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

Jun Sawada, Ryosuke Orimoto, Tatsuro Misu, Takayuki Katayama, Hitoshi Aizawa, Asuka Asanome, Kae Takahashi, Tsukasa Saito, Ryogo Anei, Kyousuke Kamada, Naoyuki Miyokawa, Toshiyuki Takahashi, Kazuo Fujihara, Naoyuki Hasebe

Research output: Contribution to journalArticlepeer-review

6 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1413-1416
Number of pages4
JournalMultiple Sclerosis
Volume20
Issue number10
DOIs
Publication statusPublished - 2014 Sep

Keywords

  • Anti-aquaporin (AQP4) antibody
  • Magnetic resonance imaging (MRI)
  • Neuromyelitis optica spectrum disorder (NMOSD)
  • Pathology
  • Sjögren syndrome (SS)
  • White matter lesion

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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