title: Neurological antiphospholipid syndrome: Clinical, neuroimaging, and pathological characteristics.
abstract: BACKGROUND
  Neurological antiphospholipid syndrome (NAPS) is often misdiagnosed or missed. Only limited clinical and neuroimaging information about it is available, and the pathological characteristics was rarely reported before. This study aimed to explore the clinical, neuroimaging, and pathological characteristics of NAPS.
  
  
  METHODS
  We performed a retrospective analysis of 51 patients with APS, categorized into NAPS (n = 16) and rheumatology antiphospholipid syndrome (RAPS) groups (n = 35). Demographics and clinical profile were compared between the two groups, and the neuroimaging and pathological information of NAPS was also analyzed.
  
  
  RESULTS
  The mean age of the NAPS patients, 81.25% of whom were female, was 37.56 ± 12.36 years, and the average duration was 1.32 ± 0.96 years (range = 18 days to 3.5 years). No significant differences in age, sex, disease duration, classification, and comorbidities at baseline were observed between NAPS and RAPS patients (p > 0.05). Chief complaint of headache and thromboembolic events was higher in NAPS patients than in RAPS patients (p<0.05). Neuroimaging detected multiple infarcts and demyelination lesions were distributed in subcortical and cortical area asymmetrically. Skin biopsy examination showed small vessel occlusion with inflammatory cells, while brain biopsy examination showed erythrocyte accumulation with some neuron degeneration and local demyelization. Antithrombotic and immunosuppressive therapy proved to be effective.
  
  
  CONCLUSIONS
  Headache and thromboembolic events are more common in NAPS than RAPS. Neuroimaging and biopsy examination demonstrated that NAPS is an ischemic cerebrovascular disease caused by vascular stenosis or occlusion. These characteristics might help to reduce the misdiagnosis of NAPS.
authors.affiliation: Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China.
authors.forename: De-Sheng
authors.initials: DS
authors.lastname: Zhu
authors.affiliation: Department of Pathology, Fuzhou Medical College of Nanchang University, Fuzhou 344000, China.
authors.forename: Jue
authors.initials: J
authors.lastname: Fu
authors.affiliation: Department of Neurology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.
authors.forename: Yue
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Neurology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China.
authors.forename: Shi-Xu
authors.initials: SX
authors.lastname: Li
authors.affiliation: Department of Neurology, Thomas Jefferson University, PA 19107, USA.
authors.forename: Guang-Xian
authors.initials: GX
authors.lastname: Zhang
authors.affiliation: Department of Neurology, Changhai Hospital, Second Military Medical University, Shanghai 200433, China. Electronic address: yangtaiguan@hotmail.com.
authors.forename: Yang-Tai
authors.initials: YT
authors.lastname: Guan
authors.affiliation: Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China. Electronic address: qiangdong-fudan@hotmail.com.
authors.forename: Qiang
authors.initials: Q
authors.lastname: Dong
mesh_terms: D000328:Adult; D000367:Age Factors; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D001706:Biopsy; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D003711:Demyelinating Diseases; D005260:Female; D006261:Headache / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012737:Sex Factors; D012867:Skin / Q000473:pathology; D013923:Thromboembolism / Q000209:etiology / Q000473:pathology*
keywords: Antiphospholipid antibodies; Antiphospholipid syndrome; Central nervous system; Clinical features; Neuroimaging; Pathology