Characterization of Clinical Course and Response to Treatment on Cerebral Amyloid Angiopathy-Related Inflammation
Year: 2024
Type: article
Study design: Observational Study
Abstract: Objective Characterize response to immunotherapy. Background Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an inflammatory variant of cerebral amyloid angiopathy (CAA). The current management of CAA-ri is uncertain but there is accumulating evidence that immunosuppressive treatment plays a role on the management of this condition. Design/Methods Patients were diagnosed with CAA-ri based on published criteria. We included consecutive cases seen in neurology clinics at our institution from April 12th, 2013, to March 1st, 2024. Clinical information was obtained by chart review. Exclusion criteria included patients with possible CAA-ri and patients with insufficient information. Results 19 subjects were included, 12 females and with mean age of 68 (SD 8.77) years old. The most frequent presenting symptoms were cognitive/behavioral issues (15/19), headache (9/19), focal neurologic deficits (8/19) and seizure (4/19). Every patient was treated with steroids. 13/19 were started on steroid sparing agents, 6 on cyclophosphamide (CYC) and 7 on mycophenolate (MYC). Of the 11/19 patients that had recurrence of symptoms, 2 were on steroid sparing agents (2 vs 9, p = 0.31). Escalation of therapy with a second- and third-line agent was used on 5/19 patients. 2/19 patients had age < 60 years-old and both had multiple recurrences. Due to contra-indication to MYC, one of them was treated with RTX without success. 13/19 subjects reported headache alleviation and recovered cognitive function, 7/19 had either less frequent seizure episodes, or complete resolution of seizures, and 5/19 had improved focal motor deficits. Conclusions We had too small of a sample to discuss the effectiveness of different agents and long-term use of steroid sparing agent was associated with lower recurrence rate but it was not statistically significant. Limitations of our study include small sample, retrospective design, selection bias (patients were seen at the neuroimmunology clinic at a tertiary center).
Source: Neurology
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4403206719