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Abstract Details

Characterization of Clinical Course and Response to Treatment on Cerebral Amyloid Angiopathy-Related Inflammation
Autoimmune Neurology
P1 - Poster Session 1 (12:00 PM-1:00 PM)
055
Characterize response to immunotherapy. 
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. 
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.  

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. 

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). 

Authors/Disclosures
Bruna Leles Vieira de Souza, MD (Work)
PRESENTER
Miss Leles Vieira de Souza has nothing to disclose.
Lucas Horta, MD Dr. Horta has nothing to disclose.
Bart Chwalisz, MD (Massachusetts General Hospital, Department of Neurology) Dr. Chwalisz has nothing to disclose.