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Xuất huyết não di truyền kèm amyloidosis (Hà Lan): mô hình hình thành mảng bám congophilic không có bệnh lý sợi thần kinh
Tóm tắt
Các tổn thương giống như mảng bám và bệnh lý mạch máu liên quan đến amyloid đã được nghiên cứu trong vỏ não trán của bốn bệnh nhân mắc chứng xuất huyết não di truyền kèm amyloidosis (HCHWA-D), bằng phương pháp nhuộm hoá miễn dịch [kháng thể đối với protein amyloid β (Aβ), tiền chất protein β (βPP), synaptophysin, ubiquitin (UBQ), cathepsin D, các sợi xoắn đôi (PHF) và protein axit gian thần kinh (GFAP)], phương pháp nhuộm enzym (phosphatase acid) và nhuộm bạc [nitrate methenamine bạc (MS) và Palmgren]. Trong khi các mảng bám lan rộng dương tính với Aβ và MS được tìm thấy ở tất cả bệnh nhân, thì chỉ có ba bệnh nhân lớn tuổi hơn mới cho thấy mảng bám thần kinh hoặc congophilic, với dấu hiệu dương tính với phosphatase acid và cathepsin D, chứa các neurite có dấu hiệu dương tính với βPP, synaptophysin, và UBQ, nhưng lại âm tính với PHF. Những mảng bám này được bao quanh bởi các tế bào sao phản ứng. Các phản ứng miễn dịch và enzym tương tự cũng được phát hiện xung quanh các mạch máu congophilic. Như vậy, bên cạnh sự thoái hoá thần kinh trong một tập hợp các tổn thương giống như mảng bám và xung quanh các mạch máu, nghiên cứu này cho thấy hình thái học có liên quan đến tuổi tác của các mảng bám trong HCHWA-D tương ứng với trong hội chứng Down (DS), với sự khác biệt là bệnh lý sợi thần kinh (NF) vắng mặt trong HCHWA-D, trái lại với DS. HCHWA-D có thể được coi là một mô hình cho sự hình thành mảng bám congophilic không liên quan đến bệnh lý NF.
Từ khóa
#xuất huyết não di truyền #amyloidosis #mảng bám congophilic #bệnh lý sợi thần kinh #nghiên cứu histopathologyTài liệu tham khảo
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