Cerebral amyloid angiopathy-related inflammation and rapidly progressive dementia : first case report in Colombia. Acta Neurol Colomb. ISSN Cerebral amyloid angiopathy CAA is the deposition of amyloid in the wall of intracranial blood vessels, and leads to the appearance of hemorrhage, ischemia or leukoencephalopathy. The clinical manifestations of the CAA are highly variable, such as cognitive impairment, behavioral abnormalities, focal neurological deficits, headache or seizures.

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Cerebral amyloid angiopathy: a cross-sectional study in a single center in Northeastern Brazil. Until today, the prevalence of CAA is unknown in our region. This study aims to analyze the prevalence of this entity in a specific elderly population in a tertiary hospital in Northeastern Brazil. One hundred and seventy-four patients were enrolled, of whom were women Nine patients were excluded from the study due to unavailability of MRI sequences needed for an appropriate analysis.

Out of the remaining patients, 12 7. The prevalence of CAA in our study was like those of medical literature, with a progressive age-related increase. CAA mainly occur as a sporadic disorder in older people, but it can occur as a familial syndrome in young patients.

It is difficult to assess the exact prevalence of CAA in the general population, given that the definite diagnosis still requires the histopathological analysis of the brain. However, presumptive diagnosis in vivo has become possible with specific criteria due to the emerging interest in this disease and to the improve in neuroimaging techniques over the last decades 4 , especially in magnetic resonance imaging MRI sequences that allow the detection of blood-breakdown products before the occurrence of a symptomatic ICH 4 , 5.

Epidemiology of CAA is unknown in our region according to the literature review. This study aims to evaluate the prevalence of this condition in a specific population of elderly patients followed at the Neurology service of a tertiary hospital in Northeastern Brazil, with the diagnosis established based on the modified Boston criteria 6.

MRI scan had been performed, according to medical records, due to different indications, such as cognitive impairment, headache investigation, parkinsonism, previous history of stroke or transient ischemic attack and seizures.

Detailed medical history was collected with phone calls and medical records, and included complete demographic and clinical information, medication use, previous history of stroke, brain tumor, head injury or coagulopathy. Neuroimages were obtained in a 1. The diagnosis was established according to the modified Boston criteria Table 1. Table 1. Modified Boston Criteria for cerebral amyloid angiopathy. Full post-mortem examination demonstrating:. Clinical data and pathological tissue evacuated hematoma or cortical biopsy demonstrating:.

All participants signed the Informed Consent Form. The sample analysis was performed using the Shapiro-Wilk test. A total of patients were enrolled, of whom were women Mean age of patients diagnosed with CAA was similar to the average of the sample Of these, 9 5. Figure 1. Study profile. The presence of deep location CMB thalamus, basal ganglia or brainstem , evidenced in some patients, excluded the diagnosis of CAA Figure 4 based on the modified Boston criteria.

Figure 2. Figure 3. Disseminated cortical superficial siderosis CSS in a year-old female patient A-C and year-old male patient D-E ; F: A right lobar intracerebral hemorrhage white arrow in a year-old male patient, with two lobar cortical and cortical-subcortical location microbleeds arrowheads. Figure 4. Distribution of cerebral microbleeds CMB.

In these situations, diagnosis of cerebral amyloid angiopathy is excluded. Analysis by age group of patients included in the study showed that 71 of them were between 65 and 70 years old at the time of enrollment. Among this group, 2 2. On the other hand, 32 patients were in the age group between 71 and 75 years old, and 2 6. Lastly, 62 patients were over 75 years old, and 8 Table 2. Patients diagnosed with cerebral amyloid angiopathy according to Modified Boston Criteria, by age group.

By investigating radiological findings, the hemorrhagic alterations evidenced in MRI were focal or multiple cerebral microbleeds CMBs , lobar hemorrhages and focal or disseminated CSS Table 3. Table 3. Radiological findings in patients diagnosed with cerebral amyloid angiopathy. Regarding medication use in CAA diagnosed group, 5 None of the diagnosed patients were on anticoagulant use. Among patients diagnosed with CAA, there was no report of brain tumor, previous traumatic brain injury or coagulation disorder.

Incidence of CAA, like in Alzheimer's disease, progressively increases with age. Based on a series of autopsies, the prevalence of moderate to severe CAA was estimated at 2. Symptoms related to sporadic CAA are uncommon in patients younger than 60 years old, being evident in the genetic forms for younger age groups 7 , 8. Since the original description of neurovascular amyloid deposits reported in by Oppenheim 9 , there has been a marked advance in the understanding of CAA, particularly in recent decades.

Until the initial publication of the Boston criteria in the mids, the diagnosis of this condition could only be established by a histopathological analysis of the brain tissue, either with brain hematoma evacuation, biopsy or autopsy analysis. Since then, using a combination of clinical, neuroimaging and histopathological criteria, the Boston criteria established three levels of certainty for the diagnosis of CAA: definite, probable and possible. The diagnosis, therefore, could be reached in vivo , sometimes preceding the occurrence of a symptomatic ICH 10 , 11 , In , since the publication of the modified Boston criteria, there was the inclusion of CSS as a hemorrhagic lesion, increasing sensitivity without reducing specificity of the diagnosis original criteria had a sensitivity range from To date, there is no specific treatment or prevention strategy for CAA It is known that antithrombotic therapy with anticoagulant or antiplatelet medications increases mortality after an ICH and can be harmful if the hemorrhage is associated to the amyloid pathology 10 , Therefore, the use of these therapies must be individualized.

Regarding the use of statins, scientific data is still insufficient to recommend restrictions on the use of this class of medication until now This study evaluated 4, patients with a recent stroke ischemic or hemorrhagic , or TIA, and found an increased risk of recurrent ICH in those using high-dose atorvastatin 55 ICH vs 33 in the placebo group; HR 1. Our study has limitations due to its retrospective model and the type of sample analyzed, composed only of elderly patients followed in a specialized service of Neurology in a tertiary hospital, and therefore, the results cannot be expanded to the general population.

However, it serves as a basis for a better understanding on CAA in the local reality, encouraging interest in this pathology, drawing the attention of health professionals for its occurrence and assisting the decision-making process in the diagnosed cases. CAA is a vascular disorder that plays an important role as a cause of lobar ICH, particularly in the elderly population. Although it is not a rare condition, as the studies show, it is still an underdiagnosed disease.

The prevalence increases with age and the inadvertent use of medications such as antiplatelet agents and anticoagulants may be harmful and could contribute to a worse clinical outcome in these cases. The diagnosis of this condition can be defined in a simple and non-invasive way with clinical and neuroimaging data, particularly with the use of MRI sequences that allow the early detection of hemoglobin-breakdown products and with the application of well-established criteria in the literature.

Biffi A, Greenberg SM. Cerebral amyloid angiopathy: a systematic review. J Clin Neurol. Viswanathan A, Greenberg SM. Cerebral amyloid angiopathy in the elderly. Ann Neurol. Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry. Sporadic cerebral amyloid angiopathy: pathophysiology, neuroimaging features, and clinical implications.

Semin Neurol. Pantoni L. Cerebral small vessel disease: from pathogenesis and clinical characteristics to therapeutic challenges. Lancet Neurol. Greenberg SM, Charidimou A. Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Cerebral amyloid angiopathy research: on the verge of an explosion?

Int J Stroke. Diagnosis of cerebral amyloid angiopathy. Sensitivity and specificity of cortical biopsy. Oppenheim G. Neurol Centralbl. Advances in our understanding of the pathophysiology, detection and management of cerebral amyloid angiopathy. Eur Neurol Rev. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Cerebral amyloid angiopathy without and with cerebral hemorrhages: a comparative histological study. Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy.

Can patients be anticoagulated after intracerebral hemorrhage? A decision analysis. Cerebral microbleeds are a risk factor for warfarin-related intracerebral hemorrhage. Risk vs benefit of anti-thrombotic therapy in ischaemic stroke patients with cerebral microbleeds.

J Neurol. Long-term prognosis after intracerebral haemorrhage: systematic review and meta-analysis. Hemorrhagic stroke in the stroke prevention by aggressive reduction in cholesterol levels study.

The increasing impact of cerebral amyloid angiopathy: essential new insights for clinical practice.


Cerebral amyloid angiopathy

Cerebral amyloid angiopathy: a cross-sectional study in a single center in Northeastern Brazil. Until today, the prevalence of CAA is unknown in our region. This study aims to analyze the prevalence of this entity in a specific elderly population in a tertiary hospital in Northeastern Brazil. One hundred and seventy-four patients were enrolled, of whom were women


Cerebral amyloid angiopathy CAA , is a form of angiopathy in which amyloid beta peptide deposits in the walls of small to medium blood vessels of the central nervous system and meninges. The amyloid material is only found in the brain and as such the disease is not related to other forms of amyloidosis. Since this can be caused by the same amyloid protein that is associated with Alzheimer's dementia, brain bleeds [5] are more common in people who have a diagnosis of Alzheimer's disease. However, they can also occur in those who have no history of dementia. The bleeding within the brain is usually confined to a particular lobe [6] and this is slightly different compared to brain bleeds which occur as a consequence of high blood pressure hypertension - a more common cause of a hemorrhagic stroke or bleeding in the brain. CAA has been identified as occurring either sporadically generally in elderly populations [8] or in familial forms such as Flemish, Iowa, and Dutch types. Abnormalities in each of these identified clearance pathways have been linked to CAA.


CAA irrupts as a intracranial hemorrhage, which can be a cerebral hematoma or multifocal microhemorrhages. There is evidence that uremic environment and glomerular filtration rate decline in patients with chronic kidney disease, aggravate cognitive functions and are related to microhemorrhages occurrence. In this regard, it is interesting the case of an 82 years old patient with chronic kidney disease and hemodialysis for four years, who was evaluated due to a 48 hours disorientation state. His antecedents included memory impairment for nine years and an episode of multiple microangiopathic infarcts eight years ago.



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