Neurodegenerative Disease Dementia
New Diagnostic Criteria for the Behavioural Variant of Frontotemporal Dementia
Yolande AL Pijnenburg Neurologist, Department of Neurology and Alzheimer Centre, VU University Medical Centre
Abstract
In September 2011, the International Behavioural Variant Frontotemporal Dementia Criteria Consortium established a new set of clinical diagnostic criteria for the behavioural variant of frontotemporal dementia (bvFTD). Following the 1998 criteria by Neary et al., where five core items had to be present to make a diagnosis of bvFTD, the new criteria incorporate six clinical hallmarks and demand that at least three of these are present. Moreover, a degree of probability and a role for neuroimaging findings are introduced within the criteria. The new diagnostic criteria have a sensitivity of 76 % for probable bvFTD and a sensitivity of 86 % for possible bvFTD. They provide a good starting point for a more accurate diagnosis of bvFTD.
Keywords Frontotemporal dementia, diagnosis, clinical, sensitivity, pathology
Disclosure: The author has no conflicts of interest to declare. Received: 31 October 2011 Accepted: 10 November 2011 Citation: European Neurological Review, 2011;6(4):234–7 Correspondence: Yolande AL Pijnenburg, Department of Neurology and Alzheimer Centre, VU University Medical Centre, PO Box 7057, 1007 MB Amsterdam, The Netherlands. E:
y.pijnenburg@vumc.nl
Frontotemporal dementia (FTD) is a neurodegenerative disorder mainly affecting the frontal and/or temporal lobes, leading to dementia with prominent behavioural and/or language disturbances. Symptom onset is most often between 45 and 65 years. Arnold Pick described the disease with remarkably focal lobar atrophy in 1892 and it was later termed Pick’s disease by Alois Alzheimer.1,2
frontotemporal lobar degeneration (FTLD) with Pick bodies consisting of tau-positive intraneuronal inclusions is not the only pathological correlate of FTD. A variety of tau-positive abnormalities are responsible for about 45 % of FTLD (FTLD-tau), whereas another 45–50 % of FTD cases are associated with ubiquitin-positive, TAR DNA-binding protein-43 (TDP-43)-positive pathology (FTLD-TDP).3–5
Lastly, about
5–10 % of FTLD cases have ubiquitin-positive, TDP-43-negative, fused in sarcoma protein (FUS)-positive pathology (FTLD-FUS).6
FTD has
a relatively high degree of heritability. Mutations in the tau gene, progranulin gene and the recently discovered C9orf72 gene are responsible for up to 25 % of FTD cases.7–11
In the late 1980s, renewed interest in non-Alzheimer dementias arose as it became evident that, among patients who had died from dementia, 10 % suffered from frontotemporal cortical degeneration without Alzheimer’s disease pathology.12
characteristics of these patients were described in more detail.13,14
Subsequently, the clinical In
1994, the Lund and Manchester groups published clinical and neuropathological criteria for FTD, whereby clinical symptoms were merely listed and pathological syndromes were subdivided into a frontal lobe degeneration type consisting of non-specific neurodegenerative changes, the Pick type including Pick bodies and the motor neurone disease type which at the time was non-specific as well.15
The clinical symptoms of FTD were refined into a stricter corpus 234
With some adaptations by an American working group on FTD, proposing that the term FTLD be reserved for the pathological spectrum and the term FTD for the clinical spectrum, including both language variants (SD and PNFA) and the behavioural variant (bvFTD), the 1998 criteria have remained widely used until very recently.
Some shortcomings and unmet needs, however, have ultimately led to the development of a new set of clinical diagnostic criteria for FTD by a large international consortium, which will be described in detail in this article.17,18
Although the five core criteria for bvFTD intuitively fit very well with the clinical picture of a typical bvFTD patient, one of the practical problems these criteria raise is the interpretation of rather abstract descriptions such as ‘disturbed regulation of personal behaviour’. Obviously, this type of description leaves room for free interpretation by the clinician. Moreover, due to lack of insight bvFTD patients will not complain about their behavioural and emotional changes spontaneously and clinicians
© TOUCH BRIEFINGS 2011
of criteria in 1998.16
At present, it is known that
Here the term FTLD was used as an umbrella for three main clinical syndromes: FTD, semantic dementia (SD) and progressive non-fluent aphasia (PNFA). Whereas the latter two present with language disturbances, FTD is characterised by five core clinical criteria, all of which had to be present to make a diagnosis of FTD. These included an insidious onset and gradual progression, an early decline of social interpersonal behaviour, an early decline in the regulation of personal behaviour, early emotional blunting and an early loss of insight. Furthermore, a number of supportive features such as pre-senile onset were formulated. Exclusion features included, among others, severe amnesia and spatial disorientation.
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