Reversible acute leukoencephalopathy as a form of presentation in cerebral amyloid angiopathy

Cano, Luis; Martínez-Yélamos, Sergio; Majós, Carles; Alberti, M.; Boluda, Susana; Velasco, Roser; Rubio, Francisco

doi:10.1016/j.jns.2009.09.035

Cited by 11 publications

(6 citation statements)

References 7 publications

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“…Acute reversible leucencephalopathy is a rare manifestation of CAA, clinically presenting with rapid progression of neurological symptoms followed by clinical and radiographic improvement. In these cases neuropathological examination showed CAA to be associated with varying degrees of inflammation [116,117].…”

Section: Cerebral Infarction and White Matter Lesionsmentioning

confidence: 93%

Review: Sporadic cerebral amyloid angiopathy

Attems

Jellinger

Thal

et al. 2011

Neuropathology Appl Neurobio

300

295

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Cerebral amyloid angiopathy (CAA) may result from focal to widespread amyloid-β protein (Aβ) deposition within leptomeningeal and intracortical cerebral blood vessels. In addition, pericapillary Aβ refers to Aβ depositions in the glia limitans and adjacent neuropil, whereas in capillary CAA Aβ depositions are present in the capillary wall. CAA may cause lobar intracerebral haemorrhages and microbleeds. Hypoperfusion and reduced vascular autoregulation due to CAA might cause infarcts and white matter lesions. CAA thus causes vascular lesions that potentially lead to (vascular) dementia and may further contribute to dementia by impeding the clearance of solutes out of the brain and transport of nutrients across the blood brain barrier. Severe CAA is an independent risk factor for cognitive decline. The clinical diagnosis of CAA is based on the assessment of associated cerebrovascular lesions. In addition, perivascular spaces in the white matter and reduced concentrations of both Aβ(40) and Aβ(42) in cerebrospinal fluid may prove to be suggestive for CAA. Transgenic mouse models that overexpress human Aβ precursor protein show parenchymal Aβ and CAA, thus corroborating the current concept of CAA pathogenesis: neuronal Aβ enters the perivascular drainage pathway and may accumulate in vessel walls due to increased amounts and/or decreased clearance of Aβ, respectively. We suggest that pericapillary Aβ represents early impairment of the perivascular drainage pathway while capillary CAA is associated with decreased transendothelial clearance of Aβ. CAA plays an important role in the multimorbid condition of the ageing brain but its contribution to neurodegeneration remains to be elucidated.

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Section: Cerebral Infarction and White Matter Lesionsmentioning

confidence: 93%

Review: Sporadic cerebral amyloid angiopathy

Attems

Jellinger

Thal

et al. 2011

Neuropathology Appl Neurobio

300

295

View full text Add to dashboard Cite

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“…The detection of hemosiderin deposition 2 , 5 , 12 ) due to the presence of intracerebral microbleeding and/or white matter lesions 7 , 8 ) on head MRI is useful for confirming the diagnosis of CAA. Approximately half of CAA cases are accompanied by subcortical hemorrhage or microbleeding 13 ) , and CAA patients usually have significantly more lesions in the white matter than healthy individuals 14 ) .…”

Section: Discussionmentioning

confidence: 99%

“…CAA-derived cerebral hemorrhage 1 , 2 ) tends to occur in the subcortical region, both temporally and spatially, in multiple ways. Recently, a complex clinical condition involving subarachnoid hemorrhage and/or multiple episodes of microbleeding in the deep white matter has been reported 3 , 4 , 5 , 6 ) , and white matter lesions have been identified on head MRI imaging examinations 7 , 8 ) . We experienced a case of localized subarachnoid hemorrhage that developed at the fornix.…”

Section: Introductionmentioning

confidence: 99%

Cerebral amyloid angiopathy with atypical imagingfindings of subarachnoid hemorrhage

2015

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Cerebral amyloid angiopathy (CAA) is observed in most cases of nonhypertensive subcortical hemorrhage involving elderly patients. We herein describe the case of a female in whom a convexal subarachnoid hemorrhage was observed at 55 years of age. The cerebral hemorrhage occurred repeatedly; however, no obvious vascular lesions were observed on a cerebral angiography, and no signs of microbleeding or lesions in the deep white matter were identified on magnetic resonance imaging (MRI). Partial excision of the right frontal cortex and hematoma evacuation were performed, and histopathological examination showed deposition of an acidophilic substance with positive staining for Direct Fast Scarlet (DFS) in the cerebral vascular wall. Finally, brain hemorrhage due to CAA was diagnosed. This case suggests that CAA is an important differential diagnosis in patients with localized non-aneurysmal subarachnoid hemorrhage in the convexity sulcus.

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“…However, in vivo image analytical methods have not as yet been developed for evaluating the development and relative contribution of CAA and parenchymal amyloid deposition to overall amyloid burden in AD brains studied longitudinally. CAA lesions can be found in 80 – 85% of post-mortem brains of patients with AD and Down’s Syndrome, and extensive CAA is seen in approximately ½ of AD patients (9). It is possible that both CAA and parenchymal amyloid contribute to the clinical AD progression (49).…”

Section: Critical Barriers In Longitudinal Pet Imagingmentioning

confidence: 99%

Longitudinal Positron Emission Tomography in Preventive Alzheimer's Disease Drug Trials, Critical Barriers from Imaging Science Perspective

Shokouhi¹,

Campbell²,

Brill³

et al. 2016

Brain Pathology

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Recent Alzheimer’s trials have recruited cognitively normal people at risk for Alzheimer’s dementia. Due to the lack of clinical symptoms in normal population, conventional clinical outcome measures are not suitable for these early trials. While several groups are developing new composite cognitive tests that could serve as potential outcome measures by detecting subtle cognitive changes in normal people, there is a need for longitudinal brain imaging techniques that can correlate with temporal changes in these new tests and provide additional objective measures of neuropathological changes in brain. Positron emission tomography (PET) is a nuclear medicine imaging procedure based on the measurement of annihilation photons after positron emission from radiolabeled molecules that allow tracking of biological processes in body, including the brain. PET is a well-established in vivo imaging modality in Alzheimer’s disease diagnosis and research due to its capability of detecting abnormalities in three major hallmarks of this disease. These include 1) amyloid beta plaques, 2) neurofibrillary tau tangles and 3) decrease in neuronal activity due to loss of nerve cell connection and death. While semi-quantitative PET imaging techniques are commonly used to set discrete cut-points to stratify abnormal levels of amyloid accumulation and neurodegeneration, they are sub-optimal for detecting subtle longitudinal changes. In this study, we have identified and discussed four critical barriers in conventional longitudinal PET imaging that may be particularly relevant for early Alzheimer’s disease studies. These include within and across subject heterogeneity of AD-affected brain regions, PET intensity normalization, neuronal compensations in early disease stages and cerebrovascular amyloid deposition.

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Reversible acute leukoencephalopathy as a form of presentation in cerebral amyloid angiopathy

Cited by 11 publications

References 7 publications

Review: Sporadic cerebral amyloid angiopathy

Review: Sporadic cerebral amyloid angiopathy

Cerebral amyloid angiopathy with atypical imagingfindings of subarachnoid hemorrhage

Longitudinal Positron Emission Tomography in Preventive Alzheimer's Disease Drug Trials, Critical Barriers from Imaging Science Perspective

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