Cerebral amyloid angiopathy-related inflammation: current status and future implications

Abstract

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive; consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.

Probable Cerebral Amyloid Angiopathy–Related Inflammation Associated With Sitravatinib

“…In our patient, premorbid CAA is suggested by elevated phosphorylated tau to amyloid beta 42 ratio in CSF and histopathologic evidence of vascular amyloid deposition, whereas perivascular inflammation likely represents an autoimmune response to the presence of amyloid. 6 Development of CAA-ri has been previously reported in the setting of checkpoint inhibition, 7 although interestingly our patient is thus far tolerating pembrolizumab and ipilimumab/nivolumab without evidence of disease recurrence. Further studies are needed to define the precise role, if any, of RTKs, PD-1, and CTLA-4 in the pathophysiology of CAA-ri.…”

Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation

“…Clinical and neuroradiological differential diagnoses of CAA-ri include posterior reversible encephalopathy syndrome, reversible cerebral vasoconstriction syndrome, mitochondrial encephalopathy, lactic acidosis, stroke-like syndrome, Varicella Zoster virus and other infectious vasculitides, sarcoidosis, systemic amyloidosis, giant cell arteritis, primary angiitis of the CNS, and vascular malformations [ 18 , 19 ]. Patient’s past medical history, clinical presentation, blood and CSF analysis, and neuroradiological findings at brain MRI generally help to achieve a reliable diagnosis.…”

Secondary Headache Disorders: Approach, Workup, and Special Considerations for Select Populations

“…Other more commonly prescribed medications that can cause secondary headaches include calcium channel antagonists, corticosteroids, dipyridamole, estrogen, histamine receptor antagonists, hydralazine, nonsteroidal anti-inflammatory drugs, nitrates, phosphodiesterase inhibitors, sympathomimetics, tamoxifen, tetracyclines, and trimethoprim. 48 This list is not exhaustive and thorough medication reconciliation is a critical part of the workup of headaches, especially as patients age.…”