Highlights
- •Distinguishing seronegative NMOSDs from multiple sclerosis is a clinical challenge with important treatment implications.
- •Challenging cases with overlapping features of MS and NMOSDs may demand longitudinal follow-up to make a definite diagnosis.
- •The area postrema expresses aquaporin-4 abundantly and area postrema syndrome is one of the typical features of NMOSDs.
- •Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgGs) and autoantibodies against aquaporin-1 (AQP1-Abs) are the potential new markers for seronegative NMOSDs.
Abstract
Background
Area postrema syndrome is considered as one of the most typical presentations of neuromyelitis
optica spectrum disorders (NMOSDs) (
Wingerchuk et al., 2015
). The involvement of area postrema is rarely seen in multiple sclerosis (MS). We
are here report a case of a young woman with multiple sclerosis, presented with intractable
vomiting, and her MRI brain showed acute T2 hyperintense signal over the area postrema.Case presentation
A 36-year-old Asian woman who is known to have schizophrenia and multiple sclerosis
since 2012. She was noted to have spastic gait, and the MRI brain in 2012 showed multiple
perpendicular periventricular T2 lesions suggestive of multiple sclerosis (MS). However,
she defaulted her neurologist follow-up and was not on any treatment for MS. She was
admitted in 2016 with intractable vomiting, and her MRI brain showed T2 hyperintense
signal over area postrema with focal contrast enhancement. Her MRI cervical spine
was normal. The visual evoked potential study showed bilateral prolonged P100 latencies.
Oligoclonal bands were detected in her CSF analysis. Both the serum aquaporin-4 IgG
(AQP4 IgGs) antibody and myelin oligodendrocyte glycoprotein (MOG-IgGs) were negative.
Her intractable vomiting resolved after a short course of intravenous methylprednisolone.
She was treated as MS with interferon-beta 1a. She has been in remission since 2016,
and her functional status also improved from the expanded disability status scale
(EDSS) of 2.0 (in 2016) to 1.0 (in 2020).
Conclusion
We proposed that although area postrema lesion is typically seen in NMOSDs, it may
also be seen in MS. Current MRI criteria for MS and NMOSDs are not sufficiently specific,
and the diagnostic criteria should only be used in the appropriate clinical context.
Keywords
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Article info
Publication history
Published online: June 30, 2020
Accepted:
June 30,
2020
Received in revised form:
June 29,
2020
Received:
June 1,
2020
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.
