Inflammatory Cerebral Amyloid Angiopathy, Amyloid-β–Related Angiitis, and Primary Angiitis of the Central Nervous System

Moussaddy, Aimen; Levy, Ariel; Strbian, Daniel; Sundararajan, Sophia; Berthelet, France; Lanthier, Sylvain

doi:10.1161/strokeaha.115.010024

Cited by 60 publications

(57 citation statements)

References 5 publications

(7 reference statements)

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“…Amyloid beta-related angiitis appears to be closely related to CAA-I, although it has more vessel wall destruction 10. CAA-I typically shows perivascular inflammation 6 10.…”

Section: Discussionmentioning

confidence: 99%

Relapsing cerebral amyloid angiopathy-related inflammation: the wax and the wane

et al. 2017

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Cerebral amyloid angiopathy-related inflammation (CAA-I) is a rare variant of cerebral amyloid angiopathy (CAA). Its precise pathophysiology remains uncertain and we currently have limited evidence on which immunosuppressive agents are the most effective in its treatment. The disease course of CAA-I disorders can vary from an isolated clinical event to recurrent episodes. We present a case of biopsy-confirmed CAA-I that gives insight into its potential relapsing nature and the challenges of its long-term management.

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“…Amyloid beta-related angiitis appears to be closely related to CAA-I, although it has more vessel wall destruction 10. CAA-I typically shows perivascular inflammation 6 10.…”

Section: Discussionmentioning

confidence: 99%

Relapsing cerebral amyloid angiopathy-related inflammation: the wax and the wane

et al. 2017

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“…Concurrent findings of CAA and perivascular inflammation have been designated as CAArelated inflammation (CAA-RI); when frank vessel wall inflammation with fibrinoid necrosis is present (as in our case), the term ABRA is used. 1,2 The average age at CAA-RI and ABRA onset is >50 years, which is older than in primary angiitis of the CNS but younger than in CAA. 3,4 The clinical presentation varies significantly between patients, but generally includes some combination of subacute cognitive or functional decline over a period of several months, new onset of seizure-like episodes, recurring or persistent headaches, perceptual disturbances such as hallucinations, or neurologic deficits such as visual disturbance or hemiparesis.…”

Section: Discussionmentioning

confidence: 99%

Clinical Reasoning: An 81-year-old woman with decreased consciousness and fluctuating right facial droop

et al. 2020

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An 81-year-old woman presented to the hospital after a week of intermittent episodes of decreased level of consciousness, right facial droop, and slurred speech, lasting approximately 30 minutes. Her medical history included chronic obstructive pulmonary disease, dyslipidemia, orthostatic hypotension, and 2 previous TIAs. She had fallen once in the preceding week, presumably as a result of her drowsiness. On examination, she was afebrile with a heart rate of 81 bpm and a blood pressure of 108/65 mm Hg. She was alert and cooperative, but disoriented to the year and location. Language examination was normal. Cranial nerves, motor, coordination, and sensory examinations were all normal without any focal deficits. However, when assessed during one of her episodes, she was less responsive, had decreased verbal output, and was noted to have a right facial droop. Basic serologic tests (complete blood count, electrolytes, renal function tests) were unremarkable. A head CT showed bilateral subarachnoid hemorrhage over the cerebral hemispheres without further abnormality. Questions for consideration: 1. What is your differential diagnosis? 2. Which investigations would you perform at this point? GO TO SECTION 2

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“…In humans, idiopathic CAA-related inflammation is a rare disorder [36, 37] that manifests in various ways [38] and has been given a number of monikers, such as Aβ-related angiitis (ABRA), cerebral amyloid inflammatory vasculopathy, and others [39]. CAA-related inflammation in humans involves the incursion of inflammatory cells [36], and in many (but not all) cases, CAA-related inflammation responds favorably to immunosuppressive agents [36, 39, 40].…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, the timecourse of changes can be systematically monitored in the model, including behavioral proficiency and potential biomarkers in the CSF and blood. The model may also aid in determining why some ARIA patients suffer from significant cognitive impairment following treatment with anti-inflammatory/immunosuppressive agents [38, 41–44]. …”

Section: Discussionmentioning

confidence: 99%

Amyloid-Related Imaging Abnormalities in an Aged Squirrel Monkey with Cerebral Amyloid Angiopathy

Heuer

Jacobs

et al. 2017

JAD

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Amyloid-related imaging abnormalities (ARIA) in magnetic resonance imaging scans have emerged as indicators of potentially serious side effects in clinical trials of therapeutics for Alzheimer’s disease. These anomalies include an edematous type (ARIA-E) that appears as hyperintense (bright) regions by T2-weighted MRI, and a type characterized by the deposition of hemosiderin (ARIA-H) that elicits a hypointense signal, especially in T2* and susceptibility-weighted imaging. ARIA in general has been linked to the presence of Aβ-type cerebral amyloid angiopathy, an accumulation of misfolded Aβ protein in the vascular wall that impairs the integrity of brain blood vessels. However, the pathobiology of ARIA remains poorly understood, in part due to the absence of an animal model of the disorder that would enable a contemporaneous analysis of tissue integrity in the affected region. Here we describe both ARIA-E and ARIA-H in an aged squirrel monkey (Saimiri sciureus), a nonhuman primate model of naturally occurring cerebral amyloid angiopathy. Histopathologic examination of the anomalous region revealed reactive astrocytosis and microgliosis, infiltration of systemic inflammatory/immune cells, damage to axons and myelin, and hemosiderin deposition. The disruption of axons in particular suggests that ARIA-E could have functional consequences for affected regions. The squirrel monkey model can be useful for studying the pathogenesis and long-term effects of ARIA, and for testing the safety and efficacy of emerging therapies for Alzheimer’s disease.

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Inflammatory Cerebral Amyloid Angiopathy, Amyloid-β–Related Angiitis, and Primary Angiitis of the Central Nervous System

Cited by 60 publications

References 5 publications

Relapsing cerebral amyloid angiopathy-related inflammation: the wax and the wane

Relapsing cerebral amyloid angiopathy-related inflammation: the wax and the wane

Clinical Reasoning: An 81-year-old woman with decreased consciousness and fluctuating right facial droop

Amyloid-Related Imaging Abnormalities in an Aged Squirrel Monkey with Cerebral Amyloid Angiopathy

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