Kusumi - Epidemiology of Inflammatory Neurological and Inflamatory Neuromuscular Diseases in Tottori, Japan

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

Regular

Article

Epidemiology

of

inflammatory neurological and

inflammatory neuromuscular diseases in Tottori

Prefecture, Japan

M A S A Y O S H I K U S U M I ,

MD,

KE NJI NAKASHI M A,

MD,

HI DE AKI NAKAYAMA,

MD'

A N D

K A Z U R O T A K A H AS H I,

MD'

Division ofNeuroha, Institute ofNeurologica1 Sciences, 2D epartmen t ofHygiene, Faculty of Medicine, Tottori University, Yonago,

Japan

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.

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

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

Key words

INTRODUCTION

The inflammatory neurological and inflammatory neuro-

muscular diseases commonly seen

at

neurological hospitals

in Japan include meningitis, encephalitis, Guillain-Barre

syndrome (GBS), chronic inflammatory demyelinating

polyradiculoneuropathy (CIDP), polymyositis/dermato-

myositis (PM/DM), periarteritis nodosa (PN) and HTLV-1

associated myelopathy (HAM). However, there are only a

few reports regarding the epidemiology of these conditions

in

We therefore carried out an epidemiological

study of the incidence of these seven inflammatory neuro-

logical 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

M D

Division

of

Neurology

Institute of Neuro logical Sciences Faculty

of

Medicine T ottori Univer sity 86

Nishimachi Yonago Tottori 683, Japan

Received 4 November

1994;

revised 7 December

1994;

accepted

19

Decem-

ber 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, 53.1 o 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 prefec-

ture, and we were therefore confident that we could carry

out a neuroepidemiological study in this area without over-

looking any patients with neurological and neuromuscular

condition^.^.^


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170

M . Kusumi et

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 ; ~N, the American

College of Rheumatology;lo and HAM, World Health

Organi~ation.~he diagnoses in all cases were confirmed by

neurologists. In the analysis of the incidenceof PM/DM and

PN, we obtained diagnoses according to the written applica-

tions 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 Student’s

t-test.

RESULTS

Meningitis

During the period 1988-1992,29 patients (16 males and 13

females) were diagnosed

as

having meningitis in Yonago

r- City Toltori

C i t t h

Kurayoshi City

Figure 1

and map of the Prefecture showing the four principal cities.

Map of Japan showing the location of Tottori Prefecture

City. The average incidence of meningitis was 4.38 k2.52

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 16.36 years) and that in females (30.4 16.36,

39.7 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%.

Table 1 Distribution of the onset

of

meningitis and encephalitis during the period 1988 to 1992 in Yonago City

Meningitis Encephalitis

Incidence per Incidence per

Year Population

No.

patients

100 000

per year No. patients

100

000 per year

1988 131 773

1989 132 078

1990 132 361

1991 132 598

1992 132 957

2

6

4

11

6

1.52

4.54

3.02

8.30

4.51

0.00

1.51

1.51

0.00

1.50


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Epidemiology of diseases in Japan

171

Jan Feb Mar Apr May

Jun Jul

Aug

Sep

OC:

Nov Oec

Figure 2.

Monthly incidence of meningitis in Yonago City.

15-

21- 31-

41- 51- 61-

71-

Age years)

Figure

3. Age at onset of meningitis.

m)

male;

0)

emale.

Encephalitis

During the same period (1988-1992), six patients (three

males and three females) wree diagnosed as having enceph-

alitis 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 8.86

years (male, 61.3 9.00; female, 57.0 j

10.1).

The origin of

encephalitis was HSV Type 1 in four

of

the patients.

Guillain-Barre syndrome

During the same period (1988-1992), 35 patients (15 males

and 20 females) were diagnosed

as

having

G S

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

2 /

z

n

(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, 37.7k 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 Prefec-

ture.

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

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

were identified. The prevalence was

9.92

per 100000

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 .56 per

100

000 popula-

8

7

6

= 5

z

0 3

m

a

z

.

2

1

0

Jan Feb Mar Apr May Jun Jul Aug Sep Oc: Nov Df?C

Figure 5.

Prefecture.

Monthly incidence of Guillain-Barre syndrome in Tottori

Jan Feb Mar Apr May Jun

Jul

Aug Sep

Oc: NOV

Dec

Monthly incidence of encephalitis in Yonago City.

igure

4.

0 4

10-

20

n

n

30 40

50

60 70

Age years)

Figure 6.

age in males m) was younger than that in females

0).

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


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M. Kusumi

et al.

172

Table 2.

the period 1988-1992 in Tottori Prefecture

Distribution of the onset of Guillain-Barre syndrome (GBS),polymyositis/dematomyositis (PM/DM) and periarteritis nodosa (PN) during

GBS PM/DM PN

Incidence per

ncidence per Incidence per

Year Population No. patients

100

000 per year No. patients

100

000 per year No. patients

100

000 per year

1988 618

000

7 1.13 5 0.81 0

0.00

1989 618 000

6 0.97

6 0.97 2 0.32

1990 615 741

8 1.30

12 1.95 5 0.81

1991 616

000

7

1.14

3 0.49 2 0.32

1992 614 410 7 1.14

5 0.81 0.16

Table

3.

dermatomyositis (PM/DM), periarteritis nodosa

(PN), HTLV-1

associated myelopathy

(HAM) (Tottori

Prefecture)

Number of patients diagnosed with prevalence of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), polymyositis/

No. Prevalence per No. Prevalence per Prevalence per

Disease male

100

000 population female

100

000 population

Total 100000

population

~

CIDP

4 1.36 1 0.31 5 0.81

PM/DM

17 5.76

44 13.71 61 9.92

PN 8 2.71

8 2.49 16 2.59

HA

3 1.01

5 1.56 8 1.30

tion. Th e yearly incidence ranged from 0.48 in 1991to 1.94

in 1990. The mean age

a t

onset was 52.58k21.30 years

(males, 53.3 f 0.63; 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.

Periarteri t is

nodosa

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

0-

10-

20 30

40- SO 60

73-

Age years)

Figure 7 Age at onset of

polymyositis/dermatomyosis

m) male;

q

emale.

0.32

f

.31 per 100 000 population. The yearly incidence

ranged from

0

n 1988 to 0.81 in 1990. The mean age at

onset was 55.3

f

9.69 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-

graphical localization of the incidence within the Prefecture.

m V - 1 associated myelopathy

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).

5 r

0- 1 1 - 2 1 - 3 1 - 41-

51-

61- 71-

Age years)

Figure

8

Age at onset of periarteritis nodosa.


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kpidemiology

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Figure 9.

associated myelopathy.

The location of residence of the patients with HTLV-1

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 inci-

dence 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 Ku-

mamoto Prefecture.' The methodology used in the Ku-

mamoto 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 preva-

lence 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 else-

where are rare. In Rochester, USA, the reported incidence is

0.7 to 1.8 per 100

000

population per year and the preva-

lence

6 . 3

per

100000

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

PN 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

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

geographical localization in Japan;3 in addition, the localiza-

tion 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 in-

crease, to a variable degree each Summer, with peak inci-

dence 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 reduc-

tion 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 association with malignancy.21

In conclusion, our study clarified the epidemiological

characteristics of these inflammatory neurological and in-

flammatory 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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Kusumi - Epidemiology of Inflammatory Neurological and Inflamatory Neuromuscular Diseases in Tottori, Japan