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

doi:10.1177/1352458514540834

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 journal › Article › peer-review

7 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 language	English
Pages (from-to)	1413-1416
Number of pages	4
Journal	Multiple Sclerosis
Volume	20
Issue number	10
DOIs	https://doi.org/10.1177/1352458514540834
Publication status	Published - 2014 Sept

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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Sawada, J., Orimoto, R., Misu, T., Katayama, T., Aizawa, H., Asanome, A., Takahashi, K., Saito, T., Anei, R., Kamada, K., Miyokawa, N., Takahashi, T., Fujihara, K., & Hasebe, N. (2014). A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion. Multiple Sclerosis, 20(10), 1413-1416. https://doi.org/10.1177/1352458514540834

Sawada, J, Orimoto, R, Misu, T, Katayama, T, Aizawa, H, Asanome, A, Takahashi, K, Saito, T, Anei, R, Kamada, K, Miyokawa, N, Takahashi, T, Fujihara, K & Hasebe, N 2014, 'A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion', Multiple Sclerosis, vol. 20, no. 10, pp. 1413-1416. https://doi.org/10.1177/1352458514540834

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title = "A case of pathology-proven neuromyelitis optica spectrum disorder with Sj{\"o}gren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion",

abstract = "A woman with Sj{\"o}gren 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 Sj{\"o}gren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.",

keywords = "Anti-aquaporin (AQP4) antibody, Magnetic resonance imaging (MRI), Neuromyelitis optica spectrum disorder (NMOSD), Pathology, Sj{\"o}gren syndrome (SS), White matter lesion",

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

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AU - Misu, Tatsuro

AU - Katayama, Takayuki

AU - Aizawa, Hitoshi

AU - Asanome, Asuka

AU - Takahashi, Kae

AU - Saito, Tsukasa

AU - Anei, Ryogo

AU - Kamada, Kyousuke

AU - Miyokawa, Naoyuki

AU - Takahashi, Toshiyuki

AU - Fujihara, Kazuo

AU - Hasebe, Naoyuki

N1 - Publisher Copyright: © The Author(s), 2014.

PY - 2014/9

Y1 - 2014/9

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AB - 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.

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

Abstract

Keywords

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