Spontaneous intracerebral haemorrhage associated with early-onset cerebral amyloid angiopathy and Alzheimer’s disease neuropathological changes five decades after cadaveric dura mater graft

Acta Neuropathologica Communications

Case Report

Giorgio Giaccone¹  Elisa Pellencin¹  Nicole Campanella¹  Marcella Catania¹  Emanuela Maderna¹  Giuseppe Di Fede¹  Emanuele Dainese²  Andrea Salmaggi²  Lucio Aniello Mazzeo²  Ilaria Salemi²  Daniela Vismara²  Riccardo Milani² 

[1] Fondazione IRCCS Istituto Neurologico Carlo Besta, Unit of Neurology 5 - Neuropathology, Milan, Italy;Ospedale Alessandro Manzoni, ASST di Lecco, Lecco, Italy;

关键词: Cerebral amyloid angiopathy;    Alzheimer’s disease;    Iatrogenic;    Neurosurgery;   

DOI  :  10.1186/s40478-023-01528-7

 received in 2022-12-22, accepted in 2023-02-11,  发布年份 2023

来源: Springer

【 摘 要 】

Cerebral amyloid angiopathy (CAA) is a small vessel disease, causing spontaneous intracerebral hemorrhage (ICH) in the elderly. It is strongly associated with Alzheimer disease (AD), as most CAA patients show deposition of Aβ—i.e. the basic component of parenchymal Alzheimer amyloid deposits—in the cerebral vessels. Iatrogenic early-onset CAA has been recently identified in patients with a history of traumatic brain injury or other cerebral as well as extra-cerebral lesions that led to neurosurgery or other medical procedures as intravascular embolization by cadaveric dura mater extracts many years before the first ICH event. In those patients, a transmission of Aβ seeds from neurosurgical instruments or from cadaveric dura mater exposure was suggested. We report a 51-year-old woman with unremarkable family history who presented abruptly with aphasia and right hemiparesis. A cerebral left lobar haemorrhagic stroke was documented by neuroimaging. Accurate anamnesis revealed a neurosurgical procedure with cadaveric dura mater graft at the age of 2 years for an arachnoid cyst. The neuropathological examination of the cerebral parietal biopsy showed severe amyloid angiopathy in many leptomeningeal and cortical vessels, as well as abundant parenchymal Aβ deposits, neurofibrillary tangles and neuropil threads. The mechanism involved in the human-to-human transmission of the Aβ proteinopathy remains to be clarified. In our patient the cadaver derived dura used for grafting is a very strong candidate as the source of the transmission. A systematic monitoring of individuals who have had neurosurgical procedures in early life, especially those involving cadaveric dural grafts, is required to determine the ratio of those affected by CAA many years later and unaffected. Moreover, our report confirms that in addition to vascular and parenchymal Aβ pathology, neurofibrillary changes indistinguishable from AD may develop in specific conditions with long latency period from the neurosurgical or embolization procedure.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

Fig. 2