Background and Objectives The goal of this work was to investigate the natural history and outcomes after treatment for spontaneous amyloid-related imaging abnormalities (ARIA)-like in cerebral amyloid angiopathy–related inflammation (CAA-ri). Methods This was a multicenter, hospital-based, longitudinal, prospective observational study of inpatients meeting CAA-ri diagnostic criteria recruited through the Inflammatory Cerebral Amyloid Angiopathy and Alzheimer9s Disease βiomarkers International Network from January 2013 to March 2017. A protocol for systematic data collection at first-ever presentation and at subsequent in-person visits, including T1-weighted, gradient recalled echo–T2*, fluid-suppressed T2-weighted (fluid-attenuated inversion recovery), and T1 postgadolinium contrast-enhanced images acquired on 1.5T MRI, was used at the 3-, 6-, 12-, and 24-month follow-up. Centralized reads of MRIs were performed by investigators blinded to clinical, therapeutic, and time-point information. Main outcomes were survival, clinical and radiologic recovery, intracerebral hemorrhage (ICH), and recurrence of CAA-ri. Results The study enrolled 113 participants (10.6% definite, 71.7% probable, and 17.7% possible CAA-ri). Their mean age was 72.9 years; 43.4% were female; 37.1% were APOEε4 carriers; 36.3% had a history of Alzheimer disease; and 33.6% had a history of ICH. A history of ICH and the occurrence of new ICH at follow-up were more common in patients with cortical superficial siderosis at baseline (52.6% vs 14.3%, p < 0.0001 and 19.3% vs 3.6%, p < 0.009, respectively). After the first-ever presentation of CAA-ri, 70.3% (95% confidence interval [CI] 61.6%–78.5%) and 84.1% (95% CI 76.2%–90.6%) clinically recovered within 3 and 12 months, followed by radiologic recovery in 45.1% (95% CI 36.4%–54.8%) and 77.4% (95% CI 67.7%–85.9%), respectively. After clinicoradiologic resolution of the first-ever episode, 38.3% (95% CI 22.9%–59.2%) had at least 1 recurrence within the following 24 months. Recurrence was more likely if IV high-dose corticosteroid pulse therapy was suddenly stopped compared to slow oral tapering off (hazard ratio 4.68, 95% CI 1.57–13.93; p = 0.006). Discussion These results from the largest longitudinal cohort registry of patients with CAA-ri support the transient and potentially relapsing inflammatory nature of the clinical-radiologic acute manifestations of the disease and the effectiveness of slow oral tapering off after IV corticosteroid pulse therapy in preventing recurrences. Our results highlight the importance of differential diagnosis for spontaneous ARIA-like events in β-amyloid–driven diseases, including treatment-related ARIA in patients with Alzheimer disease exposed to immunotherapy drugs.

We peformed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes’ tremor and palatal tremor. The frequencies of these movement disorders were 4 Hz and 3 Hz, respectively. Vim thalamotomy stopped the Holmes’ tremor but not the palatal tremor. Our observations suggest different mechanisms for these two involuntary movements. A 57-arm 11 months after a pontine hemorrhage. Transoral carotid ultrasonography revealed periodic motion of her posterior pharyngeal wall with a frequency of 3 Hz. Recording of neuronal activities in the thalamus revealed a 4Hz rhythmic discharge time that was associated with her tremor in the contralateral arm. A left Vim thalamotomy was performed. The resting tremor of the upper limb stopped, but the kinetic tremor recurred 6 months after the thalamotomy. No effect was observed on her palatal tremor. The different effects of Vim thalamotomy on the Holmes’ tremor and palatal tremor suggest different oscillation sources for these two involuntary movements.

Aim: In-stent intimal hyperplasia (ISH) observed after carotid artery stenting (CAS) may lead to in-stent restenosis. We aimed to investigate whether contrast-enhanced carotid ultrasonography (CEUS) and magnetic resonance imaging (MRI) plaque imaging prior to CAS are predictive for ISH at 6 months after CAS.Material and method: A total of 14 patients (13 men, 1 woman; mean age, 74.2 years) were prospectively enrolled. CEUS and MRI plaque imaging were performed before CAS. ISH was diagnosed by carotid angiography at 6 months after CAS. Patients were divided into two groups based on the thicknessof ISH and age, risk factors, enhancement in CEUS, MRI plaque imaging and number of replaced stents were compared between groups.Results: Carotid angiography at 6 months after CAS revealed ISH in 10 patients. Plaque enhancement on CEUS was observed in 6 patients, all of whom showed ISH. A significant association was seen between plaque enhancement on CEUS and development of ISH (χ2 test, CEUS enhancement (+) 100% vs. CEUS enhancement (-) 50% p=0.040). Carotid plaques in 12 patients were diagnosed as unstable by MRI plaque imaging. Presence of ISH was significantly associated with unstable plaque diagnosed by MRI plaque imaging (χ2 test, unstable 83% vs. stable 0%; p=0.016).Conclusion: Carotid plaque MRI and CEUS may be useful to predict ISH after CAS.