**4. Conclusion**

112 Neuromuscular Disorders

**3.5 Outcome of HMV patients and MV inpatients with Duchenne muscular dystrophy** 

One hundred ninty four cases with Duchenne muscular dystrophy among 262 cases continued HMV, while 46 cases with myotonic dystrophy among 60 cases continued HMV (Table 8). Twenty two cases with Duchenne muscular dystrophy were switched to hospitalization.

**Continuing HMV 194 - 46 -**

**Death 29 67 6 56**

**institution 10 - - - Withdrawing MV - - 3 - Unknown 7 2 - - Others - - 2 15 total 262 476 60 222**

Caregivers for most of HMV patients with Duchenne muscular dystrophy were patients' families (Table 9). In particular, patients' mothers were playing important role in continuing HMV. Similarly, caregivers for HMV patients with myotonic dystrophy were patients'

> **Caregiver DMD MD Mother 94 6**

**Father 3 2**

**Husband 2 Wife 1 Sister 1 daughter 1 foundation 2 Helper 2 2 (Response) (134/262) (17/60)**

Table 9. HMV-continuing cases main caregiver (Duchenne muscular dystrophy and

**Continuing hospitalization - 407 - 151**

**Transition to hospitalization 22 - 3 -**

Table 8. Outcome (Duchenne muscular dystrophy and myotonic dystrophy )

families. On reflecting their age, some caregivers were patient' spouse (Table 9).

**Parents 22**

**Family 3 Mother/sibling 1 Mother/uncle 1 Mother/grandmother 1 Grandmother 1**

**Introduction to other** 

**3.6 Caregivers for HMV patients** 

myotonic dystrophy)

**HMV Inpatient HMV Inpatient**

**DMD MD**

**and myotonic dystrophy** 

Approximate 2500 beds for patients with muscular dystrophy and related disorders are now provided among 27 institutions in Japan. In accordance with progress in therapeutic strategies for respiratory failure (American Thoracic Society Documents, 2004) and heart failure (Ishikawa, 1999; Matsumura, 2010), the life span of patients with muscular dystrophy prolonged (Bushby 2010a, b). Now, most inpatients admitted to muscular dystrophy wards have severe general conditions and many are assisted by mechanical ventilation (Tatara, 2006; 2008), which is accordance with our data of MV patients in this study.

In recent two decades, social welfare systems and home medical care systems in Japan have been changing gradually. HMV has been penetrating into home medical care (Joseph, 2007). The number of HMV patients has been increasing (Tatara, 2006). Stable mechanical ventilated patients have been getting back home.

Our study demonstrated that the course of HMV patients was fairly good, although there was difference between Duchenne muscular dystrophy and myotonic dystrophy in long term outcome. However, the support system for patients and caregivers is not perfect. Our study also showed that burden of caregivers was supposed to be severe. The system for patients and caregivers should be adjusted (Dybwik, 2011). And safety net systems also should be adjusted to avoid accidental event leading to patient's death.

The muscular dystrophy wards may be requested to offer the circumstances for those who have difficulties in continuing HMV. There is necessarily needs for hospitalization of HMV patients (Windisch, 2008).

Study limitation: This study has limitation on bias of collecting patients' information. Specifically, information of HMV patients were reported from 14 institutes among 27 institutes, and MV inpatient information is the result of extraction from muscular dystrophy wards database. Extracted data from database has some ambiguous points in connection with obscure time-sequential analysis.

Comparison Between Courses of Home and Inpatients

**National Center of Neurology and Psychiatry** 

*The Lancet Neurology*, Vol.9, pp 177–189.

132, pp 671-676.

1363.

*Health Services Research*, Vol. 11, pp 115-123.

dystrophy.*American Heart Journal* Vol.137, pp 895–902.

**6. References** 

**National Hospital Organization:** 

Mechanical Ventilation in Patients with Muscular Dystrophy in Japan 115

Akita National Hospital, Nishitaga National Hospital, East Saitama National Hospital, Shimoshizu National Hospital, National Hakone Hospital, Niigata National Hospital,

Hyogo-cyuo National Hospital, Hiroshima-Nishi Medical Center, Matsue Medical Center,

Miyazaki Higashi Hospital, Minami Kyushu National Hospital, Okinawa National Hospital

American Thoracic Society Documents (2004). Respiratory Care of the Patient with

Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Cripe L, Kaul A, Kinnett K,

Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Cripe L, Kaul A, Kinnett K,

Dybwik.K, Nielsen.E.W,Brinchmann. B. S (2011).Home mechanical ventilation and

Ishikawa Y, Bach JR, Minami R (1999). Cardioprotection for Duchenne's muscular

Joseph S. L, Peter C. G (2007). Current Issues in Home Mechanical Ventilation. *Chest,* Vol.

Matsumura T, Tamura T, Kuru S, Kikuchi Y, Kawai M (2010).Carvedilol can prevent

Tatara K, Fukunaga H, Kawai M (2006). Clinical survey of muscular dystrophyin hospitals

of National Hospital Organization. *IRYO*, Vol. 60, pp 112-118.

Duchenne Muscular Dystrophy. ATS Consensus Statement *American Journal of* 

McDonald C, Pandya S, Poysky J, Shapiro F, Tomezsko J, Constantin C, for the DMD care considerations working group (2010a). Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and

McDonald C, Pandya S, Poysky J, Shapiro F, Tomezsko J, Constantin C, for the DMD care considerations working group (2010b). Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care.

specialized health care in the community: Between a rock and a hard place. *BMC* 

cardiac events in Duchenne muscular dystrophy. *Internal Medicine* Vol. 49, pp 1357-

Tokushima National Hospital, Oomuta Hospital, Nagasaki Kawatana Medical Center,

Asahikawa Medical Center, Yakumo Hospital, Aomori National Hospital,

Iou National Hospital, Nagara Medical Center, Suzuka National Hospital,

Kumamoto Saishunso National Hospital, Nishibeppu National Hospital,

*Respiratory and Critical Care Medicine*, Vol 170, pp 456–465.

psychosocial management. *The Lancet Neurology,* Vol.9, pp 77-93.

Nara Medical Center, Utano Hospital, Toneyama National Hospital,

On analysis of institutes-restricted HMV patients group and MV inpatients group, differences in regard to therapeutic conditions among institutions may be problem.

#### **5. Acknowledgments**

This study was supported by a Research Grant for Nervous and Mental Disorders from the Ministry of Health, Labour and Welfare of Japan.

We are grateful to the members of the FUKUNAGA (1999-2005) and SHINNO (2006-2011) muscular dystrophy research groups of the National Hospital Organization for the data collection.

Institutions specializing in muscular dystrophy treatment in Japan (Fig.4)

Fig. 4. Institutions specializing in muscular dystrophy treatment in Japan

#### **National Hospital Organization:**

114 Neuromuscular Disorders

On analysis of institutes-restricted HMV patients group and MV inpatients group,

This study was supported by a Research Grant for Nervous and Mental Disorders from the

We are grateful to the members of the FUKUNAGA (1999-2005) and SHINNO (2006-2011) muscular dystrophy research groups of the National Hospital Organization for the data

differences in regard to therapeutic conditions among institutions may be problem.

Institutions specializing in muscular dystrophy treatment in Japan (Fig.4)

Fig. 4. Institutions specializing in muscular dystrophy treatment in Japan

**5. Acknowledgments** 

collection.

Ministry of Health, Labour and Welfare of Japan.

Asahikawa Medical Center, Yakumo Hospital, Aomori National Hospital,

Akita National Hospital, Nishitaga National Hospital, East Saitama National Hospital,

Shimoshizu National Hospital, National Hakone Hospital, Niigata National Hospital,

Iou National Hospital, Nagara Medical Center, Suzuka National Hospital,

Nara Medical Center, Utano Hospital, Toneyama National Hospital,

Hyogo-cyuo National Hospital, Hiroshima-Nishi Medical Center, Matsue Medical Center,

Tokushima National Hospital, Oomuta Hospital, Nagasaki Kawatana Medical Center,

Kumamoto Saishunso National Hospital, Nishibeppu National Hospital,

Miyazaki Higashi Hospital, Minami Kyushu National Hospital, Okinawa National Hospital

#### **National Center of Neurology and Psychiatry**

#### **6. References**


**6** 

*Japan* 

**Myopathy in Autoimmune Diseases –** 

*3Neurological Institute, Southern TOHOKU Research Institute for Neuroscience 4Pathology Department, Southern TOHOKU Research Institute for Neuroscience* 

Myopathy, which clinically shows muscular pain (myalgia), weakness, cramps, stiffness and spasm, is one of neuromuscular disorders due to inflammation and/or dysfunction of muscle fibers. "Myositis", which is a general term for inflammation of the muscle, is pathologically an inflammatory myopathy seen seen mainly in autoimmune disorders including dermatomyositis (DM). The myopathy is classified by National Institute of Neurological Disorders and Stroke (NINDS) as indicated in Table 1 (1). We here focus myopathy on primary Sjögren's syndrome (pSjS) associated with myalgia "mimicking DM", as previously reported (2), and the inflammatory myopathy of DM (Table 2). Most of SjS is a secondary disorder to systemic autoimmune diseases including systemic lupus erythematosus (SLE), systemic sclerosis, DM, and so on. However, SjS, which is not associated with other autoimmune diseases, is considered to be an idiopathic primary disorder characterized by sicca symptoms. It is known that pSjS may be associated with fever, fatigue, myalgia, arthralgia, cutaneous vasculitis, etc. in addition to sicca

DM is also characterized by myalgia, muscular weakness and fatigue due to inflammatory myopathy that ultimately progresses to muscle degeneration and the cutaneous involvements. The skin manifestations include helio-trope-like colored erythema and swelling on the eye-lids, cheeks, neck and upper extremities of the sun-exposed areas and Gottron's papules on the dorsa of the hand fingers (3). Although the etiology of DM remains unknown, internal maligmant disorders including lung and/or other organ cancers are

frequently associated. Generally DM is classified as shown in Table 3 (9).

**1. Introduction** 

symptoms (4-8).

Corresponding Author

 \*

**Primary Sjögren's Syndrome** 

Fumio Kaneko1,2,\*, Ari Togashi2, Erika Nomura2,

*Southern TOHOKU Research Institute for Neuroscience 2Dermatology, Southern TOHOKU General Hospital,* 

**and Dermatomyositis** 

Teiji Yamamoto3 and Hideo Sakuma4 *1Institute of Dermat-Immunology and Allergy,* 

