matter hyperintensities and cerebellar hemispheres. The serum cell-based assay study demonstrated a high anti-aquaporin-4 immunoglobulin G titer (>1:160) confirming the diagnosis of neuromyelitis optica. She was taken for bilateral laminectomy from C3 to C6. Despite intravenous methylprednisolone and plasmapheresis treatment, no significant recovery was achieved, necessitating tracheostomy and percutaneous endoscopic gastrostomy. Subsequent rituximab treatment led to a mild improvement, with no new lesions on repeat magnetic resonance imaging. This case raises suspicion of the potential for neuromyelitis optica to occur as a paraneoplastic phenomenon, strengthening the need for vigilance in patients with malignancies.

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