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Journal of Alzheimer’s Disease 48 (2015) 703–709
DOI 10.3233/JAD-150275
IOS Press
703
Telling the Story of Frontotemporal
Dementia by Bibliometric Analysis
Davide Guidoa, Gabriella Morandia,∗, Fernando Palluzziband Barbara Borronic
aUniversity of Pavia, Department of Brain and Behavioral Sciences, Medical and Genomic Statistics Unit,
Pavia, Italy
bPolitecnico, Department of Electronics, Information and Bioengineering, Milan, Italy
cUniversity of Brescia, Department of Neurology, Brescia, Italy
Accepted 25 June 2015
Abstract. In this paper, we reconstructed the medical history of frontotemporal dementia (FTD) by reviewing the literature
and analyzing papers with the highest impact through citation index. Several research studies and groups involved in FTD have
been reviewed. An increasing amount of knowledge has been made available in the last 20 years through a large number of
publications, leading to a better definition of the genetic and clinical bases of the disease. A total of 1,436 references (articles and
reviews), published in 395 journals, were retrieved through the Scopus database. The two highest publication peaks (i.e., largest
number of publications) were found in 2000 and 2008. The most cited papers considering both total citation number and the
number of citations within the first two years after publication refer to: (i) the genetic bases of FTD, (ii) the clinical criteria that
progressively refined the different FTD phenotypes, and (iii) FTD epidemiology. Advanced neuroimaging techniques, genotype-
phenotype heterogeneity, and animal models gave us a broader understanding of various aspects of the disorder. These findings
confirm the great interest in FTD research. The analysis of the literature might help in guiding future goals in the field.
Keywords: Bibliometric analysis, citations analysis, frontotemporal dementia, frontotemporal lobar degeneration
INTRODUCTION
Frontotemporal dementia (FTD) is the second
most frequent form of neurodegenerative early-onset
dementia [1]. It clinically presents with either behav-
ioral symptoms (behavioral variant FTD) or language
disturbances (primary progressive aphasia), but it
can overlap with motor neuron disease, progressive
supranuclear palsy, and corticobasal syndrome [2–4].
In the last few years, a huge step forward in the knowl-
edge of the disease has been made, since causative
genes that lead to autosomal dominant inherited FTD
have been identified [5], and neuropathological hall-
marks have been carefully described [6].
∗Correspondence to: Gabriella Morandi, University of Pavia,
Department of Brain and Behavioral Sciences, Medical and
Genomic Statistics Unit, Via A. Bassi 21, I-27100, Pavia, Italy.
Tel.: +39 0382 987570; Fax: +39 0382 987570; E-mail: gabriella.
morandi@unipv.it.
The present work aimed at investigating the increas-
ing literature in the field of FTD, using a bibliometric
analysis. We carried out an objective (quantitative)
overview of the existing FTD literature. Specifically,
our study (i) investigated the trend of scientific litera-
ture by providing a historical perspective; (ii) evaluated
the most cited articles and articles with the highest
impact and visibility in the literature; and (iii) analyzed
the citations per calendar year cohorts.
This methodological approach has already been
used in a number of neurological disorders, such
as stroke [7], over 44 neurological disorders [8],
and dementia [9, 10] and to investigate the top 100
researchers in Alzheimer’s disease [11].
Citation analysis, being the source of the biblio-
metric indicators, has been increasingly studied both
in the content and methodological aspects, and cita-
tion databases such as Scopus and Web of Science are
increasingly recognized as useful tools for assessing
the scientific relevance of research topics.
ISSN 1387-2877/15/$35.00 © 2015 – IOS Press and the authors. All rights reserved
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704 D. Guido et al. / Bibliometric Analysis in FTD
The database chosen was Scopus. We searched
for “fronto-temporal dementia or frontotemporal
dementia” on September 1, 2014. This yielded 1,745
documents of which the oldest dates back to1990.
This approach allowed us to evaluate the impact of
the FTD literature on clinical grounds and to guide the
reader through the landscape works and key research
advances.
MATERIALS AND METHODS
To retrieve documents on FTD, we used the Scopus
database. Elsevier developed Scopus in 2004, cov-
ering 19,500 peer-reviewed journals (100% Medline
coverage).
We searched for “frontotemporal dementia” or
“fronto-temporal dementia” in article titles. We did
not specify any starting year, whereas the end date
was December the 31st 2013, both for search and cita-
tion count. In Scopus, citations are accurately available
from 1996. Our analysis was carried out considering
only articles and reviews.
We conducted a descriptive analysis on the growth
pattern, language, and core journals. Subject categories
of the journals and their ranking position were derived
from the Scimago journal rank, an open platform based
on journals in the Scopus database.
The output retrieved from the publications was con-
verted to an Excel®file, and the statistical analyses
were performed using the R software [12]. Article per-
formance was evaluated using the following indices:
(a) the overall number of times the article was cited
(total citations, i.e., TC); (b) the number of times the
article was cited within the first 2 years after publica-
tion year (TC2); and (c) the citations per publication,
computed as the number of citations divided by the
number of articles published (citation per publication,
CPP).
The cumulative citation numbers (cumulative CPP)
per chronological years and article life were analyzed
by a linear regression model [13]. Our model used log-
scaling, expressed as log(C) = B ×Y+A+E,where C
is the response cumulative citations per publication in
the calendar year, B is the log-citation rate (log-number
of times cited/year), Y is the explanatory ‘article life’,
A is the potential visibility when the paper is pub-
lished (the log-number of times cited in the publication
year), and E is the model error term. Model parameters
and fitted values were computed by Generalized Least
Squares to manage time autocorrelations [14]. The
parameters were re-expressed in the original counting
scale: exp(B)–1 represented the average percentage of
the annual change of the cumulative number of cita-
tions, exp(A) was the average number of times cited
in the publication year, and exp(fitted values) was the
average cumulative number of citations per publication
conditional to article life (in years).
RESULTS
Literature overview
The literature search yielded 1,745 documents. Arti-
cles and reviews were 1,436 out of 1,745 (82.3%, 1,223
articles and 213 reviews), while the others (17.7%)
were conference papers, letters, notes, editorials, brief
reports, and book chapters. Figure 1 reports the trend of
papers, both articles and reviews, by publication year.
The oldest articles found by Scopus were published in
1990. Time trend showed a growing number of publi-
cations across years except for 2002 and 2010. In 2013,
this trend increased by 47% compared to the previous
year.
Of the 17 languages published, English was pre-
dominant (89%), followed by French (2.5%) and
Spanish (2.1%). German and Japanese covered 1.6%
of publications, followed by Portuguese (1.4%). Other
languages with less than 1% were Dutch, Polish,
Chinese, Italian, Turkish, Hungarian, Czech, Danish,
Hebrew, Russian, and Swedish.
A total of 395 journals published the 1436 doc-
uments retrieved. Twelve journals (3% of the total
journals) published 476 documents (33% of all the
studies), while 229 journals (58%) published only 1
article. Journals that published 20 or more papers
were reported in Table 1; their subject category and
Fig. 1. Numbers of articles on frontotemporal dementia published
over time.
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D. Guido et al. / Bibliometric Analysis in FTD 705
Table 1
List of journals with more than 20 papers with subject category and position in ranking (defined by quartiles: Q1 means the best ranking and
Q4 the worst ranking)
Journals Number of papers Number of articles Number of reviews Subject category / Quartile
Neurology 93 (6.5%) 89 4 Neurology (clinical) Q1∗
Art and Humanities (miscellaneous) Q1
Dement Geriatr Cogn Disord 74 (5.2%) 71 3 Geriatrics and gerontology Q1
Psychiatry and mental health Q1
Cognitive neuroscience Q1
Brain 48 (3.3%) 45 3 Art and Humanities (miscellaneous) Q1
Medicine (miscellaneous) Q1
Neurology (clinical) Q1
Arch Neurol 45 (3.1%) 42 3 Neurology (clinical) Q1
Art and Humanities (miscellaneous) Q1
J Neurol Neurosurg Psychiatry 40 (2.8%) 38 2 Neurology (clinical) Q1
Art and Humanities (miscellaneous)
Psychiatry and mental health Q1
Surgery Q1
Acta Neuropathol 28 (1.9%) 24 4 Neurology (clinical) Q1
Pathology and forensic medicine Q1
Cellular and molecular neuroscience Q1
Neurocase 25 (1.7%) 22 3 Neurology (clinical) Q2
Art and Humanities (miscellaneous) Q2
Neurobiol Aging 25 (1.7%) 25 – Neurology (clinical) Q1
Neuroscience (miscellaneous) Q1
Aging Q1
Developmental biology Q1
Geriatrics and gerontology Q1
Alzheimer Dis Assoc Disord 25 (1.7%) 24 1 Geriatrics and gerontology Q1
Clinical psychology Q1
Gerontology Q1
Psychiatric and mental health Q1
Ann Neurol 25 (1.7%) 24 1 Neurology Q1
Neurology (clinical) Q1
J Alzheimers Dis 25 (1.7%) 23 2 Psychiatry and mental health Q1
Geriatrics and gerontology Q1
Clinicalpsychology Q1
Neuropsychologia 23 (1.6%) 21 2 Arts and humanities (miscellaneous) Q1
Behavioral neuroscience Q1
Cognitive neuroscience Q1
Experimental and cognitive psychology Q1
Total 476 (33%) 448 28
TOTAL 1,436 1,223 2,13
∗quartiles indicating the highest impact: Q1 means first (highest) quartile (Q4 represents the lowest quartile, Q2 and Q3 the in between quartiles).
highest ranking position in Scimago journal rank for
2013 were quoted (Quartile 1, i.e., Q1, means the
highest values and Q4 the lowest value).
Our results follow Bradford’s law [15], which states
that a small core of journals produce approximately
one-third of all published documents.
The principal subject category was Clinical Neurol-
ogy, which included 341 journals in 2013. Except for
Neurocase, which was positioned in Q2, all the jour-
nals considered had the highest rank and were in the
Q1 category. The highest ranks were held by Brain:
a Journal of Neurology, followed by Acta Neuropatho-
logica (5◦), and Archives of Neurology (now JAMA
Neurology) (14◦). The second journal accounting
for the greatest number of papers was Dementia
and Geriatric Cognitive Disorders,inQ1,butin
a different subject category. The journal that pub-
lished the greatest number of reviews was Current
Opinion in Neurology (n=7), positioned in Q1, fol-
lowed by Handbook of Clinical Neurology (n= 6),
which was in Q3. Current Neurology,Neuroscience
Reports (both in Q1), Ideggyogyaszati Szemle and
The Neurologist (both in Q3) published 5 reviews
each.
We found comparable results considering the Impact
Factor, i.e., the best known indicator based on the Web
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706 D. Guido et al. / Bibliometric Analysis in FTD
of Science. In the same year and category, comprising
194 journals, we found Brain,Acta Neuropathologica,
Neurology,Neuroscientist, and Current Opinion in
Neurology in the top ranking.
Citation analysis
Considering all the published works, the total num-
ber of citations was 46,791 until December 31, 2013.
Excluding self-citations, the number was reduced to
39,376. Thus, self-citation was about 16% of the total,
i.e., approximately one out of six was an author’s
self-citation.
In Table 2, the chronological trend of citations
divided by time period is reported. The citations from
2000 to 2013 accounted for 71.2% of the total, but the
highest number of CPP (citation per publication) was
found in the 1995–1999 cohort.
The percentage distribution of the citation indexes
over a long-time (TC) and short-time (TC2) period
appeared comparable (Table 3). The median values of
TC2 (the number of citations within the first 2 years
after publication) were half of TC (the number total
citation) (4 versus8). Nevertheless, both TC and TC2
distributions were proportionally similar in distribu-
tion and outlier numbers, i.e., the number of citations
more than 1.5 times the difference in the third and first
quartiles of the observed index. Specifically, the arti-
cles with zero citations were 19% (275/1436) versus
20% (237/1149) and the upper outliers with more than
76 (TC) and 24 (TC2) citations were 9.5% (137/1436)
versus 7.2% (81/1149).
Table 4 and Fig. 2 illustrate the parameters and the
fitted lines of the citation models, respectively. On
average, the articles of the 2004–2008 cohorts had
good citation impacts (intercept >1) in the literature.
In particular, papers published in 2006 had the largest
visibility (intercept = 3.44) within the first year from
publication (being cited averagely 3.44 times). This
effect was mainly due to the papers by Baker [16] and
Cruts [17].
Table 2
Chronological trend of total citations and citations per publication
(CPP)
Years Publications Total citations CPP
1990–1994 6 84 14
1995–1999 135 8,195 60.7
2000–2004 339 14,774 43.6
2005–2009 491 13,279 27
2010–2013 465 3,044 6.5
TOTAL 1,436 39,376
Overall, the most recent cohorts (after 2008) had
higher citation/year rate (slope) than the oldest ones
(those published before 2008), as the recent articles
were on average more cited. Nevertheless, some papers
published before 2008, i.e., 1996, 2000–2003, had
good relevance trend, having great percentages of
change, but they showed low intercept parameter, i.e.,
low citation impacts in the publication years.
Finally, Table 5A-B quoted the most cited papers.
The results were different when we considered the total
citations or the citation received within the first two
years after publication. Four authors were present in
both lists. The work by Nearly and colleagues [18]
ranked first with 1,242 citations, while Baker’s paper
[16] was first regarding TC2 and it was fourth in total
citations.
Table 3
Statistics of total citations and total citations within the first 2 years
after publication
Total citations Total citations 2 years
Number of articles 1,436 1,149∗
Lower outlier∧0 (19.2%) 0 (20.6%)
First quartile (Q1) 1 1
Median (Q2) 8 4
Third quartile (Q3) 31 10
Upper outlier∧>76 (9.5%) >24 (7.0%)
∗Articles published from 1996 to 2011. ∧, Outlier values:
lower = Q1+1.5 ×(Q1-Q3) and upper = Q3+1.5 ×(Q3-Q1). Q, quar-
tiles, according to position in ranking (quartile: Q1 means the highest
values and Q4 the lowest values).
Fig. 2. Regression lines of cumulative citations (CPP) on article life
across publication years (from 1996 to 2012).
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D. Guido et al. / Bibliometric Analysis in FTD 707
DISCUSSION
In this work, we gave an overview of FTD history
by reviewing the literature. We assessed the number of
publications and citations as well as journal ranks, and
Table 4
Parameters of the fitted lines estimated by Generalized Least Squares
Year Intercept Slope
1996 <0.01 207.7
1997 0.02 93.7
1998 0.17 70.2
1999 0.07 87.3
2000 0.03 112.0
2001 0.05 114.9
2002 0.12 105.1
2003 0.03 141.1
2004 1.69 51.1
2005 2.27 53.3
2006 3.44 51.5
2007 2.35 57.9
2008 1.11 92.8
2009 0.83 136.0
2010 0.76 201.5
2011 1.06 219.3
2012 1.53∗338.0∗
∗Ordinary Least Squares estimates because of lack of degree of free-
dom. Intercept is the average number of times cited when a paper
is published (i.e., article life =0 for chronological cohorts); slope is
the average percentage change of number of times cited/year.
analyzed which authors/articles achieved the highest
impact.
FTD knowledge has been increasing, especially in
recent years, due to: (i) a better and careful definition
of clinical picture [3, 4]; (ii) the characterization of
the key players in the molecular pathogenesis, such
as Tau and TDP43 [19]; (iii) the identification of
monogenic causes of the disease, such as mutations
within Microtubule Associated Protein Tau (MAPT),
Granulin (GRN), or C9orf72 [16, 17, 20, 21]. These
advances have been demonstrated by an increasing
number of publications. We observed two main peaks
in the trend of the number of publications, namely in
2000 and 2008 (see Fig. 1), that underlined the two key
points in FTD literature: on the one hand, the identifi-
cation of MAPT mutations and FTD-Tau disease that
occurred in 1998 [20, 22], and on the other hand, the
identification of GRN mutations [16, 17] that occurred
in 2006.
Although we cannot comment on the raw citation
data for the last years, the highest value of CPP was
obtained in the 1995–1999 cohort (Table 2). The most-
cited paper analysis, both considering total citations
and the number of citations within the first two years
after publication, accounts for the great interest in the
genetic basis of FTD. Among the most cited papers, we
found those identifying MAPT and GRN mutations in
Table 5
Most (top-10) cited papers by total citations (A) and by total citations within the first 2 years after publication (B)
(A) Authors Year TC Breakthroughs
Neary [18] 1994 1,242 Definition of clinical criteria
Poorkaj [2] 1998 841 Identification of MAPT mutations
McKhann [25] 2001 745 Definition of clinical criteria
Baker [16] 2006 600 Identification of GRN mutations
Cruts [17] 2006 525 Identification of GRN mutations
Watts [23] 2004 502 Identification of VCP mutations
Ratnavalli [26] 2002 457 Epidemiology of FTD
Skibinski [24] 2005 337 Identification of CHMP2B mutations
Hodges [27] 2004 309 Clinico-pathological correlates of FTD
Rosen [28] 2002 305 Description of patterns of atrophy in FTD
(B) Authors Year TC2 Breakthroughs
Baker [16] 2006 197 Identification of GRN mutations
Cruts [17] 2006 191 Identification of GRN mutations
Poorkaj [22] 1998 175 Identification of MAPT mutations
Mackenzie [6] 2010 134 Definition of neuropath criteria
Rascovsky [3] 2011 129 Definition of revised bvFTD criteria
Zhou [30] 2010 101 Description of functional networks in FTD
Seelaar [2] 2011 100 Review on FTD aspects
Gotz [31] 2008 83 Review on animal models in FTD
Skibinski [24] 2005 82 Identification of CHMP2B mutations
D’Souza [29] 1999 80 Identification of MAPT mutations
Year, publication year; TC, total citations; TC2, citations within first 2 years after the publication year; MAPT, Microtubule
Associated Protein Tau; GRN, Granulin; VCP, Valosin-Containing Protein; FTD, Frontotemporal Dementia; bvFTD,
behavioral variant FTD. Breakthroughs of each article is briefly reported.
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708 D. Guido et al. / Bibliometric Analysis in FTD
causing autosomal dominant FTD [16, 17, 22], but also
the more rare mutation within Valosin-Containing Pro-
tein (VCP) and CHMP2B genes [23, 24]. As expected,
the clinical criteria that progressively refined the
description of the different FTD phenotypes received
the highest number of citations [18, 25], as well as the
few available studies on FTD epidemiology [26]. Fur-
thermore, clinical-pathological [27] and neuroimaging
[28] studies related to FTD have been quoted in the
top ten papers with the highest citation index. Moving
from total number of citations to number of cita-
tions within two years from publication, we found
comparable results [16, 17, 22, 29]. However, more
recent papers were quoted, depicting the neuropatho-
logical hallmarks [19], the new clinical criteria [3], and
advanced neuroimaging techniques to detect FTD sig-
nature [30]. Moreover, among the most cited papers
within two years after publication, particular interest
was devoted to genotype-phenotype heterogeneity [2]
and animal models [31].
All together, these findings highlight many clinical,
genetic, and neuroimaging aspects of FTD, giving us a
broader understanding of the disorder. Therapeutics is
a major aspect missing in FTD literature, demonstrat-
ing that this is still an orphan disorder, and no significant
pharmacological interventions are available yet.
ACKNOWLEDGMENTS
The authors would like to thanks Mr. Robert Ellis
for editing the manuscript.
Authors’ disclosures available online (http://j-alz.
com/manuscript-disclosures/15-0275r1).
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