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Management of behavioural variant frontotemporal dementia
I have been reading an extensive review of behavioural variant frontotemporal dementia which features in the January issue of the Lancet Neurology journal. The authors provide a comprehensive overview of the literature of this condition, as it currently stands, and I thought that the paper was interesting. Indeed, Piguet and colleagues are correct to discuss caregiver stress in the management of behavioural variant frontotemporal dementia. However, I would like to point out that it is the impulsivity and risk-taking behaviours of such patients which can be very distressing to the carers of such patients, particularly since the patients themselves tend not to have much insight into their behaviour and personality changes. It is precisely these types of symptoms which I showed could be treated using a psychostimulant, methylphenidate, in a placebo-controlled double-blind study, albeit with a small sample size. The full paper is visible HERE.
It is important to note however that methylphenidate has never been licensed routinely for this indication, and any individuals on pharmacological treatment for dementia should be entirely guided by their own physician.
Currently, no disease-specific treatment interventions for FTD exist. Consequently, treatment largely remains supportive and involves a combination of non-pharmacological and pharmacological measures aimed at reducing the effect of distressing symptoms.120 The role of pharmacological interventions in FTD remains uncertain, and only small and often conflicting treatment trials have been done so far; these studies have not considered the effect on carer stress as a major outcome variable. Selective serotonin reuptake inhibitors have been used to treat disinhibition and challenging behaviours, but evidence for their use remains contradictory.121, 122 Atypical antipsychotics such as olanzapine have been used for patients with prominent agitation, aggressive behaviour, or psychosis.123 Anticholinesterase inhibitors, the mainstay of AD therapy, do not have an established role in the treatment of FTD. One study reported improvement in measures of behavioural disturbance and carer stress with rivastigmine,124although deterioration in neuropsychiatric symptoms without cognitive improvement was shown with donepezil.125 Several drugs under development attempt to reduce aggregation of tau or TDP-43 and hence slow the fundamental pathological process in FTD.120, 126
Piguet O, Hornberger M, Mioshi E, Hodges JR.Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol. 2010 Dec 10. [Epub ahead of print] Neuroscience Research Australia, Randwick, NSW, Australia.
Abstract on MEDLINE here.
Dr Shibley Rahman on neuroscience and the law
My review on decision making and neuropsychiatry is here that I did over a decade ago. I think the last decade has taught me that we could do with learning a lot about how to measure abnormalities in decision-making in an objective way, and that there needs to be much greater convergence between neuroscience and the law. This review was published a long time ago in the world-leading journal Trends in Cognitive Sciences, and has, I feel, stood the test of time in the growing quantity and quality of research in this area.
For example, people can fly off the handle and kill their partner if provoked; this provocation defense in murder, bringing it down to manslaughter, is inadequately understood in terms of the neuroscientific mechanisms. Likewise, someone under law can lose it altogether at the drop of a hat, an ‘irresistible impulse‘, and bet billions on a horse winning the Grand National, and then refusing to pay because of a moment of madness (‘the insanity defense‘).
I feel, in my own area of dementia, quantifying risk-taking in behavioural variant frontotemporal dementia, will become important in the law. This is because currently the criminal courts have to rely on ‘expert evidence’, and it can be hard for a defense to argue for any organic basis for risky behaviour in the absence of any clear findings on a brain scan, for example. Especially when you consider that the standard of proof in the criminal courts is ‘beyond reasonable doubt’, this is of enormous significance. There may be a time when, if neuroscientific evidence can meet the Daubert standard, then they may be allowed in the defense of such a patient in the criminal courts. I have thought about this a lot, and have come to an important conclusion in parallel to this discussion; despite the rather disinhibited and risk-taking behaviour of some patients with behavioural variant frontotemporal dementia, it is rather rare for these behaviours to be criminal. I predict confidently, in the next decade, there will be a huge amount of work on the moral cognition of patients with behavioural variant frontotemporal dementia, especially in relation to that part of the brain I have written much about, the ventromedial prefrontal cortex.
Dr Shibley Rahman is an expert in behavioural variant frontotemporal dementia from Cambridge and London, and an active member of the World Neuroethics Society. In addition, he is a graduate of English law, and about to complete a Master of Law in London.
