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Mult Scler. 2014 Sep;20(10):1413-6. doi: 10.1177/1352458514540834. Epub 2014 Jul 1.

A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion.

Author information

1
Division of Neurology, Asahikawa Medical University, Japan sawadajun@gmail.com.
2
Asahikawa Medical University, Japan.
3
Tohoku University Graduate School of Medicine, Japan.
4
Division of Neurology, Asahikawa Medical University, Japan.
5
Tokyo Medical University, Japan.

Abstract

A woman with Sjögren syndrome manifesting as aphasia with a left deep cerebral white matter lesion tested positive for anti-aquaporin 4 (AQP4) antibody. Open biopsy of the lesion revealed active demyelination with edematous changes and the preservation of most axons, indicating a non-necrotic demyelinating lesion. Immunostaining for AQP4 was diffusely lost, whereas the loss of glial fibrillary acidic protein immunostaining was limited but with highly degenerated astrocytic foot processes in perivascular areas. These results suggested neuromyelitis optica spectrum disorder (NMOSD) pathology rather than Sjögren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.

KEYWORDS:

Neuromyelitis optica spectrum disorder (NMOSD); Sjögren syndrome (SS); anti-aquaporin (AQP4) antibody; magnetic resonance imaging (MRI); pathology; white matter lesion

PMID:
24986696
DOI:
10.1177/1352458514540834
[Indexed for MEDLINE]

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