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Friday, September 21, 2012

The Hard Facts About bvFTD Nobody Wants To Tell You. Mortality And Progression.

The first question I had when I was diagnosed with bvFTD, (Well, right after, "What the Hell is that?"), was probably something like, "Well, Doc., how long do I have to live?" And then the doctor usually side-steps the question. They gave me some vague guesses, but really didn't answer the way I wanted. After all, plans need to be made!

Following are a couple studies that shed some light on the survival probabilities, or lack thereof. Remember that every case of bvFTD is different, and statistics do not predict individual outcomes. As a case in point, I was diagnosed a little over 2 and a half years ago, so I should be just about dead. Well, I am frakkin-well not dead yet, and not even considering it as an option! Screw'em!

It is interesting that there were 24 out of 91 phenocopy cases in the first study. This seems like a really high percentage to me. Not much is said about the criteria for diagnosis, or inclusion. The numbers are still dismal. The second study seems to do a slightly better job of addressing the non-progressive phenocopy cases of bvFTD, but the numbers are still dismal.
Title: Determinants of Survival in Behavioural Variant Frontotemporal Dementia.

Authors: Beatrice Garcin, Patricia Lillo, Michael Hornberger, Olivier Piguet, Kate Dawson, Peter Nestor, John Hodges

Journal: Neurology


Background: Behavior variant FTD (bvFTD) is a common cause of non-Alzheimer dementia. Little is known about its rate of progression but a recently identified subgroup seems to have an excellent prognosis (phenocopy cases) whereas the pathological cases decline rapidly. Data about natural history are needed to provide the best information to the patients and their families.

Methods: We estimated survival in a large group of bvFTD patients (n=91) and reviewed their demographic and clinical features to determine how they affect prognosis.

Results: Median survival in the whole group of 91 patients was 10.6 years from symptom onset, and 7.3 years from diagnosis. Log rank tests showed that being a female, having a positive family history, language impairment and motor symptoms at first assessment were associated with a significant shorter survival. The estimated hazard ratio indicated that younger age at onset, a higher score on MMSE and ACE were associated with longer survival. After the exclusion of 24 “phenocopy” cases, the analysis was repeated in a subgroup of 67 patients. In this latter group, median survival was 7.9 years from symptom onset and 4.0 years from diagnosis. The only factor associated with shorter survival was the presence of language impairment.

Conclusions: The prognosis of bvFTD is poorer when there are language features at presentation. This study also provides evidence for the existence of a benign subgroup of patients with clinical features of bvFTD.

...and an excerp from another study, this one targeted at Phenocopy bvFTD, which shows little or no progression:

Nonprogressive behavioural frontotemporal dementia: recent
developments and clinical implications of the ‘bvFTD phenocopy
Christopher M. Kippsa,b, John R. Hodgesc,d and Michael Hornbergerc,d
Wessex Neurological Centre, Southampton University
NHS Trust, bDepartment of Clinical Neurosciences,
University of Southampton, Southampton, UK,
Neuroscience Research Australia and dSchool of
Medicine, University of New South Wales, Sydney,
Correspondence to Dr Christopher Kipps, Consultant
Neurologist and Honorary Senior Clinical Lecturer,
Wessex Neurological Centre, Southampton University
NHS Trust, Southampton SO16 6YD, UK
E-mail: christopher.kipps@soton.ac.uk
Current Opinion in Neurology 2010, 23:628–632
Purpose of review
The clinical features of behavioural variant frontotemporal dementia (bvFTD) are well
established; however, recent work has identified patients fulfilling diagnostic criteria for
the disease who do not appear to progress clinically. This review describes means of
distinguishing this group at an early stage from patients who are likely to deteriorate.
Recent findings
Despite indistinguishable clinical profiles, studies in a cohort of bvFTD patients showed
a particularly good prognosis in a subgroup of predominantly male patients in whom
initial structural imaging was normal. This could not be explained by differences in
disease duration, and was confirmed by subsequent PET studies. Retrospective review
of clinical data in these groups verified that the current clinical diagnostic criteria are
both insensitive to true progressive bvFTD, particularly in the early stages, and also
poorly specific. In contrast, measures of activity of daily living performance, executive
function and tests of social cognition appear to have better discriminatory value for
patients who show clear clinical progression, with many individual diagnoses verified by
post mortem examination in this group.
It remains doubtful that the nonprogressive group have a neurodegenerative disease.
The implication for the current clinical diagnostic criteria and their proposed revision is

Moreover, a retrospective survival analysis of a bvFTD
cohort [5] showed that those patients with abnormal scans
were largely dead or institutionalized within 3 years, in
line with median survival time in pathologically proven
cases of bvFTD [7 ]. However, patients with scan ratings
in the control range had a significantly better disease
prognosis, with some patients surviving for 10 years or

So, the other question I had right away was something like, "How fast will this disease progress?" Once again the answer was unconvincing. They said that since my onset seemed to be rather slow, the progression should also be slow, so that maybe I had 10-12 years.

But how do you know if you have bvFTD if you are a fast or a slow progresser, or a phenotype case who may not even progress at all? That book I talked about, What If It's Not Alzheimer's?, gives some general guidelines that may be useful. I still highly recommend this book as I use it as a reference, and will repost the post about it if I can figure out how. It should also be available through a link on the sidebar of this blog somewhere - maybe lower left. It is worth the modest price.

According to the book,  losing 2 points per year, as in a declining test score on the MMSE (mini–mental state examination (MMSE) or Folstein test) is about average progression. Four points a year or more would indicate a rapid progression. If you lose 1 or no points you are a slow progresser, or even a phenocopy case. It may take several years to get a good idea of progression, and again: every case of bvFTD is different.

Comments are welcome.


  1. I just wondered how you are doing...Are you still you?

  2. I am wondering how you are doing as well.