Amyloid Beta-Related Angiitis—A Case Report and Comprehensive Review of Literature of 94 Cases

Background

Amyloid Beta-Related Angiitis (ABRA) is a rare cause of central nervous system vasculitis complicating cerebral amyloid angiopathy. Data regarding its prevalence, clinical features, management, and outcomes are scant.

Objectives

To describe a patient with ABRA and discuss clinical features and management of ABRA.

Methods

A case report and review of literature were conducted of all reported cases of ABRA in the English literature.

Results

The exact etiology of ABRA is not clear, though it is thought to be secondary to an inflammatory response to beta amyloid (Aβ) in the walls of blood vessels. Role of ApoE e4/e4 genotype and its association with autoimmune diseases have been reported. ABRA shares many clinical features with primary CNS vasculitis. Patients with ABRA are relatively younger than those with non-inflammatory cerebral amyloid angiopathy (CAA), but older than patients with primary central nervous system vasculitis (PCNSV). Acute-onset cognitive behavioral abnormalities, focal neurological deficits, seizures, or unusual headaches are the most common presentations of ABRA. Majority have elevated CSF proteins. Up to 70% of patients have ApoE e4/e4 genotype. MRI is the most important diagnostic tool and is almost always abnormal. Characteristically, MRI shows hyperintensities on T2-weighted (T2W) or fluid-attenuation inversion recovery (FLAIR) images with minimal gadolinium enhancement. On susceptibility-weighted images (SWI), a majority of the patients have the presence of microbleeds at cortico-subcortical junction. It may be possible to diagnose typical patients based on clinical features and MRI findings alone, obviating the need for brain biopsy. Brain biopsy is the gold standard and shows transmural granulomatous vasculitis superimposed on CAA. ABRA responds well to steroids in majority. Patients usually need additional immunosuppressants, especially to prevent relapse. MRI abnormalities resolve with treatment and recur with the relapse.

Conclusions

ABRA is a rare but treatable cause of progressive dementia and should be considered in the differential diagnosis of rapid-onset CNS dysfunction in patients older than 60 years. It has characteristic MRI findings and responds well to steroids and other immunosuppressant therapy.     

Blood oxygen level dependent angiography (BOLDangio) and its potential applications in cancer research

Clinically, development of anti‐angiogenic drugs for cancer therapy is pivotal. Longitudinal monitoring of tumour angiogenesis can help clinicians determine the effectiveness of anti‐angiogenic therapy. Blood oxygen level dependent (BOLD) effect has been widely used for functional imaging and tumour oxygenation assessment. In this study, the BOLD effect is investigated under different levels of oxygen inhalation for the development of a novel angiographic MRI technique, blood oxygen level dependent angiography (BOLDangio). Under short‐term (<10 min) generalized hypoxia induced by inhalation of 8% oxygen, we measure BOLD contrast as high as 25% from vessels at 9.4T using a simple gradient echo (GRE) pulse sequence. This produces high‐resolution 2D and 3D maps of normal and tumour brain vasculature in less than 10 minutes. Additionally, this technique reliably detects metastatic tumours and tumour‐induced intracranial hemorrhage. BOLDangio provides a sensitive research tool for MRI of vasculature under normal and pathological conditions. Thus, it may be applied as a simple monitoring technique for measuring the effectiveness of anti‐angiogenic drugs in a preclinical environment. Copyright © 2012 John Wiley & Sons, Ltd.     

Stability of the age distribution of measles cases over time during outbreaks in Bangladesh, 2004-2006

Despite recommendations from WHO to conduct measles outbreak response vaccination campaigns based on the age distribution of cases at the beginning of an outbreak, few data exist to specifically examine whether the age distribution of cases remains constant over time in a measles outbreak. This analysis explores this question with use of measles outbreak surveillance data from Bangladesh from the period 2004-2006. Pearson χ(2) tests were conducted of age distributions over 2 periods during 41 large laboratory-confirmed measles outbreaks. Statistically significant changes in age distribution over time were observed in 24% of the outbreaks. No single pattern was detected in the shifts in age distribution; however, an increase in the proportion of cases occurring among infants <9 months of age was evident in 6 outbreaks. These findings suggest a need to consider the possibility of a shift in the age distribution over time when planning an outbreak response vaccination campaign.