Neurological antiphospholipid syndrome: Clinical, neuroimaging, and pathological characteristics

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.

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.

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.

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.