Analysis on two cases of cerebral amyloid angiopathy-related inflammation

Abstract

Objective To study the clinical features, auxiliary examination, diagnosis and treatment, and prognosis of cerebral amyloid angiopathy-related inflammation (CAA-ri). Methods and Results Two elderly female cases with CAA-ri during February 2017 to February 2018 in our hospital were reported. Both of them manifested with cognitive impairment, gait abnormality and decreased daily life ability. Lumbar puncture of 2 cases showed the pressure of cerebrospinal fluid (CSF) was normal, white blood cell (WBC) count was generally normal and protein quantification was increased (988 and 975 mg/L). One case presented decreased CSF amyloid-β protein 40 (A β₄₀) and 42 (A β₄₂), and the other one had decreased Aβ₄₂ . One case was apolipoprotein E (ApoE) ε3/ε3, and the other was ε3/ε4. Brain MRI showed long T₁ and long T₂ abnormal signals with flaky fusion, and susceptibility-weighted imaging (SWI) showed multiple cortical and subcortical microhemorrhage. They were diagnosed as CAA-ri, and the symptoms were obviously improved after methylprednisolone implosive sequential therapy. Conclusions In addition to cognitive impairment, gait abnormality can also be the main clinical manifestation of CAA-ri. Typical imaging changes are white matter hyperintensity on T₂WI and FLAIR fusion sequences, with multiple cortical and subcortical hemorrhagic lesions on SWI. Once the typical clinical and imaging changes meet the diagnostic criteria, the diagnosis of CAA-ri can be made without biopsy. Immunotherapy is effective for most patients.

DOI: 10.3969/j.issn.1672-6731.2019.01.011