Brain Involvement in Neuromyelitis Optica Spectrum Disorders

Abstract

Background: Neuromyelitis optica spectrum disorders (NMOSDs) are severe inflammatory demyelinating disorders of the central nervous system. Brain involvement is increasingly recognized.Objective: To study brain involvement in NMOSDs among Hong Kong Chinese patients.

Rituximab reduces attacks in Chinese patients with neuromyelitis optica spectrum disorders

“…Distinction between optico-spinal multiple sclerosis and NMO is sometimes difficult in patients who had brain lesions, which could be identified in up to 60% of patients with NMO [7,8]. Two of our patients (Nos.…”

MRI characteristics of neuromyelitis optica spectrum disorder

“…12 Although nonspecific small dots and patches of hyperintensity in subcortical and deep white matter on T2-weighted or fluid-attenuated inversion recovery sequences are the most common findings in NMOSD, certain lesions have a location or appearance characteristic for NMOSD. 6,7,[11][12][13][14][15] Before the discovery of anti-AQP4 antibody, brain MRI abnormalities were reported in only 13% to 46% of patients with NMO. 1,8,16 However, when excluding the brain MRI criteria, the incidence of brain MRI abnormalities increased to 50% to 85% using the revised 2006 NMO diagnostic criteria 3,11,13,17,e1-e3 and to 51% to 89% in seropositive patients with NMOSD.…”

“…Lesions in the corpus callosum have been described in 12% to 40% of patients with NMOSD. 12,15,26 Because both NMO and MS frequently have callosal lesions, location by itself is not a unique finding that differentiates NMOSD from MS. However, while the callosal lesions in MS are discrete, ovoid, and perpendicular to the ventricles and involve inferior aspects of the corpus callosum (figure 2A), e13,e14 NMOSD lesions are located immediately next to the lateral ventricles, following the ependymal lining ( figure 1C.a).…”

“…However, it is now known that MS-like lesions may appear in 10% to 12.5% of cases, 11,e3 and 5% to 42% of patients with NMO fulfill the Barkhof criteria. 6,14,15,47 A recent report showed that 13% and 9% of patients with NMOSD, respectively, met Barkhof and the European Magnetic Imaging in MS diagnostic criteria for MS on brain MRI at onset. 7 Lesion probability maps have not found statistically significant lesion locations in patients positive for anti-AQP4 antibody over those with MS. 6 However, distinguishing features were identified on MS brain MRI that were sensitive and specific, such as the presence of a lateral ventricle and inferior temporal lobe lesion, Dawson fingers, or an S-shaped U-fiber lesion, to classify the patient as MS.…”

A Case of Primary Sjögren’s Syndrome Presenting as Mass-Like Encephalitis, With Progression to Neuromyelitis Optica Spectrum Disorder

“…11 However, brain involvement in NMOSD is increasingly being recognized, 12,13 and up to 60% of patients with NMOSD had brain abnormalities on follow-up. 6 Typical patterns of brain involvement in NMOSD, although not pathognomonic, include lesions of the dorsal medulla/area postrema, periependymal regions in the brainstem, diencephalic structures, or cerebral hemispheres or long lesions spanning much of the corpus callosum or corticospinal tracts, corresponding to regions of high AQP4 expression.…”