Frontotemporal dementia progresses to death faster than Alzheimer disease

Roberson, Erik D.; Hesse, Jared; Rose, Kenneth D.; Slama, Hilary; Johnson, Julene K.; Yaffe, Kristine; Forman, Mark S.; Miller, Carol A.; Trojanowski, John Q.; Kramer, Joel H.; Miller, Bruce L.

doi:10.1212/01.wnl.0000173837.82820.9f

Cited by 250 publications

(220 citation statements)

References 35 publications

Supporting

Mentioning

189

Contrasting

Unclassified

Order By: Relevance

“…The lack of correlation might reflect weaknesses in diagnostic definitions, or could be due to the relatively small sample number. Earlier studies of FTD survival show a median survival time of three years from clinical presentation (15). In this study, rapid progression was defined as distinct frontal and/or temporal atrophy in a brain MRI after a maximum three years of disease duration, and survival time was not included in the definition.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Novel cerebrospinal fluid biomarkers of axonal degeneration in frontotemporal dementia

Mattsson

Rüetschi²,

Pijnenburg³

et al. 2008

Mol Med Rep

View full text Add to dashboard Cite

Abstract. Frontotemporal dementia (FTD) is a heterogeneous disease with substantial interpersonal variance in aggressiveness. Novel biomarkers for rapidly progressive FTD could improve diagnosis and provide clues regarding its pathogenesis. In this study, surface-enhanced laser desorption/ionization time-of-flight (SELDI-TOF) mass spectrometry (MS) was used to analyze peptide profiles in cerebrospinal fluid (CSF) from 24 FTD patients. Thirteen patients had rapidly progressive FTD with distinct pathology in a brain MRI after less than 3 years of disease duration. Eleven patients had slowly progressive FTD with a normal brain MRI, but had abnormal findings in SPECT/PET after more than 5 years of disease duration. The axonal damage marker CSF neurofilament light-chain (NF-L) was measured in all subjects to evaluate the amount of axonal degeneration. A CSF NF-L level of 150 ng/l was used as a cutoff point for high NF-L expression. SELDI-TOF analysis of peptides in the range of 2000-20000 m/z revealed one peak with m/z of 6378 that was expressed at a significantly different level (p<0.01) when rapidly versus slowly progressive cases of FTD were compared. Eleven peaks were expressed at different levels when high versus low CSF NF-L were compared. Using chromatographic purification followed by tandem mass spectrometric analysis, five of these peaks were identified as follows: C-terminal fragment of neuroendocrine protein 7B2 (3512.84 Da), C-terminal fragment of osteopontin (7658.19 Da) as well as its mono-and diphosphorylated forms (7738.16 Da and 7818.13 Da, respectively) and pancreatic ribonuclease (14566.33 Da). The peak intensity of pancreatic ribonuclease was higher in patients with low NF-L expression, while the other peptides had a lower peak intensity in this group. Altered levels of these peptides have also been described in other neurodegenerative diseases. Taken together, these data suggest that differentially-expressed peptides are general markers of axonal degeneration. Further studies are needed to verify their prognostic value in FTD.

show abstract

Section: Discussionmentioning

confidence: 99%

“…Although it has been suggested that FTD is an aggressive disease with a significantly shorter survival time than Alzheimer's disease (AD) (15), a more benign variant with a lack of gross atrophy on structural imaging or post-mortem macroscopy has been identified (5,6).…”

Section: Introductionmentioning

confidence: 99%

Novel cerebrospinal fluid biomarkers of axonal degeneration in frontotemporal dementia

Mattsson

Rüetschi²,

Pijnenburg³

et al. 2008

Mol Med Rep

View full text Add to dashboard Cite

show abstract

“…Most research efforts have concentrated on defining typical neuroimaging features of FTD cross-sectionally, but less is known about its clinical Roberson et al, 2005) and anatomical (Gregory et al, 1999;Chan et al, 2001;Whitwell et al, 2004) evolution over time.…”

Section: Introductionmentioning

confidence: 99%

A tensor based morphometry study of longitudinal gray matter contraction in FTD

et al. 2007

View full text Add to dashboard Cite

Frontotemporal dementia (FTD) is a neurodegenerative disease characterized by progressive behavioural abnormalities and frontotemporal atrophy. Here we used tensor based morphometry (TBM) to identify regions of longitudinal progression of gray matter atrophy in FTD compared to controls. T1-weighted MRI images were acquired at presentation and 1-year follow-up from 12 patients with mild to moderate FTD and 12 healthy controls. Using TBM as implemented in SPM2, a voxel-wise estimation of regional tissue volume change was derived from the deformation field required to warp a subject's late to early anatomical images. A whole brain analysis was performed, in which a level of significance of p<0.05 corrected for multiple comparisons (family wise error-FWE) was accepted. Based on prior studies, a region of interest (ROI) analysis was also performed, including in the search area bilateral medial and orbital frontal regions, anterior cingulate gyrus, insula, amygdala and hippocampus. Within this ROI a level of significance of p<0.001 uncorrected was accepted. In the whole brain analysis, the anterior cingulate/paracingulate gyri were the only regions that showed significant atrophy change over 1 year. In the ROI analysis, the left ventromedial frontal cortex, right medial superior frontal gyrus, anterior insulae and left amygdala/ hippocampus showed significant longitudinal changes. In conclusion, limbic and paralimbic regions showed detectable gray matter contraction over 1 year in FTD, confirming the susceptibility of these regions to the disease and the consistency with their putative role in causing typical presenting behaviours. These results suggest that TBM might be useful in tracking progression of regional atrophy in FTD.

show abstract

“…1 The differential diagnosis for FTLD and AD has a potential impact for treatment and prognosis. [2][3][4] Currently available drugs such as the acetylcholinesterase (AchE) inhibitor for treating AD are often ineffective for the treatment of FTLD. 5 Furthermore, FTLD often shares clinical features with AD, whereas AD is often associated with FTLD clinical syndromes.…”

mentioning

confidence: 99%

Atrophy Measurement of the Anterior Commissure and Substantia Innominata with 3T High-Resolution MR Imaging: Does the Measurement Differ for Patients with Frontotemporal Lobar Degeneration and Alzheimer Disease and for Healthy Subjects?

Moon¹,

Kim²,

Ro³

et al. 2008

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

show abstract

Frontotemporal dementia progresses to death faster than Alzheimer disease

Abstract: Frontotemporal lobar degeneration progresses more rapidly than Alzheimer disease, and the fastest-progressing cases are those with the frontotemporal dementia clinical subtype, coexisting motor neuron disease, or tau-negative neuropathology.

Cited by 250 publications

References 35 publications

Novel cerebrospinal fluid biomarkers of axonal degeneration in frontotemporal dementia

Novel cerebrospinal fluid biomarkers of axonal degeneration in frontotemporal dementia

A tensor based morphometry study of longitudinal gray matter contraction in FTD

Atrophy Measurement of the Anterior Commissure and Substantia Innominata with 3T High-Resolution MR Imaging: Does the Measurement Differ for Patients with Frontotemporal Lobar Degeneration and Alzheimer Disease and for Healthy Subjects?

Contact Info

Product

Resources

About