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

authorA 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 NMOSD

Biopsy-proven case of Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system.

A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. A cerebrospinal fluid examination revealed elevated protein levels and positive Epstein-Barr virus (EBV) DNA. Human immunodeficiency virus was negative. Brain biopsy showed perivascular lymphocytic infiltration in the parenchyma and meninx with EBV-encoded small RNA (EBER). Since this case did not fulfill the criteria for chronic active EBV infection (CAEBV), she was diagnosed with Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system. High-dose methylprednisolone, acyclovir, ganciclovir and foscarnet were not effective. Although EBV is a causative agent of infectious mononucleosis (IM), lymphomas and nasopharyngeal carcinomas, vasculitic pathology of the central nervous system with EBV reactivation in the elderly is rare. Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis

当院の脳血管障害と悪性腫瘍の合併症例に関する検討

雑誌掲載版要旨：【目的】悪性腫瘍を合併した脳卒中患者の特徴を解析することを目的とした．【方法】2011 年1 月から2012 年12 月の間当院に入院した脳卒中患者291 例のうち，悪性腫瘍を合併した症例に関して，その頻度や腫瘍の種類，脳卒中の病型，転帰などについて検討した．【結果】悪性腫瘍合併例は30 例（10％）で，その内訳として肺癌と膵癌が7 例と最も多かった．脳卒中の病型では脳梗塞が21例，脳出血が7 例，一過性脳虚血発作が2 例であった．脳梗塞の発症機序に関して，9 例がTrousseau 症候群による可能性が考えられた．脳卒中による症状の転帰について改善が12 例，不変が12 例，悪化が6 例であった．【結論】脳卒中患者全体の1 割強に悪性腫瘍が合併しており，生命や機能予後に対する影響が大きいため，今後その病態や治療法について検討していく必要があると考えられた