Pattern Recognition of the Multiple Sclerosis Syndrome

During recent decades, the autoimmune disease neuromyelitis optica spectrum disorder (NMOSD), once broadly classified under the umbrella of multiple sclerosis (MS), has been extended to include autoimmune inflammatory conditions of the central nervous system (CNS), which are now diagnosable with serum serological tests. These antibody-mediated inflammatory diseases of the CNS share a clinical presentation to MS. A number of practical learning points emerge in this review, which is geared toward the pattern recognition of optic neuritis, transverse myelitis, brainstem/cerebellar and hemispheric tumefactive demyelinating lesion (TDL)-associated MS, aquaporin-4-antibody and myelin oligodendrocyte glycoprotein (MOG)-antibody NMOSD, overlap syndrome, and some yet-to-be-defined/classified demyelinating disease, all unspecifically labeled under MS syndrome. The goal of this review is to increase clinicians’ awareness of the clinical nuances of the autoimmune conditions for MS and NMSOD, and to highlight highly suggestive patterns of clinical, paraclinical or imaging presentations in order to improve differentiation. With overlay in clinical manifestations between MS and NMOSD, magnetic resonance imaging (MRI) of the brain, orbits and spinal cord, serology, and most importantly, high index of suspicion based on pattern recognition, will help lead to the final diagnosis.

Note: One spinal cord lesion can substitute for vone brain lesion.
Note: A spinal cord is equivalent to an infratentorial lesion and can contribute with brain lesions to the required number of T2 lesions. CEL spinal cord lesion is considered equivalent to a brain CEL Note: A CEL is not required for DIS. If a subject has a brainstem or spinal cord syndrome, the symptomatic lesions are excluded from the criteria and do not contribute to the lesion count.
Note: If a patient has a brainstem or spinal cord syndrome, or optic neuritis, the symptomatic lesion(s) is not excluded from the criteria and can contribute to the lesion count.
DIT can be demonstrated by the following manner: 1. If a first scan occurs > 3 months after the onset of the clinical event, the presence of a CEL is sufficient to demonstrate DIT, provided that it is not at the site implicated in the original clinical event. If there is no CEL, a followup scan is required 3 months later. A new T2 or CEL at this time then fulfills the criteria for DIT. If the first scan is performed < 3 months after the onset of the clinical event, and a second scan performed three months or longer after the clinical event shows a new CEL, then this provides sufficient evidence for DIT. If no enhancing lesion is seen at this second scan, a further scan, not before 3 months after the first scan that shows a new T2 lesions or a CEL, will suffice. DIT can be demonstrated by the following two ways using imaging: 1. Detection of CEL at least three months after the onset of the initial clinical event, if not at the site corresponding to the initial event.
2. Detection of a new T2 lesion if it appears at any time compared with a reference scan done at least 30 days after the onset of the initial clinical event.
DIT can be demonstrated by the following manner: 1. A new T2 and/or CEL(s) on follow-up MRI, with reference to a baseline scan, irrespective of the timing of the baseline MRI.

Simultaneous presence of asymptomatic CEL and non-CEL at any time.
DIT can be demonstrated by the following manner: 1. A new T2 and/or CEL(s) on follow-up MRI, with reference to a baseline scan, irrespective of the timing of the baseline MRI. 2. Simultaneous presence of asymptomatic CEL and non-CEL at any time.   (1c). Repeat MRI of the brain 6 months later showed a significant improvement in T2 hyperintensity (1d) underlining the evanescent nature of NMOSD lesions.  Figure S3: Sagittal and axial FLAIR MRI of the brain demonstrate diffuse involvement and swelling of the corpus callosum (3a and 3c) with high intensity rim and lower intensity core. Axial T1 with contrast demonstrates heterogeneous contrast enhancement. Repeat brain MRI (3b), 8 months later, demonstrates resolved edematous state with some callosal atrophy. Figure S4: 40-year-old Caucasian women presenting with intractable hiccups, nausea and vomiting and a dorsal brainstem lesion with a linear component involving the medulla and cervicomedullary junction seen on sagittal STIR (4a) and enhancing with contrast on T1. Aquaporin 4 antibody was positive. 3b 3d 3c 4a 4b 3a Figure S5: 43-year-old female, with AQP4-NMOSD; axial FLAIR demonstrates non-specific white matter lesions, (5a) a periventricular lesion around the posterior horn of the left lateral ventricle (5b), confirmed on sagittal FLAIR (5c). 5b 5c 5a