A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion
| Autor: | Takayuki Katayama, Hitoshi Aizawa, Ryogo Anei, Toshiyuki Takahashi, Tsukasa Saito, Naoyuki Miyokawa, Kyousuke Kamada, Asuka Asanome, Ryosuke Orimoto, Naoyuki Hasebe, Tatsuro Misu, Kae Takahashi, Kazuo Fujihara, Jun Sawada |
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| Rok vydání: | 2014 |
| Předmět: |
Pathology
medicine.medical_specialty magnetic resonance imaging (MRI) Apraxias Biopsy white matter lesion Apraxia Lesion Leukoencephalopathies Aphasia medicine Humans Glucocorticoids Autoantibodies Aquaporin 4 Cerebral Cortex Neuromyelitis optica Sjögren syndrome (SS) Glial fibrillary acidic protein biology business.industry Multiple sclerosis Neuromyelitis Optica Neuromyelitis optica spectrum disorder (NMOSD) Middle Aged medicine.disease Immunohistochemistry Magnetic Resonance Imaging White Matter Sjogren's Syndrome Treatment Outcome Neurology anti-aquaporin (AQP4) antibody biology.protein Female pathology Neurology (clinical) medicine.symptom business Vasculitis Immunostaining |
| Zdroj: | Multiple Sclerosis Journal. 20:1413-1416 |
| ISSN: | 1477-0970 1352-4585 |
| DOI: | 10.1177/1352458514540834 |
| Popis: | author 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 |
| Databáze: | OpenAIRE |
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