In spite of the large number of elderly patients with Parkinson disease (PD) in Japan, there have been insufficient studies to provide useful information for their clinical management. In recent years, various researches clarifying the characteristics of elderly PD patients have been gradually accumulated, but there have been very few studies on “very” old–onset PD.
The term “elderly PD” includes both patients who have been on treatment for a long time and have become elderly, and those who have developed PD at old age. Characteristics of old–onset PD patients include severe motor and non–motor symptoms from the onset, rapid symptom progression and a short life expectancy, high risk of developing axial symptoms, hallucinations and dementia, while the lower risk of developing motor complications (wearing off and dyskinesia) and impulse control disorders. Neuropathological findings include a high incidence of Alzheimer's pathology as well as Lewy bodies. In terms of clinical feature, rigid–akinetic phenotype is significantly more common. In addition, pharmacokinetic changes specific to the elderly are also important.
The number of elderly PD patients is expected to increase rapidly in Japan, which is facing an unprecedented super–aging society. As a frontier for elderly PD patients, it is necessary to take the lead in establishing research results and evidence that contribute to the clinical management of elderly PD patients.
The current status and issues of transitional medicine in epilepsy care was outlined in this review article. There are three main types of transition in epilepsy care in Japan : (1) complete transfer to adult department after a certain overlapping period, (2) continued care by both pediatric and adult departments, and (3) continued care by pediatric department. In (1), patients with idiopathic generalized epilepsy and favorable focal epilepsy of childhood onset are included. Although diagnosis and treatment are not difficult in this group, a long–term treatment surely produces additional care as follows. It is important to understand the special condition of long–term treatment for school attendance and employment, and special precautions for driving, marriage, and pregnancy. In (2) and (3), a wide range of patients with generalized or focal epilepsy with hereditary or congenital diseases are included, and comorbidities other than epilepsy such as developmental or progressive disorders. The transition can be relatively smooth in patients with lesser medical care needs and good seizure control. On the other hand, if the patients suffer from psychiatric comorbidities or conditions that is unique for pediatric population and need the support by multiple departments, then the transition can be rather complicated. In order to conduct smooth transition for the sake of patients in the future, it is necessary in advance to have psychosocial support, patient education, and joint conferences with medical staff in order to share sufficient information among them. In addition, appropriate medical care and treatment should be provided by regional medical institutes and affiliated organizations together with different role to endorse equalization and standardization of epilepsy care. With the recent COVID–19 global pandemic, clinic visit in person has decreased and online treatment is recommended instead. In addition, technological advancement such as information technology system, data server system etc. appropriately endorsed by medical insurance system in Japan may further help us promote transitional medicine for epilepsy.
Transitional medicine from pediatrics to adult health care is an important issue. However, although child neurology and neurology specialize in the same neurology, the practice and research content are very different. Transitional medicine has long been regarded as an urgent issue in pediatrics, but the consciousness of the neurologist who receives the patient is not clear. The target age of neurology is mainly middle–aged and older. In addition, the number of neurologists specializing in muscle disorders is limited. Muscle disorders require interventions in a variety of areas, including respiratory, circulatory, nutritional, bone metabolism, and mental development, as well as motor dysfunction. For this reason, while it is common in pediatrics to provide comprehensive clinical care, adult health care often employ organ–based healthcare systems, which can be embarrassing for patients and their families. Various efforts have been made to address these issues. In July 2020, the Japanese Society of Neurology established the special committee for measures against transition from pediatric to adult health care. In addition to the neurologists, members of the Japanese Society of Child Neurology have joined to exchange information in both directions instead of one. There are many problems that need to be solved, such as cooperation between medical institutions and the construction of medical expense systems. The first thing to do to achieve smooth transitional medicine seems to be the establishment of communication between pediatricians and neurologists.
In recent years, importance of the health care transition has been recognized, and Japanese government has taken measures for it. In the present review, several representative neuropathies, which are sometimes developed in children, and tips for the health care transition in those neuropathies have been introduced. Especially, the autonomy of children is important in those situations and should be developed in around the age of puberty.
An improvement in efficacy treatment and development of the social support system has led to many patients with neuroimmunological disorders being able to reach adulthood
Most of them are treated by pediatricians at first and followed by neurologists when the patient reaches adulthood. Child–onset neuroimmune diseases may have different symptoms from adult–onset cases, and in some cases, diagnostic criteria, treatment guidelines, and social welfare systems may also differ. Though most cases of child–onset neuroimmune diseases are thought to become independent from their parents finally because higher brain dysfunction is not as prominent as in severe psychosomatic disorders, parents may be present during the decision–making and explanation of the treatment plan at the beginning of the transition to neurologists, and it is important to pay attention to them. In this article, we will focus on central nervous system demyelinating diseases (multiple sclerosis, neuromyelitis optica spectrum disorders, MOG antibody–related diseases), and outline the clinical features, applicable diagnostic criteria, social welfare systems, and treatment of these diseases in children.
Advances in medical technologies have made it possible for patients with severe motor and intellectual disabilities (SMIDs) to survive into adulthood. Therefore, the pediatric to adult health care transition is necessary for children with SMIDs to adapt to adult life. The causes of SMIDs are categorized according to the period of onset (i.e., prenatal period, perinatal and neonatal period, postnatal period). The diagnostic groups for SMIDs include hypoxia/asphyxia, low–birth–weight infant, chromosome disorders, infections, metabolic disorders, and brain injuries, and so on. Post–transition medical care was optimized by reviewing the diagnosis, adjusting antiepileptic medications, and taking care of the physical condition. With the cooperation of medical social workers and nurses, community–based health care coordination and allocation of social resources for appropriate use of welfare services were promoted. Providing patient–centered adult health care improved comprehension of the disease for patients with SMIDs and their families. Therefore, they were able to determine future care policies with a shared decision–making process. Face–to–face information sharing prior to referral between physicians who care for children and physicians who care for adults, especially for children with SMIDs, is known to be important and facilitates improved care. Furthermore, interprofessional collaboration with pediatricians, adult clinicians, medical social workers, nurses, and other professionals facilitates a better transition for patients with SMIDs. Neurologists engaged in medical care for patients with intractable disease are desired to participate in the transition from pediatric to adult health care. The Special Committee for Measures Against Transition from Pediatric to Adult Health Care of the Japanese Society of Neurology, which is composed of pediatric and adult neurologists, has been working toward a better transition from pediatric to adult health care since July 2020.
Due to advances in medical care, in recent years, about 90% of patients with childhood–onset diseases have reached adulthood. As the patients get older, they developed the risk of atherosclerosis and malignancy, and need to support for the autonomy and social participation. Therefore, transitional care is needed.
Although our facility is a regional core hospital with hyperacute beds, our neurology clinic accepted many cases of transition mainly due to the relocation of a nearby pediatric medical center. Most patients had epilepsy, and 40% are SMID (severe motor and intellectual disabilities), such as cerebral palsy and metabolic disease. Epilepsy patients often had mental retardation, and SMID patients often required medical treatment such as airway management and tube feeding.
In epilepsy patients, there were cases of frequent seizures due to neglect of medication, and there was a problem with patient autonomy. Patients with SMID were often hospitalized due to respiratory tract infections, and often required complicated measures in terms of environmental improvement and cooperation with community medicine.
The questionnaire survey conducted at our department revealed 70% of the cases transferred to our facility had a certificate of intellectual disability and were considered to have difficulty in autonomy, and more than 60% were worried about changing doctors.
Our experience and survey showed that patients with child–onset neurological diseases have difficulties in reconstruction of medical treatment environment and autonomy during transitional care. It seems realistic to set up a set up a preparation period for transition and have a good multidisciplinary collaboration between neurology and neuropediatrics.
＜目的＞脊髄小脳変性症患者に対する短期集中リハビリテーション（集中リハビリテーション）において，Mini–Balance Evaluation Systems Test（Mini–BESTest）を用いてバランス機能の変化を捉えられるか検討した．＜方法＞対象は，4週間の集中リハビリテーションに参加した脊髄小脳変性症患者14名である．プログラム初日と最終日にScale for the Assessment and Rating of Ataxia（SARA），Mini–BESTestによる評価を行った．SARAとMini–BESTestの総得点，およびMini–BESTestの下位項目について，Wilcoxonの符号付き順位検定を用いて検討した．＜結果＞介入前後でSARAの総得点，Mini–BESTestの総得点が有意に変化した．さらに，Mini–BESTestの3つの下位項目において点数が有意に変化した．＜結論＞本研究より，Mini–BESTestにより集中リハビリテーションによる脊髄小脳変性症患者のバランス機能の変化を捉えられる可能性が示唆された．