**1. Introduction**

104 Neuromuscular Disorders

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In Japan, 27 hospitals specialize in treatment of muscular dystrophy patients, including inpatient care, of which 26 belong to the National Hospital Organization, and the other is the National Center of Neurology and Psychiatry. Since 1999, Japanese muscular dystrophy research groups investigating nervous and mental disorder have been developing a database of cases treated at these 27 institutions. In that regard, we conducted a survey of inpatients and home-mechanical ventilation patients (HMV patients) with muscular dystrophy and other neuromuscular disorders based on data collected by the National Hospital Organization and National Center of Neurology and Psychiatry.

Herein, we examined data obtained in order to evaluate efficacy of mechanical ventilation therapy for HMV patients and mechanical ventilation-dependent inpatients (MV inpatients) with those wards.

### **2. Subjects and methods**

The database includes numbers of inpatients, gender, age, diagnosis, respiratory condition, nutritional state, number of death cases, causes of death, and other relevant findings from data collected annually on October 1 every year since 1999. Additionally we collected the data of HMV patients from 27 institutes for this study.

By using the database and newly collected HMV data, we analyzed the courses of HMV patients group and those of MV inpatients of wards. We compared data of these two groups. Examination points are mechanical ventilation periods, outcome of these two groups, and caregiver for HMV patients.

#### **2.1 Objective diseases**

Objective diseases of this study were muscular dystrophy and spinal muscular atrophy, in particular Duchenne muscular dystrophy and myotonic dystrophy. Amyotrophic lateral sclerosis was not included.

Comparison Between Courses of Home and Inpatients

spinal muscular atrophy, and so on (Table 1-1).

**3.1.2 MV inpatients group** 

course.

Mechanical Ventilation in Patients with Muscular Dystrophy in Japan 107

MV inpatients group included 915 inpatients. Gender was male: 718, female: 197. The number of representative disease were as follows; 476 Duchenne muscular dystrophy, 222 myotonic dystrophy, 35 Becker muscular dystrophy, 58 limb-girdle muscular dystrophy, 19

The range of mechanical ventilation introduction age for HMV patients was 6.3~72.8 years old (mean 25.9), and that of MV inpatients was 0.0~78.0 years old (mean 33.2). The number of NPPV introduction cases of HMV patients was 420, and that of MV inpatients was 517 (Table 1-2). Fifty of NPPV cases of HMV group were switched to tracheostomy during the

We performed survival analysis of those two groups. The endpoint for HMV patients was death or transition to hospitalization, and that for MV inpatient was death. Kaplan-Meier analysis showed that 75% life time of HMV patients was 1,689 days, while that of inpatients

Fig. 1. Comparison between HMV Patients and Mechanical Ventilation Inpatients (Total)

Endpoint for HMV patient: death or transition to hospitalization

Endpoint for MV inpatient: death

**3.1.3 Mean age at starting mechanical ventilation and type of ventilation** 

Table 1-2. Mean age at starting mechanical ventilation and type of ventilation

**3.2 Survival analysis of HMV patients group and MV inpatients** 

was 2,988 days (Log Rank (Mantel-Cox) p<0.01) (Fig. 1).

### **2.2 Patients introduced HMV after 1999**

The data which we requested 27 institutes specializing muscular dystrophy care was as follows; the number of patients introduced HMV after 1999, diagnosis of disease, gender, age at being introduced HMV, type of mechanical ventilation, such as non-invasive positive pressure ventilation (NPPV) or tracheostomy intermittent ventilation (TIV), present status, death cause for death case, main caregiver, and so on.
