A case of anti-AQP4 antibody–positive neuromyelitis optica spectrum disorder with MRI-proven lesions in lumbar nerve roots

Toru, Shuta; Soejima, Itsuki; Katayama, Yuki; Saito, Kazuyuki; Yokote, Hiroaki

doi:10.1016/j.msard.2020.102557

Cited by 4 publications

(3 citation statements)

References 7 publications

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“…However, mild symptoms may simply not have been acknowledged. Moreover, polyradiculomyelitis has also been described in the context of both aquaporin-4 antibody positive neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein (MOG) associated disorder (15)(16)(17)(18). In particular, MOG-associated disorder frequently presents with longitudinally extensive lesions restricted in the SCGM of the thoracolumbar region, typically with absent contrast enhancement; in contrast, in patients with aquaporin-4 antibody positive myelitis, cervical and thoracic longitudinally extensive lesions with contrast enhancement are more frequent (19,20).…”

Section: Discussionmentioning

confidence: 99%

Acute Polyradiculomyelitis With Spinal Cord Gray Matter Lesions: A Report of Two Cases

et al. 2021

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Objective: Inflammatory polyradiculomyelitis belongs to a rare group of immune-mediated diseases affecting both the central and peripheral nervous system. We aimed to describe an unusual presentation of acute polyradiculomyelitis with marked spinal cord lesions restricted to the gray matter.Methods: Thorough examination of two case reports including clinical, MRI, serologic, electrophysiologic and CSF examinations as well as short-term follow-up.Results: We present two adult patients with acute polyradiculomyelitis and unusual spinal cord lesions restricted to the gray matter on MRI. The clinical presentation, serologic, electrophysiologic and CSF features of the two patients varied, whereas both patients demonstrated severe, asymmetrical, predominantly distal, motor deficits of the lower extremities as well as bladder and bowel dysfunction. Both patients only partially responded to anti-inflammatory treatment. Severe motor impairment and bladder dysfunction persisted even months after symptom onset.Conclusions: To our best of knowledge, these are the first reports of acute polyradiculomyelitis with distinct involvement of the lower thoracic spinal cord gray matter. Currently, it remains unclear whether gray matter lesions reflect a separate pathophysiologic mechanism or an exceedingly rare presentation of spinal cord involvement in acute polyradiculomyelitis.

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Section: Discussionmentioning

confidence: 99%

Acute Polyradiculomyelitis With Spinal Cord Gray Matter Lesions: A Report of Two Cases

et al. 2021

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“…A recent case report had shown the presence of high signal lesions within lumbar nerve roots in a patient with NMOSD [139].…”

Section: Imaging Of the Peripheral Nervous Systemmentioning

confidence: 97%

Magnetic resonance imaging in neuromyelitis optica spectrum disorder

Clarke

Arnett

Lilley

et al. 2021

Clinical and Experimental Immunology

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Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system (CNS) associated with antibodies to aquaporin-4 (AQP4), which has distinct clinical, radiological and pathological features, but also has some overlap with multiple sclerosis and myelin oligodendrocyte glycoprotein (MOG) antibody associated disease. Early recognition of NMOSD is important because of differing responses to both acute and preventive therapy. Magnetic resonance (MR) imaging has proved essential in this process. Key MR imaging clues to the diagnosis of NMOSD are longitudinally extensive lesions of the optic nerve (more than half the length) and spinal cord (three or more vertebral segments), bilateral optic nerve lesions and lesions of the optic chiasm, area postrema, floor of the IV ventricle, periaqueductal grey matter, hypothalamus and walls of the III ventricle. Other NMOSD-specific lesions are denoted by their unique morphology: heterogeneous lesions of the corpus callosum, 'cloud-like' gadolinium (Gd)-enhancing white matter lesions and 'bright spotty' lesions of the spinal cord. Other lesions described in NMOSD, including linear periventricular peri-ependymal lesions and patch subcortical white matter lesions, may be less specific.The use of advanced MR imaging techniques is yielding further useful information regarding focal degeneration of the thalamus and optic radiation in NMOSD and suggests that paramagnetic rim patterns and changes in normal appearing white matter are specific to MS. MR imaging is crucial in the early recognition of NMOSD and in directing testing for AQP4 antibodies and guiding immediate acute treatment decisions.Increasingly, MR imaging is playing a role in diagnosing seronegative cases of NMOSD.

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“…Spinal nerve root enhancement or myeloradiculitis has been observed in a few patients with AQP4-IgG-positive NMOSD [ 47 , 101 , 102 , 204 , 211 ], but was recently reported also in MOG-EM/MOGAD [ 176 , 202 , 206 , 217 ] and even in individual patients with (pediatric) MS [ 206 ]. Importantly, root enhancement should also prompt radiologists to consider spinal cord sarcoidosis as an alternative diagnosis (which may be associated with subpial, leptomeningeal enhancement; LETM; and a “trident sign” on axial imaging).…”

Section: Practical Recommendations On Imaging—mrimentioning

confidence: 99%

Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis

et al. 2023

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The term ‘neuromyelitis optica spectrum disorders’ (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and its formes frustes and to a number of closely related clinical syndromes without AQP4-IgG. NMOSD were originally considered subvariants of multiple sclerosis (MS) but are now widely recognized as disorders in their own right that are distinct from MS with regard to immunopathogenesis, clinical presentation, optimum treatment, and prognosis. In part 1 of this two-part article series, which ties in with our 2014 recommendations, the neuromyelitis optica study group (NEMOS) gives updated recommendations on the diagnosis and differential diagnosis of NMOSD. A key focus is on differentiating NMOSD from MS and from myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD), which shares significant similarity with NMOSD with regard to clinical and, partly, radiological presentation, but is a pathogenetically distinct disease. In part 2, we provide updated recommendations on the treatment of NMOSD, covering all newly approved drugs as well as established treatment options.

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A case of anti-AQP4 antibody–positive neuromyelitis optica spectrum disorder with MRI-proven lesions in lumbar nerve roots

Cited by 4 publications

References 7 publications

Acute Polyradiculomyelitis With Spinal Cord Gray Matter Lesions: A Report of Two Cases

Acute Polyradiculomyelitis With Spinal Cord Gray Matter Lesions: A Report of Two Cases

Magnetic resonance imaging in neuromyelitis optica spectrum disorder

Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis

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