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

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

来源：Multiple Sclerosis, 2014, 20(10): 1413-1416.

DOI：10.1177/1352458514540834

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

A woman with Sjogren 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 Sjogren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.

• 出版日期2014-9