Spectrum of MRI brain lesion patterns in neuromyelitis optica spectrum disorder: a pictorial review

Abstract

Neuromyelitis optica is a neurotropic autoimmune inflammatory disease of the central nervous system traditionally thought to exclusively involve the optic nerves and spinal cord. With the discovery of the disease-specific aquaporin-4 antibody and the increasing recognition of clinical and characteristic imaging patterns of brain involvement in what is now termed neuromyelitis optica spectrum disorder (NMOSD), MRI now plays a greater role in diagnosis of NMOSD based on the 2015 consensus criteria and in distinguishing it from other inflammatory disorders, particularly multiple sclerosis (MS). Several brain lesion patterns are highly suggestive of NMOSD, whereas others may serve as red flags. Specifically, long corticospinal lesions, hemispheric cerebral white matter lesions and periependymal lesions in the diencephalon, dorsal brainstem and white matter adjacent to lateral ventricles are typical of NMOSD. In contrast, juxtacortical, cortical, or lesions perpendicularly oriented to the surface of the lateral ventricle suggests MS as the diagnosis. Ultimately, a strong recognition of the spectrum of MRI brain findings in NMOSD is essential for accurate diagnosis, and particularly in differentiating from MS. This pictorial review highlights the spectrum of characteristic brain lesion patterns that may be seen in NMOSD and further delineates findings that may help distinguish it from MS.

Central nervous system demyelinating diseases in Hong Kong

“…Brainstem was the most frequently affected region (44%), followed by the hemispheric periventricular white matter (21%), deep white matter (21%), and corpus callosum (12%) 48 . These findings were consistent with later published international data on the spectrum of NMOSD brain lesions 49 …”

“…48 These findings were consistent with later published international data on the spectrum of NMOSD brain lesions. 49 A cohort in 2015 compared the MRI features of 70 MS and 31 NMOSD subjects. A Dawson's fingers appearance in the brain had the strongest correlation with MS (OR = 59.0), followed by multiple short-segment, peripherally located and asymptomatic spinal cord lesions.…”

Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis

“…Hence, the NMOSD 2015 diagnostic criteria have incorporated findings of cerebral MRI and define NMOSD-typical brain lesions ( 46 ). These lesions can be located at the periependymal surfaces of the third and fourth ventricle, in the area postrema, corpus callosum, hypothalamus or thalamus ( 130 – 132 ). In addition, subcortical or deep white matter lesions are possible.…”

Moyamoya disease presenting as thalamic infarction in a patient with neuromyelitis optica spectrum disorder

“…With the discovery of the disease-specific aquaporin-4 (AQP4) antibody and the improvement of understanding of clinical and imaging patterns of brain involvement in what is now termed neuromyelitis optica spectrum disorder (NMOSD). 1 The diagnosis of NMOSD is mainly based on the positive AQP4 antibody and the presence of at least one of the core clinical characteristics associated with optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. 2 Moyamoya disease (MMD), which was first reported by Japanese scholars Suzuki and Takaku in 1969, is a rare cerebrovascular disease characterized by bilateral progressive stenosis or occlusion of the terminal portion of the internal carotid artery and its main branches with emergence of coexisting abnormal net-like vessels.…”

“…Neuromyelitis optica is an immune‐mediated inflammatory demyelinating disease of the central nervous system typically involving optic nerves and spinal cord. With the discovery of the disease‐specific aquaporin‐4 (AQP4) antibody and the improvement of understanding of clinical and imaging patterns of brain involvement in what is now termed neuromyelitis optica spectrum disorder (NMOSD) . The diagnosis of NMOSD is mainly based on the positive AQP4 antibody and the presence of at least one of the core clinical characteristics associated with optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations …”