A case of midbrain infarction causing ipsilateral mydriasis, contralateral superior rectus palsy, and bilateral ptosis

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Non-Vitamin K Antagonist Oral Anticoagulants Do Not Increase Cerebral Microbleeds

オープンアクセス CC-BY-NC-NDBACKGROUND: Atrial fibrillation (AF) is a cardiac arrhythmia that frequently induces ischemic strokes. Nowadays, non-vitamin K antagonist oral anticoagulants (NOACs) have come into widespread use for cardiogenic embolism prevention in place of warfarin. Recently, cerebral microbleeds (CMBs) have been noticed for their potential implication in cerebral small vessel disease. We hypothesized that NOACs do not have an unfavorable influence over cerebral small vessels and investigated whether NOACs increase CMBs in AF patients in a prospective manner. METHODS: We performed baseline magnetic resonance imaging (MRI) examinations on the 69 enrolled AF patients and re-examined second round of MRI 1 year later. The enrolled patients continued the same anticoagulation therapy during the meantime. RESULTS: CMBs did not develop in the 23 patients with NOACs for 1 year. Nine patients with antiplatelets also did not develop CMBs. On the other hand, 3 of 21 patients continued on warfarin and 3 of 9 with warfarin and antiplatelets had CMBs. When divided into 2 groups according to whether the CMBs developed, significant differences in the incidence of using NOACs were observed between the 2 groups (P = .02). A multivariate regression analysis showed that warfarin was independently related to the new development of CMBs (hazard ratio, 10.75; 95% confidence interval, 1.22-94.99; P = .03). CONCLUSIONS: This is the first report to clarify that NOACs do not increase CMBs in AF patients longitudinally in 1 year. Further consideration will be continued with a much longer follow-up in large samples

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

A Sporadic Case of Fabry Disease Involving Repeated Fever, Psychiatric Symptoms, Headache, and Ischemic Stroke in an Adult Japanese Woman

Fabry disease can cause various neurological manifestations. We describe the case of a Japanese woman with Fabry disease who presented with ischemic stroke, aseptic meningitis, and psychiatric symptoms. The patient had a mutation in intron 4 of her α-galactosidase A gene, which was not detected in her family. This case suggests that Fabry disease should be considered in young patients who exhibit central nervous system symptoms such as ischemic stroke, even if there is no family history of the condition. The episodes of aseptic meningitis and stroke experienced by our patient suggest that persistent inflammation might be the mechanism underlying Fabry disease

内包・放線冠領域梗塞急性期の構音障害は左側病巣で出現しやすい

雑誌掲載版【背景と目的】内包・放線冠領域急性期梗塞で構音障害を呈する際の左右の多寡は従来さほど強調されていない。本研究では構音における同部位の左右の優位性の有無を検討することを目的とした。【方法】旭川医科大学ストロークチームが2006年から5年間に診療した全脳卒中患者を対象とし後ろ向き調査を行った。【結果】対象886例のうち、単発の内包・放線冠領域梗塞患者は96例。その中で急性期に構音障害を呈した43例中、左側梗塞は30例(69.8%)、右側梗塞は13例(30.2%)であった。また構音障害を呈さなかった53例中、左側梗塞は24例(45.3%)、右側梗塞は29例(54.7%)であった。χ2検定では左側梗塞で有意に多く構音障害を呈した(p=0.016、オッズ比2.79、95% CI 1.20-6.50)。【結論】内包・放線冠領域急性期梗塞で構音障害を呈する場合、左側に梗塞巣を有する症例が有意に多かった

30代で発症した家族性地中海熱の1例

著者最終原稿版53歳男。35歳頃より月に1日程度37度台の発熱と体幹・関節の疼痛および胸背部や肩関節痛が出現するようになり、続いて腹痛も出現し、発熱の頻度も週に1日程度に増加した。原因の特定のため当科を受診した。CRPの上昇と赤沈亢進以外に異常所見は認めず、周期的発熱に随伴する症状は漿膜炎症状や関節炎症状であったため、自己炎症疾患の可能性を考え遺伝子解析を実施した。家族性地中海熱(FMF)遺伝子のMEFV遺伝子exon 2および10にE148Q/M694Iの変異を認め、FMFと確定診断した。コルヒチン1mg/day内服で発熱はなくなり、体幹や関節の疼痛も消失し炎症反応の上昇も認めなくなったが、コルヒチンによると考えられる肝障害が出現した。コルヒチン1回0.5mg/を週2〜3回の内服に減量したところ、肝障害の改善を認め、発熱、体幹や関節の疼痛悪化もなく経過している

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