Psychiatry and Clinical Neurosciences (1995), 49, 169-174

Regular Article

Epidemiology of inflammatory neurological and

inflammatory neuromuscular diseases in Tottori
Prefecture, Japan
MASAYOSHI KUSUMI, MD,' KENJI NAKASHIMA,
KAZURO TAKAHASHI, MD'

MD,'

HIDEAKI NAKAYAMA,

'Division of Neuroha, Institute ofNeurologica1 Sciences, 2Department of Hygiene, Faculty

Japan

MD'

AND

of Medicine, Tottori University, Yonago,

Abstract

We investigated the incidence of the following conditions: inflammatory neurological and neuromuscular
diseases, adult meningitis and adult encephalitis in Yonago City, and Guillain-Barre syndrome (GBS),
chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), polymyositis/dermatomyositis (PM/
DM), periarteritis nodosa (PN) and HTLV-1 associated myelopathy (HAM) during the period 1988-1992 in
Tottori Prefecture, Japan. The annual incidence per 100 000 population was as follows: meningitis, 4.38;
encephalitis, 0.90; GBS, 1.14; PM/DM, 1.01;and PN, 0.32. The prevalence per 100 000 population CIDP,
0.81; PM/DM, 9.92; PN, 2.59; and HAM, 1.30. There was marked localization of HAM in western
Tottori, and there was seasonal variation in the prevalence of meningitis, encephalitis and GBS. The mean
age at onset of meningitis was lower than that for encephalitis. Comparison with reported data revealed
interracial differences in the epidemiology of PM/DM and PN.

Key words

chronic inflammatory demyelinating polyradiculoneuropathy, encephalitis, Guillain-Barrt syndrome,

HTLV-1 associated myelopathy, meningitis, periarteritis nodosa, polymyositis/dermatomyositis.

INTRODUCTION
The inflammatory neurological and inflammatory neuromuscular diseases commonly seen at neurological hospitals
in Japan include meningitis, encephalitis, Guillain-Barre
syndrome (GBS), chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP), polymyositis/dermatomyositis (PM/DM), periarteritis nodosa (PN) and HTLV-1
associated myelopathy (HAM). However, there are only a
few reports regarding the epidemiology of these conditions
We therefore carried out an epidemiological
in
study of the incidence of these seven inflammatory neurological and inflammatory neuromuscular diseases in Tottori
Prefecture,Japan, during the 5 year period 1988-1992. The
aims of this study were to clarify the epidemiology of these
diseases in a representative area of Japan and to compare the
prevalence of these diseases in Japan with that in other
various countries.

Correspondence address: Masayoshi Kusumi, MD, Division of Neurology,

Institute of Neurological Sciences, Faculty of Medicine, Tottori University, 86
Nishimachi, Yonago, Tottori 683, Japan
Received 4 November 1994; revised 7 December 1994; accepted 19 December 1994.

MATERIAL AND METHODS

Geographical area of investigation
We carried out a retrospective study of the incidence of adult
meningitis and adult encephalitis in Yonago City (one of
four cities in Tottori Prefecture, population 62 795 males
and 69 372 females as at 1 April 1992), and the incidence of
GBS, CIDP, PM/DM, PN and HAM in Tottori Prefecture
(population 293 894 males and 320 831 females as
at 1 April 1992), during the 5 year period from 1 February
1988 to 31 December 1992. Tottori Prefecture (area
3492.7 km2) is located in western Japan. The mean yearly
temperature is 15.1"C(range - 2.1-36.9") and the average
annual rainfall is 18 18 mm. The average annual amount of
sunshine is 2100 h. The mobility of the population of
Tottori Prefecture is low, especially among older residents.
According to the general census, 53.1Yo of the population
were engaged in commerce, 16.1% in agriculture, and
30.8% in industry. There are ample medical facilities,
notably 25 board-certified neurologists, serving the prefecture, and we were therefore confident that we could carry
out a neuroepidemiological study in this area without overlooking any patients with neurological and neuromuscular

condition^.^.^

M. Kusumi et

170

a/.

Epidemiological data collection

Cases of each conditions at the University Hospid in
Yonago City were identified by examination of the patient
records. Cases at the general hospitals were identified by
questionnaires sent to these hospitals. When no response to
our inquiry was obtained, we telephoned the hospitals
directly and asked them to send reports to us or to allow US
direct access to the patient records at the hospital. Only adult
cases of meningitis and encephalitis were included in the
study; these conditions are very common in children and it
would have been very difficult to investigate their incidence
in this group.
Diagnosis
The diagnoses of meningitis and encephalitis were made on
the basis of clinical findings, such as high fever, headache,
stiff neck, or consciousness disturbance, the findings for
cerebrospinal fluid, and the imaging findings for computed
tomography (CT) or magnetic resonance imaging (MRI).
The diagnosis of each condition was based.on the standard
criteria of the following organizations of researchers: GBS,
National Institute of Neurological and Communications
Disorders and Stroke Ad Hoc Committee; CIDP, Ad Hoc
Subcommittee of the American Academy of Neurology
AIDS Task Force;* PM/DM, B ~ h a n PN,
; ~ the American
College of Rheumatology;lo and HAM, World Health
Organi~ation.~
The diagnoses in all cases were confirmed by
neurologists. In the analysis of the incidence of PM/DM and
PN, we obtained diagnoses according to the written applications for medical aid for specific incurable diseases in Tottori
Prefecture. We calculated the prevalence of CIDP, PM/
DM, PN and HAM as at 31 December 1992, and the
incidence of meningitis, encephalitis, GBS, PM/DM and
PN during each year from 1988 to 1992.
Statistical evaluation was performed using the Students
t-test.

r-

x
City

Toltori C i t t h

Kurayoshi City

Figure 1. Map of Japan showing the location of Tottori Prefecture

and map of the Prefecture showing the four principal cities.

City. The average incidence of meningitis was 4.38 k 2.52

per 100 000 population per year (Table 1). The yearly
incidence ranged from 1.51 in 1988, to 8.30 in 1991. In
most cases of meningitis, the onset was in the early Spring
and Autumn (Fig. 2). The peak age at onset of meningitis in
males was younger than that in females (Fig. 3); there was a
significant difference between the mean age at onset in
males (30.4 f 16.36 years) and that in females (30.4 & 16.36,
39.7 f 14.32 years, respectively; P < 0.05). The type of
meningitis was aseptic (no virus ascertained as the origin) in
72.4%, viral in 17.2%, bacteria1 in 3.496, and microbacterial
in 7.0%.

RESULTS
Meningitis
During the period 1988-1992,29 patients (16 males and 13
females) were diagnosed as having meningitis in Yonago

Table 1. Distribution of the onset of meningitis and encephalitis during the period 1988 to 1992 in Yonago City
Meningitis
Year

Population

No. patients

Encephalitis

Incidence per
100 000 per year

Incidence per
No. patients

100 000 per year

1988

131 773

1.52

0.00

1989

132 078

4.54

1.51

1990

132 361

6
4

3.02

1.51

1991

132 598

11

8.30

0.00

1992

132 957

4.51

1.50

Epidemiology of diseases in Japan

171

(Fig. 5). The peak age at onset was 20-29 years in males and
60-69 years in females (Fig. 6). The mean age at onset was
46.3 20.37 years (male, 37.7 k 19.93; female, 52.7 -t
18.70). The preceding symptoms, such as upper respiratory
infections, were seen in 51.4% of the cases. There was no
geographical localization of the incidence within the Prefecture.
Jan Feb Mar Apr May Jun

Jul Aug Sep OC: Nov Oec

Figure 2. Monthly incidence of meningitis in Yonago City.

Chronic inflammatory demyelinating

polyradiculoneuropathy
This was diagnosed in five patient (4 males and 1 female).
The prevalence of CIDP was 0.81 per 100 000 population
(Table 3).

Polymyositis/dermatomyositis

15-

21-

31-

51-

41-

61-

71-

Sixty-one patients (17 males and 44 females) with PM/DM

were identified. The prevalence was 9.92 per 100 000
population. Thirty-one patients (9 males and 22 females)
were newly diagnosed during the period. The average yearly
incidence of PM/DM was 1.01 f 0 . 5 6 per 100 000 popula-

Age (years)
Figure 3. Age at onset of meningitis. (m) male; (0)female.

8
7

Encephalitis
During the same period (1988-1992), six patients (three
males and three females) wree diagnosed as having encephalitis in Yonago City. The average yearly incidence of
encephalitis was 0.90 +- 0.83 per 100 000 population. In
most of the encephalitis cases, the onset was in the Autumn
or Winter (Fig. 4). The mean age at onset was 59.2 f8.86
years (male, 61.3 f9.00; female, 57.0 j, 10.1).The origin of
encephalitis was HSV Type 1 in four of the patients.

=
5
a,

.-

z 4
a

z 2
1

0
Jan Feb Mar Apr May Jun

Jul Aug Sep Oc:

Nov Df?C

Figure 5. Monthly incidence of Guillain-Barre syndrome in Tottori

Prefecture.

Guillain-Barre syndrome
During the same period (1988-1992), 35 patients (15 males
and 20 females) were diagnosed as having GBS in Tottori
Prefecture. The average yearly incidence of GBS was
1.1420.12 per 100 000 population (Table 2). The yearly
incidence ranged from 0.97 in 1989, to 1.30 in 1990. In
most cases of GBS, the onset was in early Spring or Winter

n
m 2 /

0-4

10-

20-

30-

40-

50-

60-

70-

Age (years)

n
"
Jan Feb Mar

Apr May Jun

Jul Aug Sep Oc: NOV Dec

Figure 4. Monthly incidence of encephalitis in Yonago City.

Figure 6. Age group at onset of Guillain-Bme syndrome. The peak

age in males (m) was younger than that in females (0).

M. Kusumi et al.

172

Table 2. Distribution of the onset of Guillain-Barre syndrome (GBS), polymyositis/dematomyositis (PM/DM) and periarteritis nodosa (PN) during
the period 1988-1992 in Tottori Prefecture
GBS

PM/DM
Incidence per

PN
Incidence per

Incidence per

100 000 per year

No. patients

100 000 per year

No. patients

100 000 per year

1.13
0.97
1.30
1.14
1.14

5
6
12
3
5

0.81
0.97
1.95
0.49
0.81

6
8
7

0.00
0.32
0.81
0.32
0.16

Year

Population

No. patients

1988

618 000

1989
1990
1991
1992

618 000
615 741
616 000
614 410

2
5
2
1

Table 3. Number of patients diagnosed with prevalence of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), polymyositis/
dermatomyositis (PM/DM), periarteritis nodosa (PN), HTLV-1 associated myelopathy (HAM) (Tottori Prefecture)

Disease

No.
male

Prevalence per
100 000 population

No.
female

Prevalence per
100 000 population

Total

Prevalence per
100 000 population

CIDP
PM/DM
PN
HA

4
17
8
3

1.36
5.76
2.71
1.01

1
44
8
5

0.31
13.71
2.49
1.56

5
61
16
8

0.81
9.92
2.59
1.30

tion. The yearly incidence ranged from 0.48 in 1991 to 1.94

in 1990. The mean age at onset was 52.58k21.30 years
(males, 53.3 f 20.63; females, 52.3 -+ 22.03). The onset
showed two peaks, namely in children and elderly (Fig. 7).
There was no geographical localization of the incidence
within the Prefecture.

0.32 f 0.31 per 100 000 population. The yearly incidence

ranged from 0 in 1988 to 0.81 in 1990. The mean age at
onset was 55.3 f 19.69 years (males, 61.0 f 19.87; females,
51.5k20.44). The onset showed two peaks, namely in
young adults and the elderly (Fig. 8). There was no geo-

Periarteritis nodosa

m V - 1 associated myelopathy

Sixteen patients (8 males and 8 females) with PN were

identified. The prevalence was 2.59 per 100 000 population.
Ten patients (4 males and 6 females) were newly diagnosed
during the period. The average yearly incidence of PN was

Eight patients (3 males and 5 females) with HAM were

identified. The prevalence was 1.30 per 100 000 population.
There was a marked geographical localization of cases of
HAM in the western area of Tottori Prefecture (Fig. 9).

graphical localization of the incidence within the Prefecture.

5 r

0-

10-

20-

30-

40-

SO-

60-

73-

0-

Age (years)
Figure 7. Age at onset of polymyositis/dermatomyosis. (m) male;
(qfemale.

11-

21-

31-

41-

Age (years)
Figure 8.

Age at onset of periarteritis nodosa.

51-

61-

71-

kpidemiology ot diseases in Japan

Figure 9. The location of residence of the patients with HTLV-1

associated myelopathy.

DISCUSSION
The incidence and prevalence of meningitis and encephalitis
are high in children, but not in adults. Therefore in Japan,
there are many epidemiological reports of these diseases in
children, but few in adults.2 Beghi reported that the incidence of meningitis and encephalitis in Olmsted County in
individuals over the age of 10 years was 1.4-15.8 and
1.8-6.3, respectively,per 100 000 population per year;" the
same rates in our study was 4.38k2.52 and 0.90k0.83,
respectively. In adults, the incidence of meningitis and
encephalitis in Japan is similar to that in other countries.
The incidence of GBS in different areas of the world
ranges from 0.4 to 2.2 per 100 000 population per ear,'^-'^
and the 1.14 incidence in Tottori Prefecture is within this
range.
The prevalence of PM/DM found in our study were
higher than those reported for Kumamoto City and Kumamoto Prefecture.' The methodology used in the Kumamoto study, in contrast to our methodology, may have
excluded data from some of the hospitals in the area, and the
results of that study thus may not mirror the actual prevalence in Kumamoto. Medsger reported that the incidence of
polymyositis was 0.12-0.84 per 100 000 population per
year, and that the incidence in black people was higher than
that in white pe0p1e.l~Our data indicate that the incidence
in Japanese is higher than that in white people and similar to
that in black people.
Epidemiological reports regarding PN in Japan or elsewhere are rare. In Rochester, USA, the reported incidence is
0.7 to 1.8 per 100 000 population per year and the prevalence 6.3 per 100 000 population.16 This suggests that these
values are slightly higher than those in Japan. Our study
revealed higher incidence of PN than that reported in an
epidemiological study of P N in Japan." This discrepancy is
due to differences in methodology; in the latter study the
incidence was extrapolated based on the written applications
for medical aid for specific incurable diseases.
We observed a marked geographical localization of HAM
in western Tottori Prefecture. The prevalence of HAM
among HTLV-1 carriers in Japan aged 20 to 69 years is 65.7
per 100 000 population for males and 86.9 per 100 000 for

1-/3

females.'* The nationwide survey of HAM also indicated its

geographical localization in Japan;3 in addition, the localization in single prefecture suggests that HTLV-1 infection
shows clustering and that the spread of infection follows the
coast. Sakaiminato City, one of the largest fishing ports in
Japan, is situated in western Tottori. This city has extensive
commerce with the Kyushu area, where there are many
patients with HAM.'*
We observed seasonal variation in the onset of meningitis
and encephalitis. Yamashita et al. found that the number of
aseptic meningitis cases due to enterovirus infections increase, to a variable degree each Summer, with peak incidence usually observed in
However, our study
indicated two incidence peaks; early Spring and Autumn.
The study by Yamashita et al. included only pediatric cases,
while our study included only adult cases. This discrepancy
suggests that the causative virus infecting the central nervous
system in adult cases of meningitis and encephalitis differs
from that in pediatric cases. With GBS onset, we observed
that Campylobacter jejuni (one of the causative organisms
for GBS) has a seasonal difference of infection.20
The lower mean age at onset for meningitis compared
with that for encephalitis may have been due to the reduction of the function in the blood brain barrier by ageing. In
meningitis and GBS, the mean age in males at onset was
younger than that in females, which suggests that the
immune functions and sensitivity to these conditions are
different in the sexes. There were two incidence peaks at
onset in PM/DM. This was considered the result of the
differences in immune function and sensitivity to the disease
revealed by ageing and in the associationwith malignancy.21
In conclusion, our study clarified the epidemiological
characteristics of these inflammatory neurological and inflammatory neuromuscular diseases in Japan.

ACKNOWLEDGMENTS
The authors wish to thank the medical doctors in Totton
Prefecture, Mrs Tanaka, Division of Health Promotion and
Disease Control, Deputation of Welfare and Health, Tottori
Prefectural Office and Dr Oshiro, Department of Public
Health, Faculty of Medicine, Tottori University for having
made this work possible.
This study was supported in part by a Grant for Nervous
and Mental Disorders (No. 3A-3), from the National Center
of Neurology and Psychiatry, the Ministry of Health and
Welfare, Japan.

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