Clinical characteristics of cerebral amyloid angiopathy-related inflammation / 中华神经科杂志

ABSTRACT

Objective:To investigate the clinical, cerebrospinal fluid (CSF) and neuroimaging characteristics and their associations with prognosis in cerebral amyloid angiopathy(CAA)-related inflammation (CAA-ri).Methods:Seventeen patients with CAA-ri, 59 patients with CAA-related intracerebral hemorrhage (ICH) and 15 patients with CAA-related cognitive decline were recruited from Huashan Hospital, Fudan University from November 2015 to May 2020 and the First Affiliated Hospital of University of Science and Technology of China from January 2018 to May 2020. Vascular risk factors and imaging features of cerebral small vessel disease were compared among three groups. Clinical manifestations, CSF results, lesion features on magnetic resonance imaging, treatment options and follow-up data were collected in patients with CAA-ri. The good prognosis was defined by clinical and radiographic improvement with no disease recurrence. The associations between clinical characteristics and the immunosuppressive therapy or the good prognosis were analyzed by binary Logistic regression models.Results:Patients with CAA-ri showed earlier disease onset [(61.5±11.7) years vs (70.9±8.6) years, t=9.428, P=0.001] and more lobar cerebral microbleeds [69.0 (43.5, 134.3) vs 10.0 (5.0, 59.0), H=3.363, P=0.002] compared to patients with CAA-ICH, and higher prevalence of male (14/17 vs 6/15, χ2=6.099, P=0.014) and lower white matter hyperintensity Fazekas score [4.0 (2.0, 6.0) vs 6.0 (5.0, 6.0), H=2.461, P=0.042] compared to patients with CAA-related cognitive decline. In patients with CAA-ri, the immunosuppressive therapy was positively correlated with CSF protein>600 mg/L (odds ratio 16.50, 95% confidence interval 1.09-250.18, P=0.043), and during a follow-up of (3.0±1.9) years, the good prognosis was positively correlated with CSF protein<1 000 mg/L plus immunosuppressive therapy (odds ratio 20.00, 95% confidence interval 1.39-287.60, P=0.028). Conclusions:CAA-ri is a special subtype of CAA with earlier disease onset and higher prevalence of hemorrhagic imaging makers compared to CAA-ICH and CAA-related cognitive decline. CAA-ri patients with normal or slightly elevated CSF protein receiving immunosuppressive therapy are more likely to have good prognosis.