Marcelo D Mendonça1, André Caetano2, Miguel Pinto2, Vera Cruz e Silva3, Miguel Viana-Baptista4. 1. Neurology Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal; Centro de Estudos de Doenças Crónicas (CEDOC), Nova Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal. Electronic address: marcelomendoncasousa@gmail.com. 2. Neurology Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal. 3. Neuroimaging Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal. 4. Neurology Department, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal; Centro de Estudos de Doenças Crónicas (CEDOC), Nova Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.
Abstract
BACKGROUND: Cerebral amyloid angiopathy (CAA) is a common, often asymptomatic disease. Lobar intracerebral hemorrhage is the most frequent manifestation of CAA. Nevertheless, presentation of CAA with subacute cognitive decline, seizures, or headache with concomitant hyperintensities on T2-weighted magnetic resonance imaging (MRI) sequences and neuropathologic evidence of inflammation has been described. This disorder is known as CAA-related inflammation (CAA-ri). METHODS: Description of a stroke-like presentation of CAA-ri and systematic review of case reports and case series of CAA-ri. RESULTS: A 75-year-old woman with a history of atrial fibrillation, and a transient episode of aphasia 2 days before, presented in the emergency room with sudden onset aphasia. Brain computed tomography disclosed a left temporal hypodensity. A diagnosis of probable stroke was given. During the following days, there was a progressive clinical deterioration. MRI revealed coalescent edematous white matter lesion, hyperintense on T2-weighted sequences, and multiple lobar microbleeds on T2*-weighted sequences. A diagnosis of CAA-ri was considered, and the patient was started on steroids with clinical and imaging improvement. From our systematic review, microbleeds were present in almost 90% of patients with CAA-ri. CONCLUSIONS: Imaging findings associated with CAA-ri allow the early diagnosis and treatment of this potentially reversible disorder. Aside from the most common subacute presentations, CAA-ri can have a stroke-like presentation and be a stroke mimic.
BACKGROUND:Cerebral amyloid angiopathy (CAA) is a common, often asymptomatic disease. Lobar intracerebral hemorrhage is the most frequent manifestation of CAA. Nevertheless, presentation of CAA with subacute cognitive decline, seizures, or headache with concomitant hyperintensities on T2-weighted magnetic resonance imaging (MRI) sequences and neuropathologic evidence of inflammation has been described. This disorder is known as CAA-related inflammation (CAA-ri). METHODS: Description of a stroke-like presentation of CAA-ri and systematic review of case reports and case series of CAA-ri. RESULTS: A 75-year-old woman with a history of atrial fibrillation, and a transient episode of aphasia 2 days before, presented in the emergency room with sudden onset aphasia. Brain computed tomography disclosed a left temporal hypodensity. A diagnosis of probable stroke was given. During the following days, there was a progressive clinical deterioration. MRI revealed coalescent edematous white matter lesion, hyperintense on T2-weighted sequences, and multiple lobar microbleeds on T2*-weighted sequences. A diagnosis of CAA-ri was considered, and the patient was started on steroids with clinical and imaging improvement. From our systematic review, microbleeds were present in almost 90% of patients with CAA-ri. CONCLUSIONS: Imaging findings associated with CAA-ri allow the early diagnosis and treatment of this potentially reversible disorder. Aside from the most common subacute presentations, CAA-ri can have a stroke-like presentation and be a stroke mimic.
Authors: Claudia Uribe Roca; Fabio Maximiliano Gonzalez; Marta Ines Bala; Miguel Saucedo; Lucrecia Bandeo; Luciana Leon Cejas; Sol Pacha; Pablo Bonardo; Carlos Rugilo; Pablo Dezanzo; Rafael Torino; Gustavo Sevlever; Manuel Fernandez Pardal; Ricardo Reisin Journal: Psychiatry Investig Date: 2021-06-17 Impact factor: 2.505