Although recent advance in molecular biology have provided increasing insights into the pathophysiology of neurodegenerative diseases, there is no established disease–modifying therapy for which the efficacy has been verified in clinical trials.
Spinal and bulbar muscular atrophy (SBMA) is an adult–onset neuromuscular disease caused by the expansion of a trinucleotide CAG repeat in the androgen receptor (AR) gene. The ligand–dependent nuclear accumulation of pathogenic AR protein is central to the pathogenesis. Leuprorelin, a luteinizing hormone–releasing hormone (LHRH) analogue that suppresses testosterone production from testis, inhibits toxic accumulation of pathogenic AR, thereby mitigating histopathological and behavioral impairments in a mouse model of SBMA. A randomized placebo–controlled multi–centric phase 3 clinical trial showed an effect of the drug on motor functions, furthermore there was a significant improvement of swallowing function in a subgroup of patients whose disease duration was less than 10 years. In addition, a long–term follow–up study compared with natural history group of patients revealed that the progression of motor dysfunction and occurrence of pneumoniae due to swallowing dysfunction were very much diminished with leuprorelin treatment.
Amyotrophic lateral sclerosis (ALS) is an adult–onset neurodegenerative disease characterized by progressive upper and lower motor neuron loss, which leads to limb and bulbar paralysis and respiratory failure. We constructed a multicentre registration and follow–up system called the Japanese Consortium for Amyotrophic Lateral Sclerosis research (JaCALS). Using this registry system, several investigations have been performed. We hope that our report will be helpful for clinicians who want to provide medical, social, and nursing care to patients with ALS with proper timing, and to researchers as they plan clinical trials for ALS.
There are still some intractable neurological disorders with unknown causes that have a progressive clinical course. It is important to develop treatments for such neurological disorders. The Japanese Society of Neurological Therapeutics (JSNT) was established in 1983 for this very reason.
There has been substantial progress in the treatment of neurological disorders, such as stroke, Parkinson's disease, Alzheimer's disease, neuroimmunological diseases, migraine, epilepsy, and so on, using not only drug therapy, but also gene therapy, tissue engineering, nerve stimulation and rehabilitation. In order to develop treatments for neurological disorders, the individual activities of JSNT are also important.
JSNT is involved in exchanges with the American Society for Experimental NeuroTherapeutics (ASENT) in the USA. The mission of ASENT is to facilitate cooperation between academia, government, industry, the clinical community and advocacy groups to enable and advance the discovery, translation, and development of neurotherapeutics. Through communication with ASENT, the importance of information exchange and cooperation between these parties has been further recognized. To further the development of new treatments for neurological diseases, JSNT is communicating with the Pharmaceuticals and Medical Devices Agency (PMDA) in Japan through an active exchange of views. The PMDA is also serving as a consultant on drug strategy in JSNT meetings. JSNT has examined unmet medical needs for neurological diseases through collaboration with ASENT. JSNT has studied the current status of treatment for essential tremor in Japan and has clarified the characteristics and differences of essential tremor under practical medical conditions between Japan and foreign countries.
Education for young doctors and co–medical staff and lifelong education about the treatment of neurological disorders are also important. JSNT is preparing some educational programs and has published some practice parameters and guidelines for the treatment of neurological disorders.
In JSNT, we will have the online–only journal publishing high quality peer reviewed articles related to treatment of neurological disorders. Now, we will plan to obtain the legal personality of JSNT, in order to work more active operations.
Thus, JSNT is contributing to progress in the treatment of neurological disorders.
Several autoantibodies are associated with autoimmune encephalitis. Some of these antibodies are directed against intracellular neuronal antigens such as Hu and Ma2, which are strongly associated with paraneoplatic syndrome. In the past 10 years, various antibodies were identified that recognize neuronal cell–surface or synaptic proteins in patients associated with or without malignancy. Some of these antibodies are able to directly access receptors of neurotransmitters or channels and are responsible for causing neurological syndromes. Autoimmune encephalopathy with these antibodies generally responds to immunotherapies, such as steroids, plasmapheresis, and intravenous immunoglobulin as well as immunosuppressant and anti–cancer treatments in cases of paraneoplastic syndrome.
Patients with N–methyl–D–aspartate (NMDA) receptor antibodies, which are the most common in autoimmune encephalopathy, often cause psychiatric manifestation, memory impairment, seizures, dyskinesia, catatonia, autonomic instability and respiratory failures. Although 86% of patients become worse at the stage of mRS5, almost 80% of all patients recover to the stage of less than mRS2 with immunomodulatory therapy and careful management for their general condition. Detection of those antibodies in both serum and CSF using cell–based assays is important for definite diagnosis. Availability of screening systems of antibodies and covering health insurance for immunomodulatory therapy for autoimmune encephalitis are highly expected.
The neuroplasticity, especially synaptic plasticity, plays critical roles for maintaining great flexibility of the nervous system in humans. Several non–invasive stimulation methods have been reported to induce such plasticity in human brain, such as transcranial magnetic stimulation, direct or alternative current stimulation, high voltage electric stimulation and so on. In this communication, I will summarize a new stimulation method (quadripulse stimulation, QPS) and show several clinical applications of repetitive transcranial magnetic stimulation (rTMS).
QPS : In this intervention, a burst of four monophasic magnetic pulses separated by a certain inter–stimulus interval (ISI) was given once five seconds (inter–burst interval IBI of 5 seconds) for 30 minutes over the target area (the primary motor cortex in this communication) with a magnetic coil. The motor evoked potentials (MEPs) were recorded before and after the intervention, and the time course of those amplitudes was used to evaluate the motor cortical excitability changes for estimating the motor cortical plasticity. QPS with an ISI of (QPS5) was most effective to induce the long term potentiation (LTP), and QPS with an ISI of 50ms (QPS50) for the long term depression (LTD). Several physiological characters of QPS were consistent with the synaptic plasticity. Its resistance to BDNF polymorphism and less interindividual variability have been reported recently.
Dopamine and QPS : Dopamine is one of the substances to modify the degree of plasticity. We studied effects of dopamine (D1, D2) and dopamine agonist (D2) on the plasticity induced by QPS. The dopamine enhanced both LTP and LTD whereas the agonist had no effects on either LTP or LTD. Based on these results, we concluded that the plasticity studied by QPS over M1 should reflect the synaptic plasticity within M1 modified by ventral tegmentum (VTA)–M1 dopamine projection and should have no correlation with the basal ganglia–motor–cortical loop directly influenced by substantia nigra.
Clinical applications of rTMS for the treatment : We summarized previous clinical or preclinical rTMS trails for Parkinson disease, cortical myoclonus and paraparesis.
Parkinson's disease is a neurodegenerative disease characterized by the cardinal motor features, which is clinically evident and diagnosed when the motor symptoms appear. Pathological or neuroimaging researches have already revealed that pathobiological initiation of Parkinson's disease is several years prior to motor manifestations. In the Parkinson's disease at–risk syndrome study, Parkinson's disease before clinically evident is divided into 4 phases. The phase illustrated by the cardinal motor features but without clinical diagnosis of parkinsonism is defined as the pre–diagnostic phase. The phase before showing motor features is defined as the pre–motor phase and the pre–clinical phase, which are illustrated by only nonmotor symptoms and only neuroimaging evidence, respectively. Recent advances in therapeutic innovation can target the disease modifying effect in Parkinson's disease. In order to define at–risk individuals of Parkinson's disease and enable to recruit appropriate subjects to clinical trials developing the disease modifying therapy of Parkinson's disease, the concept of prodromal Parkinson's disease is proposed. Prodromal Parkinson's disease is regarded as individuals in the pre–motor phase and the pre–clinical phase. Some clinical researches aiming to detect biomarkers of prodromal Parkinson's disease are ongoing in the world. In 2015, the Movement Disorder Society reported a research criteria of prodromal Parkinson's disease. Brand–new therapeutic candidates are also reported in the international congresses. This review summarizes the recent developments in understanding the concept of prodromal Parkinson's disease.
How to diagnose and manage for involuntary movement is one of the attractive issues in neurology. In this issue I provide how to make a diagnosis on abnormal involuntary movement. Abnormal involuntary movements are consist of tremor, dystonia, chorea myoclonus, tic and so on. They are frequently observed in neurology clinic. It is important to diagnose of them by history taking, inspection, neurological examination, and confirmed with both imaging such as MRI and electrophysiological examination. Electrophysiological examination consist of the surface electromyography, the evoked potentials (auditory, visual, spinal and so on), and equilibrium function tests. I hope this issue encourage your practice on movement disorders.
EMG is widely used for the diagnosis of neuromuscular disease. However misunderstandings of its principals lead to wrong diagnosis. In this lecture, I emphasized several basic concepts of EMG ; (1) Action potential in a volume conductor is recorded as a triphasic wave, (2) Fibrillation potential, positive sharp wave and fasciculation potential are very important spontaneous activities to evaluate disease activity, (3) Regional muscle fiber density of a MU effects largely on the MUP waveform.
Various diseases including Alzheimer's disease (AD), vascular dementia (VaD), dementia with Lewy bodies (DLB), frontotemporal lobar degeneration (FTLD), etc. cause dementia. Accurate diagnosis of dementia is essential to provide appropriate treatment. Routine diagnostic steps include a careful history taking, mental status screening, neurological examinations, laboratory and imaging studies, and neuropsychologic testing.
AD accounts for more than half of all cases of dementia. Many researchers believe AD is caused by the accumulation of amyloid beta protein and tau protein in the brain, leading to memory loss, changes in judgment and other behavioral changes characteristic of AD. Other pathophysiological changes in the brain could evoke other forms of dementia as well.
Understanding the characteristic features and pathology of each type of dementia could be useful in the accurate diagnosis of patients for the appropriate treatment and support services.
This paper outlines the characteristics of major dementia useful for differential diagnosis, in addition to their current treatments.
White matter disorders or leukoencephalopathies comprise disorders that exclusively or predominantly affect the white matter of the brain. In this section, we pick up 5 white matter disorders in the limelight, that is, neuronal intranuclear inclusion disease (NIID), fragile X tremor–ataxia syndrome (FXTAS), hereditary diffuse leukoencephalopathy with spheroids (HDLS), CADASIL and Alexander disease. NIID is a neurodegenerative disease characterized by eosinophilic intranuclear inclusion in neuronal and other organ cells in H&E slides. Recently, we reported that skin biopsy is useful for antemortem diagnosis of sporadic and familial NIID, and the number of NIID cases increase remarkably. Sporadic NIID presented dementia, ataxia, bladder dysfunction, general convulsion. And laboratory data showed leukoencephalopathy, DWI high intensity in cortico–medurally junction, NCV abnormality and protein level elevation in cerebrospinal fluid. Familial NIID classified in two groups, dementia dominant group and weakness dominant group. FXTAS present tremor and ataxia. MCP sign of head MRI is characteristics observation. Histopathological feature of intranuclear inclusion of FXTAS is resemble to those of NIID, so analysis of FMR1 CGG repeat is necessary to exclude NIID. HDLS onset age is from 40 to 50 years of age, and present dementia and personality change. White matter damage around lateral ventricle and thinning of corpus callosum are characteristics observation. Neuropathological feature of HDLS is the presence of neuroaxonal spheroids. CSF–1R has been identified as a causative gene. CADASIL present migraine with aura, subcortical ischaemic events, mood disturbances, apathy, cognitive impairment and dementia. Histopathological investigation shows a specific arteriopathy affecting the small penetrating arteries that show a thickening of the arterial wall, the presence of a non–amyloid granular osmophilic material within the media. This arteriopathy is also present in skin. NOTCH3 is reported as causative gene. Alexander disease caused by dominant mutations in the GFAP gene. Three age–dependent clinical subtypes have been adopted, but recently, new categories in two subtypes based on phenotype analysis with GFAP mutation. Head MRI pattern of young patient showed extensive white matter abnormalities with frontal predominance, and adult–onset patients present atrophy of the lower brainstem extending caudally to the cervical spinal cord.
Recent clinical management of bacterial meningitis (BM) is reviewed. Bacterial meningitis can present as an acute fulminant disease that progress rapidly in a few hours or a subacute infection that progressively worsens during several days. The mortality rate with BM and the frequency of neurologic sequelae among those who survive are high. BM is thus a life–threatening neurological emergency. Early recognition, efficient decision–making, and rapid institution of therapy can be lifesaving. Empirical therapy should be initiated promptly whenever BM is a significant diagnostic consideration.
Infectious Diseases Society of America (2004) and European Federation of Neurological Societies (2008) reported initial management approach of BM. The guidelines for the clinical management of BM in Japan were published in 2007, and the revision was also published in December 2014. These initial therapeutic managements for BM differ for every country. The reasons for the different initial therapeutic management are based on the following strategies. The choice of specific antimicrobial agents for initial treatment is based on the current knowledge of antimicrobial susceptibility patterns of these pathogens in the area. For initial treatment, the assumption should be that antimicrobial resistance is likely. The choice of empirical antibiotic in BM may be influenced by a number of factors, including patient's age, systemic symptoms and local pattern of bacterial resistance. If there are no epidemiologic evidences in Japan, a committee investigates it and builds data in the revised Japanese guidelines 2014 for the clinical management of BM.
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder that diffusely affects motor neurons and develops symptoms such as muscle atrophy, muscle weakness, dysarthria, dysphagia, respiratory dysfunction. At present there is no cure and the symptoms gradually develop. For improving quality of life (QOL) in the patients with ALS, cooperation among different medical specialists is critical. There are three things to be understood for the care of patients with ALS.
First, it should be understood that a patient cannot accept the disease easily. Despite a physician's careful explanation, the patient may feel frightened by the physician's words. You should observe the perturbation of the patient, because it is not uncommon to see depressive mood in the patient, even if the patient feels positive by support from family members and caregivers. Although decision–making in advance has been recommended in the medical society, it is not uncommon to see overturning the decisions by the patient with ALS, such as transition from “no endotracheal intubation” to “inclination to it”. In such situations, medical staffs become confused by the change, but it just means that the patient has perturbation in his/her mind.
Second, think about something to improve QOL of the patient. In disease like Parkinson's disease, physician's management such as selection of drugs does improve QOL. On the other hand, the limited therapeutic options for ALS has limited effects, thus gives no significant effect on QOL and only a limited role for a physician mostly focusing on diagnosis. Rather, an approach by other medical staffs are the key for improvement of QOL in the patient with an advanced stage of ALS. Given busy daily routines for each medical personnel, it seems to be difficult to meet the needs of the ALS patient, especially those who are respirator–bound. Even in these cases, it is important to “face it” by not quickly rejecting the requests. Japan ALS Association (JALSA), a patient–centered group, has been establishing local branches and offers hints for daily activities and managements.
Lastly, I wish that all the medical staffs become interested in research. At least, please keep in mind that research and technology have been steadily progressing. Please let the physicians know about patients' hard work studying the recent progress in research and asking questions on these. Patients often talk with their nearby medical staffs, rather than with physicians, on how much they eager to have a new therapy and their intention to participate in a research project for elucidation of the disease. Now is the time to see rapid progresses in the study of the disease mechanisms. It could not be impossible to find the cause of the incurable disease without effective treatment by the recent advances of science. Cooperative effort by the patient is the necessary step for the potential achievement and now is the best time to utilize the cooperation.
Meningitis and encephalitis are severe neurological infections that, if treated late and ineffectively, lead to poor neurological outcome or death. Since treatment is more efficient if given early, these conditions of meningitis and encephalitis should represent as a life–threatening neurological emergency. The management of patients with suspected meningitis or encephalitis begins with empiric treatments until the causal agent of infection is determined. However, the etiologic organism cannot always be distinguished. The goal is to identify those that are treatable, provide supportive care for those that are not, and, when possible, prevent the neurologic complications of these infections. In this article, the author will present some representative cases to describe the characteristic magnetic resonance imaging and spinal fluid analysis of bacterial meningitis, tuberculous meningitis, cryptococcal meningitis, herpes simplex encephalitis, and anti–NMDA receptor encephalitis, and discusses the choice of empirical treatments until the cause of infection is determined. Also the differential diagnosis of meningitis and encephalitis is reviewed, with an emphasis on infectious etiologies.
Team medicine is indispensable for stroke care, in particular care for acute stroke patients. In this short report, acute reperfusion therapy for ischemic stroke, including intravenous thrombolysis and acute endovascular thrombectomy, are mainly introduced.
Peripheral neuropathies are common disorders in the daily medical practice, and often cause weakness, numbness, pain, and autonomic symptoms. The several specific clinical laboratory tests are useful for the diagnosis of peripheral neuropathies. Nerve conduction studies are not useful for evaluating small fibre neuropathy, such as early stage of transthyretin–related familial amyloid polyneuropathy (TTR–FAP) characterized by involvement of small fibres such as Aδ and C fibres. To evaluate small fiber neuropathies, various autonomic function tests, such as laser–Doppler flowmetry, sweating tests using capsule type sweating ratemeter, electrogastrography, R–R interval study, 123I–MIBG myocardial scintigraphy, head–up tilt test, and intraepidermal nerve fiber density, are useful. TTR–FAP is an autosomal–dominant inherited disorder characterized by systemic accumulation of amyloid fibrils in various organs and peripheral nerves. To date, more than 130 mutations in the TTR gene have been reported. In TTR–FAP, several therapies have been developed in the recent decade. In addition to liver transplantation, tetramer structure stabilizers were developed. Also, gene silencing drugs are under clinical trials.
Hereditary ATTR amyloidosis is an autosomal dominant genetic disorder with systemic deposition of amyloid fibrils induced by transthyretin (TTR) gene mutation. Clinically, this disorder is characterized by progressive nerve length–dependent sensorimotor and autonomic neuropathy, as well as non–neuropathic manifestations of cardiomyopathy, gastroenteropathy, ocular amyloidosis, and leptomeningeal amyloidosis/cerebral amyloid angiopathy. To date, more than 100 TTR gene mutations have been reported, the most common of which is V30M (p.V50M) that is found worldwide. Orthotopic liver transplantation, which replaces the variant TTR gene with the wild–type gene in the liver, the main source of serum circulating TTR was shown to be able to halt or slow neurological progression, and is at present the standard–of–care treatment in patients aged ＜50 with V30M mutation. However, large numbers of patients are not suitable transplant candidates. Recently, the clinical effects of TTR tetramer stabilisers, diflunisal and tafamidis, were demonstrated in randomised clinical trials, and tafamidis has been approved for treatment of hereditary ATTR amyloidosis in several European countries, Japan, Argentina, and Mexico. Moreover, small interfering RNAs and antisense oligonucleotides for suppression of TTR synthesis are promising therapeutic approaches to ameliorate hereditary ATTR amyloidosis and are currently in phase III clinical trials. However, these disease–modifying therapies cannot prevent the progression of ocular amyloidosis and leptomeningeal amyloidosis/cerebral amyloid angiopathy, due to variant TTR synthesis by the retinal pigment epithelium and choroid plexus. New therapeutic strategies should be developed to ameliorate ATTR ocular amyloidosis and leptomeningeal amyloidosis/cerebral amyloid angiopathy.
First–line therapies (intravenous immunoglobulin, corticosteroids, and plasmapheresis) of CIDP are already established, but cannot be excessive expectations to the effectiveness of the second–line such as immunosuppressant. Usefulness of the maintenance therapy represented by continuous half–dose IVIg or subcutaneous immunoglobulin (SCIg) has been widely recognized. However, the proper amount of immunoglobulin for maintenance therapy is not clear. In addition, the proper period to begin the withdrawal from the maintenance therapy is not clarified yet. We should find the biomarkers to distinguish the patients who need the maintenance therapy from the “benign” others with monophasic course.
From the pathogenesis of CIDP, autoantibodies that target the molecules (eg. CNTN1, NF155) near the nodes of Ranvier are detected in at least a few percent of whole CIDP patients. These autoantibodies are unique since the autoantibody–positive patients show the similar phenotype including IVIg poor–responsiveness. The usefulness of therapeutics that could remove the antibody such as plasmapheresis, rituximab, and autologous haematopoietic stem cell transplantation (AHSCT) could be expected as a promising treatment. Finally, we should carefully consider those autoantibodies when setting up future clinical trials for CIDP.
Because most of the vasculitic neuropathy patients require rigorous, long–standing immunosuppressive therapy, the accurate diagnosis, especially based on the pathological detection of vasculitic changes, is the first step of treatment. In this regard, the value of sural nerve biopsy combined with peroneus brevis muscle biopsy is still not ignorable. In the treatment of vascultic neuropathy, there are no controlled treatment trials and clinical practice is guided by experience from case series and indirectly by analogy with systemic vasculitis. Although combined therapy using prednisolone and cyclophosphamide is usually recommended by experts, tailor–made treatment regimen based on the conditions of each patient would produce the best outcome in vasculitic neuropathy. High–grade clinical evidences specialized in the treatment of vasculitic neuropathy are eagerly awaited.
Although pharmacological treatments for Parkinson's disease, such as L–dopa, show good response in the early phase, patient outcomes over the long term are unsatisfactory. As an additional treatment, cell therapy with aborted fetal tissues has been performed since 1980's. Despite positive results, the limited supply of donor source and the unstable quality of the cells prevent this therapy from becoming standard. Regenerative medicine for Parkinson's disease using induced pluripotent stem cells (iPSCs) is drawing attention, because it offers a limitless and more advantageous donor source than aborted embryos. We are preparing a clinical trial that involves the transplantation of dopaminergic neural progenitors differentiated from iPSCs. We have successfully established a protocol to induce dopamine neurons from iPSCs and have transplanted these neurons into Parkinson models of mice, rats, and cynomolgus monkeys for preclinical study.
The development of large data sets and more powerful computers over the last decade have made it possible to reveal the fundamental principles of complex functional and anatomical brain networks using several techniques. Resting state functional magnetic resonance imaging (rsfMRI), a non–invasive tool to assess functional abnormalities of the brain without specific motor or cognitive tasks, has provided important insight into the pathophysiology of normal aging and neurodegenerative disorders. Results of our preliminary analysis of 200 aged healthy subjects indicate that brain atrophy with aging is observed mainly in limbic and premotor areas. Diffusion tract imaging showed anatomical network disruption surrounding the lateral ventricle. However, rsfMRI showed that an increase of resting connectivity across multiple cortical areas was observed more frequently than a decrease. These results may reflect a compensatory phenomenon of functional brain networks against the progression of brain atrophy and disruption of anatomical brain networks with aging. The IMAGEN group demonstrated that dozens of genes linked to ion channel activity and synaptic function support connectivity functional resting state networks. These molecular components may become new targets for disease modifying therapy. In addition, rsfMRI revealed that replacement therapy, deep brain stimulation, and rehabilitation may assist the normalization or amelioration of abnormal functional network connectivities and improve symptoms in neurodegenerative disorders. Analysis of brain functional connectivity may play an important clinical role as an early diagnostic and biomarker tool.
In order to assess distribution and patterns of nerve hypertrophy in chronic inflammatory demyelinating polyneuropathy (CIDP), magnetic resonance neurography with 3–demension reconstruction of short tau inversion recovery images was performed in 40 CIDP patients. This technique clearly showed longitudinal morphological changes from the cervical roots to the nerve trunks in the proximal arm. Nerve enlargement was detected in 92% of the patients. Typical CIDP patients showed symmetric and root–dominant hypertrophy, whereas patients with multifocal acquire demyelinating sensory and motor neuropathy had multifocal fusiform hypertrophy in the nerve trunks. The patterns of nerve hypertrophy presumably reflects the different pathophysiology of each CIDP subtypes.
Cerebral small vessels have a pivotal role in maintaining exchange of energy and metabolites in the brain. Hypertension and amyloid β deposition may affect these small vessels, resulting in hypertensive small vessel disease and cerebral amyloid angiopathy (CAA), respectively, and are collectively related to the pathogenesis of microvascular lesions and dementing illness. This review focuses on the significance of cerebral microvascular lesions in cognitive impairment, and further, the relationship between cerebral microcirculation disturbance and perturbation of amyloid β clearance in Alzheimer's disease.
Alzheimer's disease (AD) is the most common cause of dementia in adult in Japan. In AD brain, two types of pathological findings including extracellular amyloid–β deposition and intracellular phosphorylated tau accumulation are observed. Although symptomatic therapy using choline esterase inhibitor and/or memantine are available for patients with AD, no effective disease–modifying drugs that may ameliorate natural history of AD has not currently established. AD is now recognized as one of largest unmet medical need among physicians. Thus, new therapeutic targets for drugs showing the disease modifying effect need to be explored. Epidemiological studies have shown that type 2 diabetes mellitus (T2DM) is associated with a nearly two–fold increased risk of AD. Although precise mechanisms of linking T2DM and AD remains unknown, accumulating evidence suggests that insulin resistance in brain plays a pivotal role in the pathogenesis of AD. This review article focuses on molecular aspect and the pharmacological strategies from the view of insulin resistance occurring in AD brain.
If we try to treat dysphagia patients by single department or single profession, we might fail. Fortunately, many professions such as nurses, speech language pathologists, pharmacists, nutritionists, and dentists also participate in therapy for dysphagia these days.
In this manuscript, I mentioned three roles of otolaryngologists : a) to coordinate team approach by a bird's–eye view, b) pathological and physiological diagnosis of dysphagia, and c) surgical interventions. a) To help patients who are suffered from disability, surgical or medical treatment are not enough. As a doctor in charge, it is important to coordinate team approach by a bird's–eye view. We are able to understand our patients precisely according to the ICF ; International Classification of Functioning, Disability and Health. b) Accurate physiological diagnosis or understanding mechanisms of dysphagia is essential to make an appropriate rehabilitation plan. We always analyze mechanisms of dysphagia anatomically by two types of tests, videofluorograpy ; VF and fiberendoscopic evaluation of swallowing ; FEES. c) It is important duty for medical doctors not to miss a treatable dysphagia. Sometimes surgical interventions can play a role effectively. They can be classified into two classes : surgery for improving function of swallowing and preventive surgery against aspiration. Surgery for improveing function of swallowing such as laryngeal suspention, cricopharyngeal myotomy or arytenoid adduction, in which procedure phonation is preserved. Selection of method is depend on mechanism of dysphagia. Preventive surgery against aspiration such as total laryngectomy, laryngotracheal separation, and glottal closure, is effective for chronic aspiration. Selection of method is depend upon patients general status.
Home medical care is needed urgently in Japan, which became super–aged society. In the field of dysphagia treatment, medical care at home is also needed because there are more dysphagic patients at home and nursing home than those at hospital. However, dysphagic treatment does not become widespread for severely short–handed of medical staffs at home and nursing home. To improve that state, we perform dysphagia treatment at home using videoendoscopy as part of home dental treatment. As the result, about 80 percent of patients thoroughly prohibited oral intake became able to take some food orally. Almost all patients of dysphagia at home or nursing home show chronic phase, therefore most of them need supportive care for dysphagia with considering end–of–life. We hope the day will come when all dysphagic patients can receive the tender care at home and nursing home with the prevalence of visiting dysphagia treatment.