Cerebral Amyloid Angiopathy-related Inflammation (CAA-ri): A Case Report

Overview

Journal Medicine (Baltimore)

Specialty General Medicine

Date 2024 Dec 20

PMID 39705440

Authors

Yao Meng

Yining Xiao

Ruohan Sun

Lingyu Li

Yanhong Dong

Affiliations

Soon will be listed here.

Abstract

Rationale: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a treatable condition characterized by an acute or subacute onset, with its primary pathological hallmark being the deposition of amyloid, predominantly β-amyloid (Aβ), within intracranial microvessels. Despite its potential for treatment, CAA-ri is a rare disorder that is frequently underrecognized by clinicians in practice. This article provides a comprehensive overview of the clinical manifestations and therapeutic approaches associated with CAA-ri, aiming to enhance awareness among healthcare professionals.

Patient Concerns: A 67-year-old male patient who suffered from a sudden decline in cognitive functioning, intermittent headache, and dysphoria underwent brain magnetic resonance imaging, susceptibility weighted imaging, and cerebrospinal fluid analysis and was considered probable CAA-ri.

Diagnosis Interventions: During the course of disease development, the patient suffered from a sudden decline in cognitive functioning, mainly in the form of unresponsiveness, decreased comprehension, and increased repetitive language, accompanied by intermittent headaches and dysphoria. Brain magnetic resonance imaging showed numerous white matter in both hemispheres. Susceptibility weighted imaging showed multiple spots of hypointensity in the bilateral cerebral and cerebellar hemispheres, a hypointensity signal in the left occipital lobe, and extensive zones of hypointensity in bilateral sulci. Cerebrospinal fluid analysis was abnormal with elevated levels of protein and low levels of P-tau, Aβ42, and Aβ1-42/Aβ1-40. The use of glucocorticoids greatly reduced his symptoms. This lends credence to the probable CAA-ri diagnosis. The symptoms can be successfully alleviated by administering methylprednisolone sodium succinate.

Outcomes: During the patient's hospitalization, immunosuppressive therapy, primarily consisting of methylprednisolone sodium succinate and methylprednisolone, was administered, resulting in a significant improvement in symptoms. Post-discharge, the patient was monitored regularly, revealing a gradual enhancement in cognitive function without recurrence. Consequently, immunosuppressive therapy was discontinued 1 year following the patient's discharge.

Lessons: CAA-ri is a rare clinical condition, and timely diagnosis and early treatment are very critical for patient prognosis.

References

Sanchez-Caro J, de Lorenzo Martinez de Ubago I, de Celis Ruiz E, Arribas A, Calviere L, Raposo N . Transient Focal Neurological Events in Cerebral Amyloid Angiopathy and the Long-term Risk of Intracerebral Hemorrhage and Death: A Systematic Review and Meta-analysis. JAMA Neurol. 2021; 79(1):38-47. PMC: 8593834. DOI: 10.1001/jamaneurol.2021.3989. View

Yang J, Chu Y, Tsai H, Jeng J . Amyloid and tau PET in cerebral amyloid angiopathy-related inflammation two case reports and literature review. Front Neurol. 2023; 14:1153305. PMC: 10175602. DOI: 10.3389/fneur.2023.1153305. View

Auriel E, Charidimou A, Gurol M, Ni J, van Etten E, Martinez-Ramirez S . Validation of Clinicoradiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation. JAMA Neurol. 2016; 73(2):197-202. DOI: 10.1001/jamaneurol.2015.4078. View

Liu Y, Xu S, Bian H, Qian Y, Li H, Shu S . Xingnaojing ameliorates synaptic plasticity and memory deficits in an Aβ induced mouse model of Alzheimer's disease. J Pharmacol Sci. 2020; 143(4):245-254. DOI: 10.1016/j.jphs.2020.05.002. View

Storti B, Gabriel M, Sennfalt S, Canavero I, Rifino N, Gatti L . Rare forms of cerebral amyloid angiopathy: pathogenesis, biological and clinical features of CAA-ri and iCAA. Front Neurosci. 2023; 17:1219025. PMC: 10363735. DOI: 10.3389/fnins.2023.1219025. View

Agarwal A, Gupta V, Brahmbhatt P, Desai A, Vibhute P, Joseph-Mathurin N . Amyloid-related Imaging Abnormalities in Alzheimer Disease Treated with Anti-Amyloid-β Therapy. Radiographics. 2023; 43(9):e230009. PMC: 10715974. DOI: 10.1148/rg.230009. View

Jakel L, de Kort A, Klijn C, Schreuder F, Verbeek M . Prevalence of cerebral amyloid angiopathy: A systematic review and meta-analysis. Alzheimers Dement. 2021; 18(1):10-28. PMC: 9290643. DOI: 10.1002/alz.12366. View

Chu S, Xu F, Su Y, Chen H, Cheng X . Cerebral Amyloid Angiopathy (CAA)-Related Inflammation: Comparison of Inflammatory CAA and Amyloid-β-Related Angiitis. J Alzheimers Dis. 2016; 51(2):525-32. DOI: 10.3233/JAD-151036. View

Regenhardt R, Thon J, Das A, Thon O, Charidimou A, Viswanathan A . Association Between Immunosuppressive Treatment and Outcomes of Cerebral Amyloid Angiopathy-Related Inflammation. JAMA Neurol. 2020; 77(10):1261-1269. PMC: 7309570. DOI: 10.1001/jamaneurol.2020.1782. View

10.

Theodorou A, Palaiodimou L, Malhotra K, Zompola C, Katsanos A, Shoamanesh A . Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis. Stroke. 2022; 54(1):178-188. DOI: 10.1161/STROKEAHA.122.040671. View

11.

Banerjee G, Samra K, Adams M, Jaunmuktane Z, Parry-Jones A, Grieve J . Iatrogenic cerebral amyloid angiopathy: an emerging clinical phenomenon. J Neurol Neurosurg Psychiatry. 2022; . DOI: 10.1136/jnnp-2022-328792. View

12.

Kawahara M . Neurotoxicity of β-amyloid protein: oligomerization, channel formation, and calcium dyshomeostasis. Curr Pharm Des. 2010; 16(25):2779-89. DOI: 10.2174/138161210793176545. View

13.

Yamashita T, Abe K . Update on Antioxidant Therapy with Edaravone: Expanding Applications in Neurodegenerative Diseases. Int J Mol Sci. 2024; 25(5). PMC: 10932469. DOI: 10.3390/ijms25052945. View

14.

Freund B, Sanchez-Boluarte S, Blackmon K, Day G, Lin M, Khan A . Incidence and risk factors associated with seizures in cerebral amyloid angiopathy. Eur J Neurol. 2023; 30(12):3682-3691. DOI: 10.1111/ene.15903. View

15.

Yamada M . Cerebral amyloid angiopathy and gene polymorphisms. J Neurol Sci. 2004; 226(1-2):41-4. DOI: 10.1016/j.jns.2004.09.009. View

16.

Wu J, Yao M, Ni J . Cerebral amyloid angiopathy-related inflammation: current status and future implications. Chin Med J (Engl). 2021; 134(6):646-654. PMC: 7990003. DOI: 10.1097/CM9.0000000000001427. View

17.

Danve A, Grafe M, Deodhar A . Amyloid beta-related angiitis--a case report and comprehensive review of literature of 94 cases. Semin Arthritis Rheum. 2014; 44(1):86-92. DOI: 10.1016/j.semarthrit.2014.02.001. View

18.

Kinnecom C, Lev M, WENDELL L, Smith E, Rosand J, Frosch M . Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007; 68(17):1411-6. DOI: 10.1212/01.wnl.0000260066.98681.2e. View

19.

Reid A, Maloney A . Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly mongol. Acta Neuropathol. 1974; 27(2):131-7. DOI: 10.1007/BF00687163. View

20.

Morley J, Farr S . The role of amyloid-beta in the regulation of memory. Biochem Pharmacol. 2014; 88(4):479-85. DOI: 10.1016/j.bcp.2013.12.018. View