Dementia in hereditary cerebral hemorrhage with amyloidosis-Dutch type is associated with cerebral amyloid angiopathy but is independent of plaques and neurofibrillary tangles

Background: Mild cognitive impairment (MCI) is considered by some to be a prodromal phase of a progressive disease (i.e., neurodegeneration) resulting in dementia; however, a substantial portion of individuals (ranging from 5–30%) remain cognitively stable over the long term (sMCI). The etiology of sMCI is unclear but may be linked to cerebrovascular disease (CVD), as evidence from longitudinal studies suggest a significant proportion of individuals with vasculopathy remain stable over time. Objective: To quantify the presence of neurodegenerative and vascular pathologies in individuals with long-term (>5-year) sMCI, in a preliminary test of the hypothesis that CVD may be a contributor to non-degenerative cognitive impairment. We expect frequent vasculopathy at autopsy in sMCI relative to neurodegenerative disease, and relative to individuals who convert to dementia. Methods: In this retrospective study, using data from the National Alzheimer’s Coordinating Center, individuals with sMCI (n = 28) were compared to those with MCI who declined over a 5 to 9-year period (dMCI; n = 139) on measures of neurodegenerative pathology (i.e., Aβ plaques, neurofibrillary tangles, TDP-43, and cerebral amyloid angiopathy) and CVD (infarcts, lacunes, microinfarcts, hemorrhages, and microbleeds). Results: Alzheimer’s disease pathology (Aβ plaques, neurofibrillary tangles, and cerebral amyloid angiopathy) was significantly higher in the dMCI group than the sMCI group. Microinfarcts were the only vasculopathy associated with group membership; these were more frequent in sMCI. Conclusion: The most frequent neuropathology in this sample of long-term sMCI was microinfarcts, tentatively suggesting that silent small vessel disease may characterize non-worsening cognitive impairment.

Download Full-text

Cerebral Amyloid Angiopathy Associated with Cerebral Hemorrhage with the Deposition of Cystatin C: Study on Japanese Cases

Amyloid and Amyloidosis 1990 ◽

10.1007/978-94-011-3284-8_92 ◽

1991 ◽

pp. 365-368 ◽

Cited By ~ 1

Author(s):

S. Fujihara ◽

K. Shimode ◽

M. Nakamura ◽

S. Kobayashi ◽

T. Tsunematsu ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Cystatin C ◽

Cerebral Hemorrhage ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

Download Full-text

Cerebral amyloid angiopathy: incidence and complications in the aging brain. I. Cerebral hemorrhage.

Stroke ◽

10.1161/01.str.14.6.915 ◽

1983 ◽

Vol 14 (6) ◽

pp. 915-923 ◽

Cited By ~ 126

Author(s):

J J Gilbert ◽

H V Vinters

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Cerebral Hemorrhage ◽

Aging Brain ◽

Amyloid Angiopathy ◽

Cerebral Amyloid

Download Full-text

Surgical Considerations in Cerebral Amyloid Angiopathy

Neurosurgery ◽

10.1227/00006123-199111000-00012 ◽

1991 ◽

Vol 29 (5) ◽

pp. 712-718 ◽

Cited By ~ 36

Author(s):

Richard Leblanc ◽

Mark Preul ◽

Yves Robitaille ◽

Jean-Guy Villemure ◽

Ronald Pokrupa

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Clinical Condition ◽

Cerebral Hemorrhage ◽

Good Condition ◽

Postoperative Hemorrhage ◽

Amyloid Angiopathy ◽

Amyloid Beta Protein ◽

Mortality And Morbidity ◽

Cerebral Amyloid ◽

Cerebral Hematoma

Abstract In cerebral amyloid angiopathy, the contractile elements of the leptomeningeal and cortical arteries are replaced by noncontractile amyloid beta protein. The incidence of amyloid angiopathy increases with advancing age. It is associated with Alzheimer's disease and spontaneous cerebral hemorrhage. The latter can have the characteristic acute computed tomographic appearance of a hematoma at the cortex-white matter junction with extension of blood into the subarachnoid, subdural, and intraventricular spaces. Multiple hemorrhages are frequent. Additional bleeding can occur after evacuation of the hematoma, and postoperative hemorrhage can occur after cortical biopsy. To elucidate the role of surgery in this condition, we have reviewed 20 consecutive operated cases of cerebral amyloid angiopathy. A first group of 8 patients with senila dementia underwent cortical biopsy without resultant hemorrhage. A second group of 6 patients in good clinical condition had delayed evacuation of a spontaneous cerebral hematoma from cerebral amyloid angiopathy because of the radiological misdiagnosis of a hemorrhage within a tumor. One patient died of a pulmonary embolism, and another had subsequent multiple hemorrhages that were ultimately fatal. A third group of 6 patients in poor neurological condition had the acute evacuation of a spontaneous cerebral hematoma to relieve intracranial hypertension. All died or were severely disabled. One had repeated hemorrhages which added a progressively more severe organic dementia onto an initial hemiplegia. We conclude that acute evacuation of a spontaneous cerebral hematoma from amyloid angiopathy patients in poor clinical condition results in a high mortality and morbidity, that delayed surgery in patients in good condition is better tolerated, and that cortical surgery in the absence of a cerebral hemorrhage can be accomplished relatively safely with meticulous attention to hemostasis. The outcome of surgery is a function of both the severity of the cerebral amyloid angiopathy and the extent of the hemorrhage that it produces, especially as it involves multiple lobes and extends into the ventricles or subdural space. Survivors remain at risk for hemorrhages that are progressively more disabling.

Download Full-text