Review: Psychiatric Disturbances in CADASIL

The featured article is ‘Psychiatric Disturbances in CADASIL’ by Valenti and colleagues. This is a brief article in which the authors present two cases of CADASIL with psychiatric disturbance and then review the literature. CADASIL stands for Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencepalopathy and is an example of a vascular condition predisposing to subcortical vascular dementia and having an identified genetic aetiology. The disease results from a mutation on the Chromosome 19 Notch3 gene.

Firstly the authors present two of their own cases – one being associated with post-partum psychosis in a 30-year old with an established mutation in the Notch3 gene and the other being a 72 year old lady with a prior history of depression. The only difficulty here is establishing causality in these cases as depression is common in the general population and the case of post-partum psychosis occurred at an early age and again causality is not demonstrated.

Next the authors go on to review the literature in the field and they clearly identify both the utilised search terms and search years in addition to stipulating other supplementary techniques they adopted for the identification of relevant papers. However it is not clear why the search years were limited to 1993-2007. The authors have assembled the results of these various papers into two tables with frequency of diagnostic categories identifiable at a glance. The authors show that depression is particularly common and that of other psychiatric diagnoses much less common. As they point out though, a number of investigators have not used stringent criteria for establishing psychiatric diagnoses. This in itself is problematic and we cannot ascertain the proportion with formal diagnoses from the table. Even more problematic is the lack of case-controls. We don’t have an age or gender breakdown for the different samples and it is not clear what comorbidity there was. A case-control study would provide evidence for a higher than expected rate of depression in this population. The authors conclude that future studies in this area should address methodological shortcomings in previous studies and establish temporal sequences from longitudinal data. Given the obvious difficulties in working with studies which have a different focus, the authors have done well to extract useful data for informing future research in this area.

Steps to Treatment 5 (complete longitudinal study, establish temporal data set for psychiatric illness in this population, utilise data to form population screening guidelines, incorporate into policy, prevention after screening)


Valenti R, Poggesi A, Pescini F, Inzitari D, Pantoni L. Psychiatric disturbances in CADASIL: A brief review. Acta Neurol Scand. 2008. 118. 291-295.

Steps To Treatment (STT)

STT = Steps To Treatment. An estimate of the number of steps between the results and translation into practice i.e. treatment. This is an opinion.


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The comments made here represent the opinions of the author and do not represent the profession or any body/organisation. The comments made here are not meant as a source of medical advice and those seeking medical advice are advised to consult with their own doctor. The author is not responsible for the contents of any external sites that are linked to in this blog.


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