<p>Inflammatory cerebral amyloid angiopathy (CAA-RI) is a variant of CAA, rarely seen in young individuals with Down syndrome (DS). We describe a 32-year-old female with DS who developed a systemic inflammatory <b>state</b> followed by acute neurological symptoms, including seizures and hemiparesis. Brain MRI disclosed asymmetric T2/FLAIR hyperintensities with leptomeningeal enhancement and focal superficial siderosis on susceptibility-weighted imaging, without microbleeds; cerebrospinal fluid showed marked inflammation. After partial improvement with corticosteroids, rituximab was started due to persistent symptoms. This case highlights CAA-RI as a cause of acute decline in DS and supports the use of rituximab in refractory cases.</p>

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Probable cerebral amyloid angiopathy - related inflammation in a 32-year-old woman with down syndrome

  • Thales Pardini Fagundes,
  • Gustavo Maximiano Alves,
  • Mateus Gustavo Favaro,
  • Joyce Yuri Silvestre Yamamoto,
  • Lucas Ravagnani Silva,
  • Lucas Giansante Abud,
  • Jorge Alberto Martins Pentiado Junior

摘要

Inflammatory cerebral amyloid angiopathy (CAA-RI) is a variant of CAA, rarely seen in young individuals with Down syndrome (DS). We describe a 32-year-old female with DS who developed a systemic inflammatory state followed by acute neurological symptoms, including seizures and hemiparesis. Brain MRI disclosed asymmetric T2/FLAIR hyperintensities with leptomeningeal enhancement and focal superficial siderosis on susceptibility-weighted imaging, without microbleeds; cerebrospinal fluid showed marked inflammation. After partial improvement with corticosteroids, rituximab was started due to persistent symptoms. This case highlights CAA-RI as a cause of acute decline in DS and supports the use of rituximab in refractory cases.