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뇌 아밀로이드 혈관병증-관련염증 증례를 통해 분석한 뇌척수액 아밀로이드 베타 및 플로르베타벤 뇌 양전자 단층촬영 소견

Patterns of Cerebrospinal Fluid Biomarkers and Amyloid Positron Emission Tomography in a Patient with Cerebral Amyloid Angiopathy-Related Inflammation

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초록

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a distinct subset of cerebral amyloid angiopathy characterized by the auto-inflammatory response to amyloid-laden small arteries of cerebral cortex and leptomeninges. Clinical features include cognitive-behavioral change, headache, focal neurologic deficits and seizure. Because anti-inflammatory treatments can rapidly relieve neurologic symptoms, early diagnosis is critical. Herein, we report a CAA-RI case with distinct laboratory findings of a decreased cerebrospinal fluid amyloid beta 1-42 level and relatively reduced florbetaben uptake in the focal inflammatory lesion during the acute phase of CAA-RI.

Abstract

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a distinct subset of cerebral amyloid angiopathy characterized by the auto-inflammatory response to amyloid-laden small arteries of cerebral cortex and leptomeninges. Clinical features include cognitive-behavioral change, headache, focal neurologic deficits and seizure. Because anti-inflammatory treatments can rapidly relieve neurologic symptoms, early diagnosis is critical. Herein, we report a CAA-RI case with distinct laboratory findings of a decreased cerebrospinal fluid amyloid beta 1-42 level and relatively reduced florbetaben uptake in the focal inflammatory lesion during the acute phase of CAA-RI.

참고문헌 (10)

  1. Chung KK, "Cerebral amyloid angiopathy related inflammation: three case reports and a review", J Neurol Neurosurg Psychiatry, 82, pp.20~26 (2011)
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  6. Piazza F, "Anti-amyloid β autoantibodies in cerebral amyloid angiopathy-related inflammation: implications for amyloid-modifying therapies", Ann Neurol, 73, pp.449~458 (2013)
  7. Boncoraglio GB, "Prodromal Alzheimer's disease presenting as cerebral amyloid angiopathy-related inflammation with spontaneous amyloid-related imaging abnormalities and high cerebrospinal fluid anti-Aβ autoantibodies", J Alzheimers Dis, 45, pp.363~367 (2015)
  8. Sengoku R, "11C-PiB PET imaging of encephalopathy associated with cerebral amyloid angiopathy", Intern Med, 53, pp.1997~2000 (2014)
  9. Carmona-Iragui M, "Cerebrospinal fluid anti-amyloid-beta autoantibodies and amyloid PET in cerebral amyloid angiopathy-related inflammation", J Alzheimers Dis, 50, pp.1~7 (2016)
  10. Ostrowitzki S, "Mechanism of amyloid removal in patients with Alzheimer disease treated with gantenerumab", Arch Neurol, 69, pp.198~207 (2012)

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