Elsevier

Brain Research

Volume 1519, 26 June 2013, Pages 19-30
Brain Research

Review
Cerebral amyloidal angiopathy—A disease with implications for neurology and psychiatry

https://doi.org/10.1016/j.brainres.2013.04.052Get rights and content

Abstract

Cerebral Amyloidal Angiopathy (CAA), which occurs sporadically in most cases but can also occur hereditarily, belongs to the group amyloidoses and is characterized by the deposition and accumulation of beta-amyloid (Aβ) in smaller arterial vessels of the brain. The deposition of Aβ leads to degenerative changes in the cerebral vessel system (thickening of the vessel wall, microaneurysm, constriction of vascular lumen, dissection), which favour the development of the clinical symptomatology most often associated with CAA. Besides haemorrhages, cerebral ischaemia, transient neurological symptoms, leukoencephalopathy as well as cognitive decline and even dementia may appear in connection with CAA. A definite diagnosis of CAA can only be made on the basis of a pathological assessment, even though diagnostic findings of cerebral neuroimaging and clinical symptoms allow the diagnosis of a probable CAA. At present, no causal therapy options are available. Although CAA is placed within the range of neurological illnesses, psychiatric symptoms such as cognitive impairment, personality change or behavioural problems as well as depression are plausible clinical manifestations of CAA and may even dominate the clinical picture. Apart from epidemiological, pathogenetical, clinical and diagnostical aspects, possible psychiatric implications of CAA are discussed in the review article.

Introduction

Cerebral Amyloidal Angiopathy (CAA) belongs to the group of amyloidoses (Biffi and Greenberg, 2011), which constitute a heterogenic range of diseases. Amyloidoses are characterized by an interstitial deposition and accumulation of proteins potentially affecting all body tissues, which are modified pathologically as to their secondary structure and are, in most cases, indissoluble (Merlini and Bellotti, 2003). These proteins, existing in the form of fibrillates, are referred to as amyloid and lead to tissue destruction and a progredient impairment of the corresponding organ function (Merlini and Bellotti, 2003, Perfetto et al., 2010). The term Cerebral Amyloidal Angiopathy also refers to a heterogenic group of illnesses of the central nervous system differing by their genetic and biochemical characteristics (Biffi and Greenberg, 2011), which can be characterized histopathologically by the presence of amyloidal fibrils in small to medium-sized, in most cases, arterial vessels of the brain. Spontaneously appearing intracerebral bleeding (ICB), which affects mostly superficial regions of the cortex, constitutes the leading symptom of CAA (Pezzini et al., 2009, Viswanathan and Greenberg, 2011). While the incidence of ICB with patients younger than 75 has decreased these last few years due to efficient antihypertensive pharmacotherapy, patients older than 74 present an increase of said incidence. Besides other factors, this can be explained by the age-dependent incidence of CAA (Lovelock et al., 2007). Clues of CAA can be found with over 50% of patients aged between 70 and 89, while it is rare under the age of 55 (Yoshimura, 2000). On the background of demographic modifications, a further increase in the prevalence of CAA may be observed in the future (Block, 2011).

CAA is considered to be a neurological disease, but can also appear with symptoms requiring psychiatric treatment. The present review paper shall enable an insight into the historical, epidemiologic, pathogenetical and clinical aspects of CAA. Possible psychiatric implications will be discussed as well.

Section snippets

Methods

A narrative review was conducted. In September 2012 the Medline database was searched using the search term “cerebral amyloid angiopathy”. Initally, n=1812 hits were retrieved. Considering only publications written in English the original number of hits decreased to n=1628. Titles and abstracts of these articles were checked for their relevance to this article. Finally, n=147 publications were considered for this review article.

Psychiatric implications of CAA

Though CAA is mainly a neurological illness, it can also appear bearing psychiatric symptoms. Currently there are no studies that address the possible psychiatric implications of CAA systematically. In the following we aim at discussing aspects of CAA that might be relevant to psychiatry.

Due to CAA being a chronic progredient illness of the central nervous system, the development of slight psychiatric symptoms during the disease progression is conceivable, especially in the first stages of CAA,

Conclusions

Apart from the typical clinical presentation of CAA that includes ICB, cerebral ischaemia and transient neurological symptoms such as cognitive impairment and dementia as well as depression, behavioural problems and/or personality change are plausible clinical manifestations of CAA. This implicates that CAA might be a disease with relevance to psychiatry. In this connection it is important to keep in mind that CAA might be the cause for occasionally unspecific psychopathological abnormalities,

Acknowledgments

None.

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