著者
渡邉 典子 相馬 ひとみ 吉野 彩香 和田 麻季子 藤岡 俊樹
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.39, no.4, pp.723-726, 2022 (Released:2022-12-27)
参考文献数
12

症例は90歳男性.10年前にAlzheimer病と血管性認知症と診断され,薬剤治療を受けていたが,重症心不全を発症してICUに入院.心不全状態は改善したがせん妄症状と,自発性の低下,不眠,日中の傾眠,食事摂取不良をきたしたため,睡眠薬,抗認知症薬,鎮静薬などの投与に加え,認知症ケアチームが介入した.患者は認知症発症以前から音楽を趣味としていたため,好きな楽曲を中心に週3回,リハビリテーションの時に聴かせ,歌う様に促したところ,1ヶ月後には自発的にリズムをとり歌うようになった.同時にABC認知症スケールは50点から60点に改善,日中の傾眠も改善し自宅退院できた.音楽療法の効果は薬物療法と違いエビデンスは低いとされているが本例では音楽療法の開始に伴い日常生活動作の改善が明らかとなった.今後,音楽療法の科学的根拠を求めた検討が必要と思われた.
著者
飯塚 高浩
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.3, pp.155-163, 2021 (Released:2022-01-19)
参考文献数
49

It has been demonstrated that neuronal surface (NS) antibodies play an important role in the pathophysiology of various neurological and neuropsychiatric disorders, including non–infectious/post–infectious encephalitis, first–episode psychosis, epileptic and non–epileptic seizures, atypical demyelinating syndrome, post–partum psychosis, progressive dementia, involuntary movements (orofacial–limb dyskinesias, faciobrachial dystonic seizures, catatonia, rigidity, stiffness, tremors, myoclonus, chorea, stereotypies, oculomotor abnormalities), and non–REM/REM sleep disorder. IgG NS antibodies are considered more likely to be pathogenic, accordingly the presence of NS antibodies supports early initiation of immunotherapy. In 2016, a practical diagnostic approach to autoimmune encephalitis (AE) was proposed to achieve prompt immunotherapy at 3 levels of evidence for AE (possible, probable, and definite) with diagnostic criteria for possible AE, probable AE, probable and definite anti–NMDAR encephalitis, autoimmune limbic encephalitis, ADEM, and Hashimoto encephalopathy. Identification of autoantibodies against NS or intracellular antigens is crucial in making a diagnosis ; however, the antibody testing results should be carefully assessed especially when measured with commercial assay alone.In this lecture, I focus on recent progress in AE, and its related disorder including cryptogenic new–onset refractory status epilepticus.
著者
森 悦朗
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.37, no.1, pp.32-38, 2020 (Released:2020-07-21)
参考文献数
36

Dementia with Lewy bodies (DLB) causes various symptoms such as psychiatric symptoms, parkinsonism, and failures of autonomic nervous system in addition to cognitive impairment, all of which are clinical and care problems. This review provides evidence–based commentary on treatment of DLB. Donepezil has been the central means since its approval in 2014 for the treatment of cognitive impairment of DLB, and evidence of it is accumulating and gives clues of the usage of it. Although there is insufficient evidence on the efficacy of donepezil for BPSD, it is still the first choice before antipsychotics. On the other hand, motor disorders due to parkinsonism are also important therapeutic targets. Levodopa is the mainstay of treatment. Recently, multicenter, placebo–controlled, randomizeouble–blind, controlled trials have shown the efficacy of adding zonisamide over levodopa treatment for parkinsonism in DLB. Unfortunately, there is no high level of evidence of treatment for a variety of other conditions, and individual patients will be treated with knowledge of other diseases.
著者
高橋 一司
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.39, no.2, pp.51-57, 2022 (Released:2022-06-30)
参考文献数
44

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.
著者
高橋 一司
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.37, no.1, pp.20-27, 2020 (Released:2020-07-21)
参考文献数
61

Nonmotor symptoms (NMS) have increasingly been recognized as an important part of Parkinson disease (PD). Although NMS are very common across all stages of PD, NMS are frequently undeclared from the patients, often under–recognized by the clinicians and remain untreated. The burden of NMS can define a patient's health–related quality of life. The management of NMS has been recognized as an important area of unmet needs in PD. There is a broad spectrum of NMS in PD, which include neuro–psychiatric disturbance (hallucination, delirium, delusion, impulse control disorders, cognitive dysfunction, dementia), autonomic dysfunction (orthostatic hypotension, constipation, urinary dysfunction, sexual dysfunction, hyperhydrosis), sleep disturbance (insomnia, excessive daytime sleepiness, sudden onset of sleep, REM sleep behavior disorders, restless legs syndrome), mood disorders (depression, anxiety, apathy), fatigue and sensory disturbance (pain, smell loss). Although the importance of a dopaminergic contribution to NMS in PD has been highlighted, the NMS of PD include a multitude of clinical systems derived from complex multi–neurotransmitter dysfunction involving not just the dopaminergic pathways but also noradrenergic, serotonergic and cholinergic pathways in the brain. In addition to the evolution of NMS as an intrinsic part of the disease, treatment used in PD can trigger, worsen, or even be the primary cause of symptoms. The symptoms and treatments of NMS in PD are often multiple and complex. The Japanese “PD clinical guideline 2018” updates the previous PD treatment guideline 2011 and incorporates new data on efficacy, safety, and implications for clinical practice of treatments for NMS of PD. By using the current evidence in the medical literature, evidence–based medicine helps to provide the best possible care to patients. Although there has been a number of placebo–controlled randomized trials of treatments of PD–related NMS and the evidence base for treating a range of NMS in PD has grown substantially in recent years, many nonmotor areas still lack an adequate evidence base of high–quality studies.
著者
角谷 寛
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.4, pp.495-498, 2021 (Released:2022-04-28)
参考文献数
10

Hypoglossal nerve stimulation (HNS) is a novel therapy in treating moderate and severe obstructive sleep apnea (OSA) in patients who cannot tolerate continuous positive airway pressure (CPAP) therapy. Studies showed that HNS decreased apnea–hypopnea index decreased by 68%, and that the device was effective even after five years. HNS has become a therapeutic option for OSA. More studies may be needed to ensure the highest safety profile.
著者
平山 幹生
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.33, no.3, pp.338-342, 2016 (Released:2016-11-10)
参考文献数
7

I present the patients with misdiagnosis that I encountered for those 40 years of practice of neurology. Misdiagnosis is named diagnostic error, or diagnostic delay. The causes are classified, 1. non–fault error (unreliable information from patients, asymptomatic development of comorbid disease), 2. system–related error (inefficient processes, failed teamwork and communication of necessary information and skills), 3. cognitive error (faulty knowledge, faulty collection of information, faulty integration). Through the experience of pitfalls with misdiagnosis, both young and mid-level neurologists learn from patients, and cultivate useful skills in the practice of neurology.
著者
山本 敏之
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.4, pp.669-673, 2021 (Released:2022-04-28)
参考文献数
33

Pneumonia is the most common cause of death in Parkinson's disease (PD), with dysphagia as the presumed cause of death. Patients with PD, who are older than 63.5 years and take more than 475mg/day of L–dopa, are at risk for dysphagia in the pharyngeal phase. However, silent aspiration is common in PD ; thus, patients may not be not aware of the risk of dysphagia. In light of this, these patients must be screened for dysphagia–related complications. Patients with PD complicated by severe oropharyngeal dysphagia have three clinical characteristics : higher modified Hoehn and Yahr stage, low body mass index, and increased difficulty in keeping food or drink in the mouth. Moreover, PD patients with severe oropharyngeal dysphagia are more likely to respond affirmatively to the following questions : “Have you lost weight in the past year?,” “Do you cough when you take some medicines?,” and “Do you have difficulty moving the food in your mouth while eating?” The Swallowing disturbance questionnaire is also useful in identifying PD patients with dysphagia. In the treatment of dysphagia in PD patients, those with poor motor symptoms should first be treated with medication. However, dysphagia in PD is not only an extrapyramidal system abnormality, but also the result of a combination of multiple causes, and does not improve with drug treatment alone. One treatment option is the chine down posture, which is useful in improving swallowing in PD patients, and is more effective when combined with expiratory muscle strength training. Further, some motor swallowing exercises on swallowing dynamic may reduce the difficulty in moving food in the mouth when chewing. Patients who have poor laryngeal elevation should be trained to strengthen the swallowing muscles, whereas those with delayed swallowing reflex onset, liquid food should be thickened to slow its passage through the pharynx. Additionally, PD patients with severe dysphagia should be considered for gastrostomy, for medication and nutritional management.
著者
城倉 健
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.4, pp.441-445, 2021 (Released:2022-04-28)
参考文献数
8

Peripheral vestibular disorders can be diagnosed by characteristic nystagmus, whereas central vestibular disorders can be diagnosed by neurologic signs/symptoms other than vertigo/dizziness. Thus, in the differential diagnosis of vertigo/dizziness, both nystagmus and neurological symptoms should be examined. When a diagnosis of benign paroxysmal positional vertigo is made, the canalith repositioning maneuver should be attempted by all practitioners. Vertigo/dizziness usually dose not persist for a long period of time due to vestibular compensation. However, some of dizziness may persist because of collapsed vestibular compensation mechanism or central sensitization. In the treatment of dizziness, it is useful to know the mechanism of chronicity.
著者
山﨑 貴史
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.4, pp.454-457, 2021 (Released:2022-04-28)
参考文献数
5

Lifestyle disease and social inactivity used to be considered as the principal cause of aging–related cognitive decline in our country. More recently, however, research emphasis has shifted to studies of neurodegenerative disease. It is now widely accepted that lifestyle disease accelerated the onset and progress of Alzheimer's disease (AD). No curative treatment is available, but epidemiological research provides a substantial amount of evidence of modifiable risk and protective factors that can be addressed to prevent or delay onset of AD and dementia. Risk of late–life dementia is determined by exposures to multiple factors experienced over the life course, and the effect of specific risk/protective factors depends largely on age. Medical and lifestyle interventions and promote social, mental, and physical activities aimed at increasing the cognitive reserve. The treatments for lifestyle disease represent an important avenue of approach to help slow or prevent the onset and progression of AD.
著者
三須 建郎
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.4, pp.470-474, 2021 (Released:2022-04-28)
参考文献数
17

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy induced by anti–aquaporin4 (AQP4) antibody and complement. Recently, it is revealed that there is a group of myelin oligodendrocyte glycoprotein (MOG) antibody positive cases in seronegative NMOSD and this MOG antibody associated disease (MOGAD) is now considered as a new disease entity. I would like to focus on the relationship between NMOSD and MOGAD especially on the pathomechanism and future treatment strategy in this symposium.
著者
溝渕 雅広
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.36, no.4, pp.345-348, 2019 (Released:2020-04-24)
参考文献数
6

The new guideline for epilepsy entitled “The clinical practice guidelines for epilepsy 2018” (Guideline 2018) was published in 2018, it updated “The clinical guidelines for the treatment of epilepsy in 2010”, aiming at standard treatments of epilepsy for all clinicians who treat epilepsy patients even if not epileptologists. The guidelines consist of two parts, the first part including the various summary of diagnosis, medical and surgical therapy, related conditions such as the status epilepticus, the psychogenic non epileptic seizures and the acute symptomatic seizures, the information providing for epilepsy patients. Second part includes systematic reviews prepared by GRADE system.The major points of updated medical treatments are as follows : (1) Although the timing of initiation of therapy is basically after two unprovoked seizures, it may start in cases of neurological abnormality, abnormal finding of EEG, epileptogenic lesion of neuroimages, family history or elder patients. It can also start according to the social status or the willing of patients. (2) The first line treatment of generalized tonic–clonic seizures is sodium valproate, other new drugs such as lamotrigine and levetiracetam are recommended in women fitting the child–bearing age. The first line treatments of focal onset seizures are carbamazepine, lamotrigine and levetiracetam, following zonisamide and topiramate. Second line treatments for focal onset seizures are other drugs including gabapentin, lacosamide and perampanel. (3) In the elder patients with co–mobidity, lamotrigine, levetiracetam and gabapentin are recommended. In women fitting the child–bearing age, the comprehensive counseling, family planning and selection of appropriate treatment are recommended before pregnancy.Current standard managements for epilepsy are established in Guideline 2018. In the near feature, it will be updated by the evidences of efficacy and tolerability of new drugs, such as lacosamide and perampanel, provided after 2016.
著者
髙橋 牧郎
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.36, no.4, pp.404-409, 2019 (Released:2020-04-24)
参考文献数
13

Parkinson's disease (PD) exhibits motor symptoms such as resting tremor, bradykinesia, and rigidity for which levodopa is successful, but often needs to be differentiated from other PD–related disorders resistant to levodopa. In PD, non–motor symptoms including autonomic symptoms such as constipation, hyperhidrosis, orthostatic hypotension, REM sleep behavior disorder (RBD), psychological symptoms such as depression and apathy, and olfactory dysfunction are merged at a high rate. Since non–motor symptoms develop before the onset of motor symptoms in PD, most of the PD cases are judged from the initial symptoms, however, atypical cases often encountered. Although conventional MRI do not show any abnormalities in PD, disappearance of nigrosome 1 can be confirmed by high magnetic field MRI susceptibility weighted images. Further, dopamine transporter scintigraphy (DaT–SPECT) is a sensitive for parkinsonism and iodine–123–metaiodobenzylguanidine (123I–MIBG) has higher specificity of PD diagnosis myocardial scintigraphy. However, the advanced stage of PD involves dementia with overlapping Aβ and tau pathology, leading to the mixed pathology of PDD/DLB which are clinically problematic. Differentiation from DLB, AD, PSP, CBD is also enabled by brain SPECT. Progress of these diagnostic tools is improving the correct diagnosis of PD, but quite a few familial PD and atypical cases exist. In this lecture, the tips as the clinical and pathological discrimination points of PD and other parkinsonian syndromes are outlined with specific cases. Further, not only various pathological background but also trauma and inflammation of the brain are required to consider the modifying factor for the diagnosis and treatment of PD.
著者
中嶋 秀人
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.37, no.3, pp.265-267, 2020 (Released:2021-02-05)
参考文献数
15

Encephalitis is associated with high morbidity and mortality, and the neurologic manifestations of this condition include fever, headache, altered mental status, convulsions, and psychiatric symptoms. Early diagnosis of encephalitis is crucial to ensure that the right treatment is given on time. Herpes simplex virus was the most common infectious cause among acute encephalitis, followed by varicella zoster virus. Thus, more than a quarter of patients were potentially treatable with aciclovir. Recent repots have showed the increasing recognition of autoimmune encephalitis, and anti–NMDAR encephalitis was the most common autoimmune condition. In this article, the author describes the characteristic clinical findings, magnetic resonance imaging and spinal fluid analysis of herpes simplex encephalitis, varicella zoster encephalitis, and anti–NMDA receptor encephalitis. Early identification of these patients may allow timely initiation of aciclovir and/or immunomodulatory therapy, and help improve clinical outcomes.
著者
岡澤 均
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.3, pp.347-352, 2021 (Released:2022-01-19)
参考文献数
15

By utilizing a new marker of necrosis pSer46–MARCKS, which was identified by comprehensive phosphoproteome analysis as a phosphoprotein changed before appearance of extracellular amyloid aggregation, we discovered that neuronal necrosis occurs much earlier in Alzheimer's disaese pathology than expected. The necrosis is induced by intracellular amyloid accumulation that deprives a critical effector molecule, YAP in Hippo signaling pathway essential for cell survival, similarly to TRIAD necrosis observed in transcriptional repression and in other neurodegenerative disease such as Huntington's disease. The initial TRIAD necrosis due to intracellular amyloid releases HMGB1 to extracellular space and induces a cluster of secondary necrosis around the primary necrotic neurons, and finally the cluster grows to an extracellular amyloid plaque. Inhibition of HMGB1 by anti–HMGB1 antibody prevents expansion of neurodegeneration and administration even after the onset significantly ameliorates the cognitive decline of Alzheimer's disease model mice. Our results present a new scheme of Alzheimer's disease pathology, which can be named as “intracellular amyloid hypothesis”.
著者
冨本 秀和
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.38, no.3, pp.337-342, 2021 (Released:2022-01-19)
参考文献数
15

The number of dementia patients is rapidly increasing worldwide, however, in some cohorts in western countries the number shows a decreasing trend oppositely. The initial trial for disease modifying drug has started in 1999 by Schenk et al., but in vail for a long duration. The obstacles to be overcome appear to be an accurate diagnosis of dementia other than Alzheimer's disease, as well as autoimmune encephalitis and a similar but milder condition named amyloid–related imaging abnormalities (ARIA). When the diagnosis of Alzheimer's disease is delayed, accumulated fibrillary amyloid may be degraded and result in ARIA as a perivascular edema and microhemorrhages after administration of anti–amyloid β (Aβ) antibodies. Therefore, an early diagnosis of Alzheimer's disease is pivotal for safe and efficient strategies for disease modifying drugs.At present, amyloid PET study and cerebrospinal fluid (CSF) Aβ42 levels are an established biomarker for early diagnosis of Alzheimer's disease. These biomarkers still have drawbacks because of high cost and invasiveness, respectively. However, more recently, peripheral blood biomarkers have been introduced. Moreover, novel findings on aggregation mechanism of anti Aβ has been revealed, thereby accelerating development of disease modifying drug for Alzheimer's disease.
著者
中島 一郎
出版者
日本神経治療学会
雑誌
神経治療学 (ISSN:09168443)
巻号頁・発行日
vol.36, no.3, pp.220-224, 2019 (Released:2019-11-25)
参考文献数
27

Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein exclusively expressed on the surface of myelin sheath in the central nervous system (CNS). Although the precise function of MOG is not yet known, it is possibly related to the maintenance of the myelin structure. Although the IgG antibodies against MOG (MOG–IgG) are investigated in various demyelinating diseases, pathogenic antibodies had not been identified in any of the diseases. Using CBA, we are now able to detect a disease–specific MOG–IgG of IgG1 subclass. The specific MOG–IgG was initially reported in a study of pediatric demyelinating diseases, followed by studies of seronegative neuromyelitis optica spectrum disorders (NMOSD) and optic neuritis. However, since the patients with MOG–IgG have various phenotype and are relatively rare compared with multiple sclerosis (MS), it is still yet unknown what a prototypic phenotype of this disease is.