| |

Received: 16 March 2016 Revised: 30 June 2016 Accepted: 4 August 2016

DOI: 10.1002/brb3.557

ORIGINAL RESEARCH

Prevalence of progressive supranuclear palsy in Yonago: change

throughout a decade

Hiroshi Takigawa|Michio Kitayama|Kenji Wada-Isoe|Hisanori Kowa|

Kenji Nakashima

Division of Neurology,Department of
Brain and Neurosciences,Faculty of Abstract
Medicine,Tottori University, Tottori, Japan Background: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder
Correspondence that is sometimes confused with Parkinsons disease, multiple system atrophy, and
Hiroshi Takigawa, Division of Neurology, other disorders. The typical clinical features are categorized as Richardsons syndrome
Department of Brain and Neurosciences,
Faculty of Medicine, Tottori University, (RS), but other clinical subtypes include PSP-parkinsonism (PSP-P) and PSP-pure aki-
Tottori, Japan. nesia with gait freezing (PSP-PAGF). In this study, we determined the prevalence of
Email: takigawa@med.tottori-u.ac.jp
PSP in a Japanese rural area compared to our previous 1999 report.
Funding information Methods: We collected data in Yonago City from 2009 to 2014 using a service-based
This work was supported by a grant
from the Research Committee for CNS study of PSP. We collected case history data from PSP patients in the area from our
Degenerative Diseases from the Ministry of hospital. The crude prevalence and 95% confidence interval (CI) were calculated using
Health, Labor, and Welfare of Japan.
the population demographics on the prevalence day of 1 October 2010. Age-and sex-
adjusted prevalence was calculated by direct standardization to the population demo-
graphics in Yonago City on the prevalence day of 1 April 1999.
Material and Results: We identified 25 patients: 16 with probable RS, 4 with possible
RS, 3 with clinical PSP-P, and 2 with clinical PSP-PAGF. The prevalence per 100,000
was 17.90 (male=18.05; female=17.76). The prevalence of PSP in Yonago in 2010
increased compared to the measurements from 1999.
Conclusion: The prevalence of PSP in Japan increased from 1999 to 2010.

KEYWORDS
epidemiology, PSP-parkinsonism, PSP-pure akinesia with gait freezing, Richardsons syndrome,
tauopathy

1| INTRODUCTION syndrome [RS]) is clinically characterized by vertical gaze palsy or hy-

pometric vertical saccades and postural instability, with falls occur-
Progressive supranuclear palsy (PSP) was described in 1964 as a dis- ring in the first year, in combination with predominantly axial rigidity,
tinct neurodegenerative disorder with symptoms of supranuclear oph- dysarthria, dysphagia, and frontal behavioral abnormalities (Burn &
thalmoplegia mainly affecting vertical gaze, pseudobulbar palsy, and Lees, 2002; Williams & Lees, 2009). Recently, clinical subtypes, such
dystonic rigidity of the neck and upper trunk (Steele, Richardson, & as PSP-parkinsonism (PSP-P) and PSP-pure akinesia with gait freezing
Olszewski, 1964). Cytoskeletal tau aggregates are the pathophysio- (PSP-PAGF), have been described indicating that the various symp-
logical hallmark of PSP (Dickson, Ahmed, Algom, Tsuboi, & Josephs, toms have the same pathophysiological background (Williams, Holton,
2010; Perez etal., 1998). The typical phenotype of PSP (Richardsons Strand, Revesz, & Lees, 2007; Williams etal., 2005).

This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium,
provided the original work is properly cited.

Brain and Behavior 2016; 6: e00557 wileyonlinelibrary.com/journal/brb3 2016 The Authors. Brain and Behavior | 1
published by Wiley Periodicals, Inc.
 |
e00557 (2 of 5) TAKIGAWA etal.

Few epidemiological studies have been performed regarding PSP, Parkinsons disease, corticobasal degeneration, multiple system
PSP. In 1999, we reported the prevalence of PSP in Yonago City, the atrophy, and Parkinsons syndrome patients at the University Hospital.
western area of the Tottori Prefecture in Japan (Kawashima, Miyake,
Kusumi, Adachi, & Nakashima, 2004). In the previous survey, PSP was
2.3|Diagnosis
conceptually equivalent to RS. The subjects were the patients with
only RS according to the diagnostic criteria of the National Institute of The patients participated in an interview regarding their clinical in-
Neurological Disorders and Stroke and Society for PSP (NINDS-SPSP; formation, including symptoms, clinical course, past history, family
Litvan etal., 1996). The elderly portion of the population of Yonago history, a detailed neurological examination by board-certified neu-
City is presently increasivng. Accordingly, changes in the prevalence rologists, and more examinations (including a magnetic resonance
of PSP are predicted. Since 2003, PSP has been listed on the Specified imaging study and 123-I-meta-iodobenzylguanidine myocardial scin-
Disease Treatment Research Program of the Japanese government. tigraphy in the Division of Neurology at Tottori University Hospital).
Moreover, subtypes of PSP have been described during the decade RS was diagnosed according to the criteria of the NINDS-SPSP. We
since our previous investigation. In this study, we determined the researched in detail the clinical history of the patients who conformed
prevalence of PSP, including subtypes, in a Japanese rural area, Yonago to the criteria of possible PSP with symptoms of vertical supranuclear
City, and compared these data to our previous report in 1999. palsy or slowing of vertical saccades, but not prominent postural insta-
bility with falls occurring in the first year. Subtypes of PSP were clas-
sified according to the reports by Williams etal., (2005, 2007). PSP-P
2| PATIENTS AND METHODS
was clinically diagnosed if the patient demonstrated the clinical fea-
tures of asymmetric onset, tremor, and a moderate initial therapeutic
2.1|Area of investigation
response to levodopa. For patients with possible PSP, we diagnosed
The investigated area, Yonago City, is located in western Japan (35 cases with a Parkinsons disease-like clinical picture with a moderate
degrees north and 133 degrees east). The adjacent Yodoe Town (area, levodopa response in the first 2years as clinical PSP-parkinsonism
25.74km2; population, approximately 9,000) became part of the new (cPSP-P). For possible PSP, we diagnosed cases with gradual onset of
Yonago City as a result of a municipal merger on 31 March 2005. The gait freezing or speech impairment, an absence of limb rigidity, no de-
new Yonago City covers an area of 132.21km2, and the population mentia, or ophthalmoplegia during the first 5years, and no sustained
was 148,271 (70,133 males and 78,138 females) on 1 October 2010. response to levodopa as clinical PSP-pure akinesia with gait freezing
The area of the initial Yonago City was 106.47km2, and the population (cPSP-PAGF).
was 139,683 (66,483 males and 73,200 females) on 1 October 2010.
The population increased from 137,420 in 1999. The percentage of
2.4|Data analysis
individuals 65years and over increased from 18.3% in 1999 to 23.4%
in 2010. The percentage of individuals less than 15years old decreased The prevalence day was defined as the date when the number of PSP
from 16.0% in 1999 to 14.5% in 2010. The population demograph- patients became constant in terms of the change in initial registrations
ics of the Yonago area were received from the Yonago city office. In and deaths of the patients. The crude prevalence per 100,000 living
new Yonago City, there are three general hospitals, five rehabilitation people and the 95% confidence interval (CI) were calculated using the
hospitals, and one University Hospital with 33 board-certified neu- population demographics generated from the former Yonago City on
rologists. Most neurologists in this rural town received their train- the prevalence day of 1 October 2010. The prevalence of each clinical
ing as a neurologist at the University Hospital and continue to work PSP subtype was calculated for the same time. Age-and sex-adjusted
closely with the University Hospital, as such, the neurological medi- prevalence was calculated by direct standardization to the popula-
cal services in the area are quite efficient. Typical cases of Parkinsons tion demographics in Yonago City on the prevalence day of 1 April
disease or Alzheimers disease were occasionally diagnosed at the 1999 and in Japan on the prevalence day of 1 October 2010. In the
general hospital. However, most atypical cases or severe cases with present study, we used the same clinical diagnostic criteria for PSP
parkinsonism or motor symptom patients such as PSP, multiple sys- that was used in a previous report by Kawashima, etal., (2004). The
tem atrophy, corticobasal degeneration and amyotrophic lateral scle- prevalence of RS (probable RS and possible RS) and PSP (RS, cPSP-P,
rosis were referred to the University Hospital, where they underwent and cPSP-PAGF) were compared between the April 1, 1999 and
detailed examination and diagnosis. The subjects living in the area of October 1, 2010 prevalence days.
former Yonago City were analyzed according to the population demo- These studies were approved by the Ethical Review Board of the
graphics generated from the former Yonago City in order to use the Tottori University Faculty of Medicine.
same area for comparison with the prevalence of PSP in 1999.

3|RESULTS
2.2|Service-based study in Yonago City
The survey was performed six times over 6years from 2009 to 2014. In this investigation, we identified 25 patients with PSP: 16 with prob-
In each survey, case data were collected from the medical records of able RS, 4 with possible RS, 3 with cPSP-P, and 2 with cPSP-PAGF.
TAKIGAWA etal. |
e00557 (3 of 5)

During this investigation period, before the prevalence day of 1 22,000 patients suffer from PSP in Japan. The crude prevalence of
October 2010, one patient with probable RS died. After the preva- patients with RS per 100,000 people was 14.32 (male=10.53; fe-
lence day, 11 patients died. Three cases were confirmed pathologi- male=17.76), and the 95% CI was 9.2722.12 (male=5.1021.74;
cally as definite PSP. Table1 summarizes the characteristics of the female=10.3830.39). The age- and sex-adjusted prevalence of RS
three autopsied PSP cases. Case 1 was diagnosed as cPSP-P, case 2 per 100,000 people in Japan on the prevalence day of 1 October 2010
and case 3 were diagnosed as probable RS. The period from onset to was 13.80 (male=10.62; female=16.63), and the 95% CI was 13.60
diagnosis of PSP was 3.22.5years. Table2 summarizes the charac- 14.01 (male=10.3710.88; female=16.3216.95).
teristics of the 25 patients with PSP who were examined in this study. In this survey, no patients that referred to the University Hospital
The crude prevalence of PSP per 100,000 people was 17.90 with a classification of possible PSP were diagnosed with any of the
(male=18.05; female=17.76), and the 95% CI was 12.1226.42 other PSP subtypes, including PSP-corticobasal syndrome (PSP-CBS;
(male=10.3331.55; female=10.3830.39). The age- and sex- Williams etal., 2005), PSP-progressive non-fluent aphasia (PSP-PNFA;
adjusted prevalence of PSP per 100,000 people in Japan on the Williams etal., 2005), or PSP with cerebellar ataxia (PSP-C; Kanazawa
prevalence day of 1 October 2010 was 17.26 (male=18.14; fe- etal., 2009).
male=16.63), and the 95% CI was 17.0317.48 (male=17.81
18.48; female=16.3216.95). It was predicted that approximately
4|DISCUSSION
T A B L E 1 Pathologically confirmed patients
This is the first report to investigate the change (during a decade) in
Case 1 Case 2 Case 3
the prevalence of PSP using the same surveillance area in Japan. The
Gender Male Female Male prevalence of PSP per 100,000 people in Yonago City in 2010 was
Age 82 72 77 17.90 (male=18.05; female=17.76). Our present data indicated
Age of onset 71 70 76 that the prevalence of PSP per 100,000 people in Yonago City in-
Symmetry of onset Asymmetric Symmetric Symmetric creased compared to 1999 (Kawashima etal., 2004) in which it was
symptoms 5.82 (male=9.14; female=2.75), and the 95% CI was 1.789.86
Supranuclear + + + (male=1.8216.47; female=1.086.65) (Fig.1). In the comparison
ophthalmoplegia
for Yonago City, the age- and sex-adjusted prevalence per 100,000
Postural instability + + people in Yonago City on the prevalence day of 1 April 1999 was
with fall in the first
11.92 (male=11.66; female=13.25), and the 95% CI was 7.3819.25
year
(male=5.8323.33; female=7.0124.76). The number of patients
Levodopa response +
in the first 2years with PSP increased even if adjusted by age and sex. When includ-
ing only RS, the prevalence of PSP per 100,000 people in Yonago
NINDS-PSPS Possible PSP Probable PSP Probable PSP
criteria City was greater in 2010 compared to the 1999 study (Fig.1). The
Clinical diagnosis in cPSP-P Probable RS Probable RS age- and sex-adjusted prevalence per 100,000 people in Yonago
this survey City on the prevalence day of 1 April 1999 was 10.23 (male=6.74;
Pathological Definite PSP Definite PSP Definite PSP female=13.25), and the 95% CI was 6.1017.14 (male=2.7416.57;
diagnosis female=7.0924.76). The crude and adjusted prevalence of RS per
RS, Richardsons syndrome; PSP, progressive supranuclear palsy; cPSP-P, 100,000 people in Yonago City increased compared to 1999. The 95%
clinical PSP-parkinsonism. CI for the adjusted PSP prevalence rate in 1999 overlaps with that in

T A B L E 2 Number of patients with RS and PSP subtypes

Adjusted
Crude prevalence Adjusted
prevalence in Yonago prevalence Duration of Time to
in Yonago City in 1999 in Japan in Age Age at onset illness diagnosis
City (per (per 2010 (per MeanSD MeanSD MeanSD MeanSD
Subtype Case (M:F) 100,000) 100,000) 100,000) (year) (year) (year) (year)

RS 20 (7:13) 14.32 10.23 13.80 77.57.1 73.67.0 4.03.5 3.02.6

Probable RS 16 (4:12) 11.45 8.07 10.95 78.66.7 74.76.4 4.03.9 2.42.6
Possible RS 4 (3:1) 2.86 2.15 2.86 74.57.9 69.08.2 3.81.9 5.31.5
cPSP-P 3 (3:0) 2.15 1.52 2.03 85.011.3 76.07.1 7.75.8 3.50.6
cPSP-PAGF 2 (2:0) 1.43 1.07 1.43 87.54.9 76.01.4 11.07.1 6.00.0
Total 25 (12:13) 17.90 11.92 17.26 77.56.5 72.56.9 5.04.4 3.22.5

RS, Richardsons syndrome; PSP, progressive supranuclear palsy; cPSP-P, clinical PSP-parkinsonism; cPSP-PAGF, clinical PSP-pure akinesia with gait freezing.
e00557 (4 of 5) | TAKIGAWA etal.

PSP/RS in 1999 (Kawashima et al., 2003) The prevalence of PSP has also been reported in areas outside of
PSP in 2010 (present study) Japan. In several population-based studies in various countries, re-
RS in 2010 (present study)
ports of PSP prevalence vary from 1.0 to 6.5 per 100,000 people (Chio,
20
Magnani, & Schiffer, 1998; Golbe, Davis, Schoenberg, & Duvoisin,
1988; Nath etal., 2001; Schrag, Ben-Shlomo, & Quinn, 1999; Wermuth,
Joensen, Bunger, & Jeune, 1997), which is in agreement with the results
PSP/RS (per 100,000 population)

15
of our previous report: 5.82 per 100,000 people (Kawashima etal.,
2004). However, the prevalence of PSP in Kochi, Japan, reported by
10 Osaki, Morita, Kuwahara, Miyano, & Doi (2011) was 18 per 100,000
people, and the age-and sex-adjusted prevalence per 100,000 people
in Japan was 10 per 100,000 with a 95% CI of 217. These subjects had
5
RS, but the authors did not include patients with PSP-P or PSP-PAGF.
In that study, the crude prevalence of RS was higher compared to our
0 data. However, when adjusted for age and sex, the adjusted prevalence
Crude prevalence rate Age- and sex-adjusted
prevalence rate was lower compared to our data. This indicates that the difference
between the two studies may be due to the proportion of the
F I G U R E 1 The prevalence of progressive supranuclear (PSP) population in the investigated area and the survey method.
and Richardsons syndrome (RS). Crude and age-and sex-adjusted
We observed almost identical rates for both sexes (12 males and 13
prevalences of PSP/RS in 1999, PSP in 2010, and RS in 2010
(adjusted to the population of Yonago City on the prevalence day of 1 females), which was inconsistent with our previous report (6 male vs.
April 1999). PSP was conceptually equal to RS in 1999. The subjects 2 female). The sex-related difference was not consistent in reports by
in 1999 included only patients with RS according to the diagnostic other investigators. Williams etal. (2005) investigated 103 pathologi-
criteria of the NINDS-SPSP cally confirmed cases of PSP, and 65 (63%) were males. In Santacruz,
Uttl, Litvan, and Grafman (1998), female patients with PSP were repre-
2010, and the point estimation prevalence in 2010 (17.26 per 100,000) sented equally (154 males, 164 females) in a sample of living individu-
falls within the adjusted 95% CI (7.3819.25) in 1999. Thus, although als, whereas men formed 61.8% in a sample (73 males, 45 females) of
the point prevalence estimation increased from 1999 to 2010, we can- deceased patients. Male patients were diagnosed later after symptom
not be 95% confident that this estimate is significantly higher than the onset than female patients (males, 33.4months; females, 24.1months)
adjusted estimate for 1999. This problem reflects the small sample size and had more severe symptoms and a shorter duration from diagno-
of PSP patients and affects the interpretation of this study. sis to death (males, 37.0months; females, 47.6months). In contrast,
Several factors may influence the prevalence of PSP. First, an increase dellAquila etal. (2013) reported no differences regarding time to
in the elderly proportion of the population of Yonago City could contrib- diagnosis, disease duration, or survival between males and females in
ute to changes in the prevalence of PSP. Second, PSP was certified as part a clinical investigation of 43 PSP patients (53.5% males). The results of
of the Specified Disease Treatment Research Program by the Japanese previous reports are therefore discrepant. The population of females
government in 2003. Patients with PSP were then able to get support increased relative to the change in the elderly proportion in Yonago City,
for medical services (at the publics expense). Thus, more PSP patients and the time to diagnosis for male patients was longer than for female
visited hospitals and were diagnosed. Additionally, a nursing-care insur- patients. The sex difference in patients with PSP thus remains unclear.
ance system was introduced in 2000 as a social security system corre- There were some limitations to this study. Most patients received
sponding to the aging societies in Japan. These changes in the social clinical diagnoses of PSP, and only three cases were confirmed patholog-
security and medical systems in Japan might improve nursing care and lead ically. The subjects were recruited according to the NINDS-PSPS criteria,
to an increased recognition and diagnosis of patients with PSP, thereby and possible RS cases were classified as a clinical subtype. The cases that
influencing the prevalence. Third, during the decade since the previous failed to meet the NINDS-PSPS diagnosis criteria were eliminated in this
investigation, some PSP subtypes, such as PSP-P and PSP-PAGF, were survey. Clinical diagnoses of PSP-P or PSP-PAGF were not allowed due to
defined. Patients with PSP subtypes presented with the specific patholog- the lack of clinical criteria, therefore in this study we provide new criteria
ical PSP findings but with different clinical manifestations. In some cases, it for cPSP-P and cPAGE. Further investigation will be required to deter-
might be difficult to diagnose patients who are in the early phases of PSP mine the accuracy of these clinical criteria using confirmed pathological
subtypes. The long-term investigation in this study might make it easier to cases. In this study, the participants were diagnosed via detailed neuro-
recognize the subtypes. The prevalence of RS in 2010 was higher than the logical examinations by multiple neurological specialists at the University
age-and sex-adjusted prevalence of PSP in Yonago City on the prevalence Hospital and were evaluated via magnetic resonance imaging of the
day of 1 April 1999. The overall increase in prevalence was not solely due brain and by 123-I-meta-iodobenzylguanidine myocardial scintigraphy,
to the appearance of subtypes, such as PSP-P and PSP-PAGF. Although which effectively differentiates PSP from Parkinsons disease (Braune,
the other subtypes, such as PSP-CBS (Williams etal., 2005), PSP-PNFA Reinhardt, Schnitzer, Riedel, & Lucking, 1999; Orimo, Ozawa, Nakade,
(Williams etal., 2005), and PSP-C (Kanazawa etal., 2009), have been Sugimoto, & Mizusawa, 1999; Rascol & Schelosky, 2009). Therefore, the
reported, no patients were diagnosed with these subtypes in this study. diagnostic accuracy was high. This study was an investigation conducted
TAKIGAWA etal. |
e00557 (5 of 5)

through medical institutions, and some patients with PSP were not able and clinical ascertainment of cases. Movement Disorders, 13,
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Neuropathology of variants of progressive supranuclear palsy. Current
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door-to-door study. Epidemiologic problems, such as immigration to
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(2004). Prevalence of progressive supranuclear palsy in Yonago, Japan.
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PSP is currently difficult to diagnose. Most patients who were diagnosed Litvan, I., Agid, Y., Calne, D., Campbell, G., Dubois, B., Duvoisin, R. C., ... Zee,
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