著者

宮﨑 雄生

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.39, no.6, pp.S133, 2022 (Released:2022-10-20)

著者

石川 英洋 新堂 晃大 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.39, no.3, pp.346-349, 2022 (Released:2022-11-22)

参考文献数

14

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Kelch–like protein 11 (KLHL11)–associated paraneoplastic syndrome (PNS) has been newly categorized as PNS. The corresponding syndromes are cerebellar and/or brainstem involvement, and in the fewer cases, limbic encephalitis. Hearing loss or tinnitus often precedes other symptoms. Findings of cerebrospinal fluid show inflammatory (protein 50mg/dL or greater and leukocyte counts exceeding 5 cells/µL) with oligoclonal bands in over 80% cases. The most common associated tumor is testicular germ cell tumor, especially seminoma. The primary testicular tumor is often spontaneously regressed with or without metastasis in lymph nodes, called a burned–out testicular tumor. Ultrasonography of testis is useful for such cases because it can detect the suggestive findings of burned–out tumors such as fibrosis and microlithiasis as hypoechoic area. The principles for management are treatment of underlying cancer and immunotherapy. KLHL11–PNS may have a more refractory course than PNS associated with antibodies against neural cell surface antigens since KLHL11 antibodies are targeting intracellular autoantigens and cause cytotoxic T–cell–mediated pathogenesis. A recent paper published by Autoimmune Encephalitis Alliance Clinicians Network recommends that tumor treatment and intravenous methylprednisolone followed by short oral taper should be performed as first–line therapy. If there is no improvement cyclophosphamide can be the next option. Then experimental therapy like IL–6 inhibitor or bortezomib can be considered in non–responded cases.

著者

川本 未知

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.3, pp.296, 2022 (Released:2022-11-22)

著者

古和 久典

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.3, pp.200-203, 2022 (Released:2022-11-22)

参考文献数

13

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The trigeminovascular theory is currently widely accepted as a pathological hypothesis for migraine. Cortical spreading depression (CSD) evoked by unidentified cause may stimulate the trigeminal nerves distributed in the dural and cerebral pial arteries. Inflammatory neurotransmitters such as calcitonin gene–related protein (CGRP) and substance P are released from the trigeminal nerve endings, causing local aseptic inflammation. This local condition propagates to the periphery via axons and further spreads aseptic inflammation, and is transmitted from the trigeminal nerve to the cerebral cortical sensory area via the brain stem, causing pain and various symptoms. Furthermore, it has been suggested that the sensitization phenomenon and the descending pain modulatory pathways are involved in the headache attacks, and that the hypothalamus is involved in the prodrome stage of headache.In addition to triptans and analgesics, empirical medication for the prophylaxis have been used to treat migraine. CGRP has been shown to play a major role in the pathophysiology of migraine in recent years. Studies have suggested that blocking CGRP signaling is an effective preventive and therapeutic strategy in patients with migraine. In Japan, two anti–CGRP antibody drugs and one anti–CGRP receptor antibody drug were launched in 2021, and their usefulness has been shown in clinical practice.

著者

菊井 祥二 竹島 多賀夫

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.4, pp.680-684, 2020 (Released:2021-05-27)

参考文献数

33

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The appearance of sumatriptan in the 1990s was an epoch–making event. Since 2000, five types of triptans have become available for use in Japan. As a result, medical treatment for headaches has become more active and the quality of medical care has dramatically improved. Increasing experience with the use of triptan for the last 20 years has allowed medical professionals to overcome various problems such as the existence of nonresponders and adverse effects of triptan, and triptan treatment has thus matured. However, some patients with migraine do not benefit from the vasoconstrictive effect of triptan stimulated by the 5–hydroxytriptamine 1B receptor. The clinical application of selective 5–hydroxytriptamine 1F receptor agonists (ditans) and calcitonin gene–related peptide receptor antagonists (gepants) that can compensate for the shortcomings of triptan is now progressing, and we hope that such drugs will become the first–choice treatment among many patients with migraine.

著者

藤井 敬之

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.475-477, 2021 (Released:2022-04-28)

参考文献数

18

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Neuropathic pain often impairs quality of life in patients and becomes a global health issue. Recently, interaction between neuropathic pain and autoantibodies have been attracting much attention. Because autoantibody–mediated neuropathic pain is often a good response to immunotherapy, the detection of pain–generating autoantibodies may lead to clinical improvement in patients with intractable neuropathic pain.In this review, we describe the clinical features of autoantibody–mediated neuropathic pain and novel candidate neuropathic pain–related autoantibodies.

著者

山本 悦子 仲俣 菜都美 間嶋 満 倉林 均 高橋 一司 荒木 信夫 山元 敏正

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.3, pp.408-411, 2022 (Released:2022-11-22)

参考文献数

10

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嚥下障害は進行期のParkinson病(Parkinson disease:PD)患者の生命予後に大きく関わる症状の一つであり,病気の進行に伴い緩徐に出現するが,自覚的,他覚的にも気づきにくいことが多い.本研究の目的は,嚥下障害の出現頻度が高い進行期PD患者を対象として,食事摂取の可否に関連する因子を明らかにし,PD患者の嚥下障害を簡便に評価できる方法を検討することである.対象:当院脳神経内科に入院した進行期PD患者37名,平均年齢は77.0±5.8歳(meant±SD),平均罹病年数は7.9±6.3年であった.方法:嚥下造影検査(videofluoroscopic examination of swallowing:VF)の結果から明らかな嚥下障害を認めたか否かをもとに対象例を嚥下障害なし群と嚥下障害あり群の2群に分けた.次に両群を簡便に分別するための因子を検討するために,年齢,性別,罹病期間,入院期間,入院から嚥下造影検査までの日数,Hoehn&Yahr stage,VF検査時点の血清アルブミン値,body mass index,mini mental state examination(MMSE),咽頭反射の有無,反復唾液嚥下テストの値,自己喀痰排出能力,最長発声持続時間(maximum phonation time:MPT),声量,発話明瞭度,握力,歩行能力,入院期間,入院からVF検査までの日数について比較した.結果:握力,自己喀痰排出能力,声量,MPT,咽頭反射で有意差を認めたが,他の項目では両群間に差はなかった.結論:握力,自己喀痰排出能力,声量,MPTが進行期PD患者の嚥下障害の有無を簡便に検出する指標となり得ることが明らかになった.

著者

西川 敦子 西野 一三

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.6, pp.622-626, 2017 (Released:2017-04-30)

参考文献数

41

被引用文献数

2

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Immune–mediated necrotizing myopathy (IMNM) is a subgroup of idiopathic inflammatory myopathies, characterized by usually subacutely progressive course, symmetrical and proximal muscle weakness and atrophy, highly elevated serum creatine kinase levels, and muscle fiber necrosis and regeneration with little or no lymphocytic inflammatory infiltration on muscle pathology. Two major autoantibodies, anti–signal recognition particle (SRP) and anti–3–hydroxy–3–methylglutaryl–coenzyme A reductase (HMGCR) antibodies, are known to be associated with IMNM. IMNM with those antibodies were initially reported in adults but are now known to be seen also in children. Anti–SRP antibodies seem to be more frequently seen in IMNM pateints than anti–HMGCR antibodies. Although anti–HMGCR necrotizing myopathy was first found in patients who were taking statins, majority of patients do not have history of statin use. Myositis associated with anti–aminoacyl tRNA synthetases (ARS) is pathologically characterized by perifascicular necrotizing myopathy with perimysial alkaline phosphatase activity. Clinically, patients typically develop triad of interstitial lung disease, mechanic's hands, and myositis, which is sometimes called anti–synthetase syndrome or anti–ARS syndrome. Of note, necrotizing muscle pathology can also be observed in a variety of other conditions, including myositis associated with anti–mitochondria M2 autoantibodies, malignancy, viral infections (HIV and hepatitis C), and connective tissue diseases. In addition, muscular dystrophy, such as dysferlinopathy or facioscapulohumeral muscular dystrophy, may also display similar muscle pathology. Not surprisingly, it can be clinically difficult to distinguish IMNM from muscular dystrophy especially in children. As IMNM is treatable by immunosuppressive therapy, it should not be misdiagnosed. Immunohistochemical analysis including MHC class I and C5b–9 may be useful as cytoplsmic upregulation of MHC class I in non–necrotic fibers and sarcolemmal deposition of membrane attack complex (C5b–9) are often observed in IMNM but not in muscular dystrophy. This review focuses on muscle pathological features of IMNM.

著者

関 守信

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.582-585, 2022 (Released:2022-12-27)

参考文献数

14

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To date, no anti–Parkinsonian drug has been proven to have disease–modifying effects, but Japanese guideline suggests that treatment should be initiated as early as possible after diagnosis unless there is a specific reason not to. Treatment should be initiated with L–dopa, dopamine agonists, or MAO–B inhibitors. The choice of initial treatment should be based on efficacy, short–term side effects, and avoidance of future motor complications. Currently, many types of dopamine agonists and MAO–B inhibitors are available, and it is possible that the choice of treatment may take into account their effects on non–motor symptoms. The author believes that the algorithm for early–stage Parkinson disease in the guideline is only an indication of what should be the mainstay of early treatment, and that combination treatment that utilizes the characteristics of each drug is important rather than relying on one drug.

著者

鈴木 圭輔

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.559-563, 2022 (Released:2022-12-27)

参考文献数

29

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Sleep disturbance is one of the important non–motor symptoms in Parkinson disease (PD) and affects the quality of life of patients. Early morning off and nighttime motor symptoms can lead to nocturnal and early morning awakenings, and restless legs syndrome can cause difficulty falling asleep. It is also important to screen for sleep apnea, which reduces sleep quality, and rapid eye movement (REM) sleep behavior disorder (RBD), which causes dream–enacting behavior and fragmented nocturnal sleep. PD patients with RBD are associated with characteristic clinical subtypes of PD. In this article, I will discuss the screening of sleep disorders in patients with PD with a focus on RBD and its management.

著者

後藤 淳

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.492-497, 2019 (Released:2020-04-24)

参考文献数

11

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In the emergency settings, rapid identification of stroke is critically important. Furthermore, uncommon causes of strokes also share the clinical significance either in the emergency room or in the general hospital.Uncommon causes of stroke contains arteriopathy (cervicocephalic artery dissections, Ehlers Danlos type IV, moyamoya disease, RCVS/PRES, etc), hematological disorders (Trousseau syndrome, antiphospholipid syndrome, etc) and cerebral venous thrombosis. Recently, some uncommon causes of strokes gain some progress in understanding its evaluation and management such as Fabry disease, PRES, artery dissections, and Moyamoya disease.On behalf of the neurologists' roles in the neurological emergency settings symposium, this article tried to disclose the road map for the era of the uncommon stroke. Uncommon causes of stroke will take neurologists to the new stage for the future stroke clinics with appropriate diagnosis and management.

著者

福永 雅喜

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.604-607, 2022 (Released:2022-12-27)

参考文献数

13

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The magnetic field strength meant by ultra–high field magnetic resonance imaging (MRI) has been changing with the development of MRI, and in recent years, it is commonly used to refer to scanners with a magnet of 7 tesla (7T) or higher. The resolution of MRI depends on tissue relaxation time and contrast, as well as the signal–to–noise ratio (SNR). Increasing the magnetic field strength not only improves SNR, but also enhancing the tissue contrast. 7T MRI has significant advantages over conventional 3T MRI, including practical sub–millimeter order spatial resolution and improved sensitivity in functional MRI (fMRI). 7T MRI enhances intra–tissue (within gray and white matter) susceptibility contrast. In addition, high resolution of fMRI at 7T provides the opportunity for the separation of input and output information based on layer specific analysis in local brain regions. With the recent development of post–processing techniques, a paradigm shift from conventional macroscopic mapping (gyrus and sulcus) to analysis of function–structure relationships at the individual level is anticipated.

著者

森 興太 岡田 靖

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.2, pp.62-66, 2019 (Released:2019-08-02)

参考文献数

15

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Hypertension is the major risk factor for stroke. In the Hisayama study, the risks of cardiovascular disease increased significantly from the lower range (120–129/80–84 mmHg) of prehypertension in a general Japanese population. Since the clinical trial SPRINT demonstrated that targeting a systolic blood pressure of less than 120mmHg reduced fatal and nonfatal major cardiovascular events, blood pressure goals in patients with hypertension currently tend to be more intensive. However, we should conduct more careful antihypertensive treatment when setting blood pressure target in the elderly or patients with cerebral artery stenosis. The meta–analysis including the majority studies for primary stroke prevention has shown that reducing LDL cholesterol with statins reduces the risk of stroke, therefore statin therapy is recommended for patients with dyslipidemia, especially having diabetes mellitus. Early detection and treatment of atrial fibrillation are valuable prevention for stroke risk reduction in the elderly. Anticoagulation should be considered even for the patients with atrial fibrillation (CHADS2 0 or 1) and other vascular risk factors. Regarding primary prevention of stroke for patients with asymptomatic cerebral atherosclerosis and silent lacunar infarction, intensive management of vascular risk factors such as hypertension and diabetes mellitus is the most important treatment for stroke prevention, and antiplatelet therapy should be taken into considered only in patients with high risk of ischemic stroke.It is also essential to educate and enlighten the knowledge of stroke risk factors and warning signs to the general public.

著者

漆葉 章典

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.2, pp.115-122, 2020 (Released:2020-08-31)

参考文献数

63

被引用文献数

2

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Immune–mediated necrotizing myopathy (IMNM) is a subgroup of idiopathic inflammatory myopathies, pathologically characterized by myofiber necrosis with little lymphocytic infiltration. Autoantibodies toward signal recognition particle (SRP) and 3–hydroxy–3–methylglutaryl–coenzyme A reductase (HMGCR) are two major autoantibodies specifically associated with IMNM. Anti–SRP autoantibody–associated IMNM patients are more likely to present with severe muscle weakness and atrophy, cervical muscle weakness, dysphagia, and respiratory insufficiency ; anti–HMGCR autoantibody–associated IMNM patients show a higher proportion of statin use, especially in elderly patients. It is occasionally challenging to distinguish from non–inflammatory myopathies such as muscular dystrophy without autoantibody testing when patients show a relatively slowly progressive course. RNA immunoprecipitation is the original detection method for anti–SRP antibodies, but it requires complicated techniques and is not suitable for clinical use. Therefore, immunoassays using recombinant 54kDa subunit protein of the SRP complex, e.g. immunodot assay and enzyme–linked immunosorbent assay, are commonly used in clinical settings. However, it should be kept in mind that false–negative results can occur in the assays because anti–SRP antibodies occasionally recognize epitopes other than the 54kDa subunit. The pathomechanism has been regarded to involve complement–mediated immunity. In light of recent advances in the pathogenesis, complement–targeting therapy for refractory patients is being discussed.

著者

蕨 陽子 林 健太郎 森島 亮 井上 智之 清水 俊夫 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.5, pp.799-802, 2022 (Released:2023-01-20)

参考文献数

8

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【目的】高齢COVID–19患者の回復期の摂食機能療法の効果と社会的意義について検討する.【方法】高齢者施設を感染経路とし,急性期治療後に当院へ転入院したCOVID–19連続14例の経過を後方視的に検討した.摂食機能療法は医師と看護師,理学療法士で開始し,隔離解除後に言語聴覚士が携わった.【結果】14例は年齢86±7(mean±SD, range 72–95)歳で,8例は認知症,2例は神経変性疾患を有した.COVID–19は86%で肺炎像を呈し,64%で酸素吸入を要した.発症から18.2±5.6(11–33)日経過した当院入院時,9例(63%)が摂食不能であった.入院後,5例は体力や意欲,認知機能が回復し摂食が回復したが,神経変性疾患2例は摂食嚥下機能が回復せず,認知症1例は先行期の問題が回復せず,残る1例は死亡した.【結論】神経変性疾患以外でCOVID–19から回復した高齢者の83%は摂食嚥下機能が回復した.神経難病診療を生かした隔離下での嚥下評価と摂食機能療法が高齢COVID–19患者の摂食嚥下機能回復に寄与し,予後の改善につながった.

著者

王子 悠 服部 信孝

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.5, pp.768-772, 2022 (Released:2023-01-20)

参考文献数

21

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We review reports published in 2021 providing new information on the management of Parkinson disease (PD) and its related disorder. Several clinical trials of drugs for disease–modifying therapy (DMT) are also underway, but no drug has yet been reported that has clearly demonstrated efficacy in either PD or secondary parkinsonism.

著者

平野 照之

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.39, no.5, pp.749-755, 2022 (Released:2023-01-20)

参考文献数

14

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Advance in acute recanalization therapy: The most significant topic in 2021 was the validation of the significance of pre–intravenous administration of alteplase in patients of large vessel occlusion (LVO) planned to mechanical thrombectomy. An integrated analysis of 1,633 patients from DIRECT–MT, DEVT, SKIP, and MR CLEAN–NO IV studies, was performed. The risk difference for functional independence was 1% (95% CI −4–5%) and for symptomatic intracranial bleeding 1% (95% CI −1–3%), suggesting non–inferiority of MT alone to MT plus alteplase in several respects. Another meta–analysis of 433 patients in 4 trials of tenecteplase (TNK) found that effective recanalization with TNK was increased 3.05 (95% CI 1.73–5.40) times compared to those with alteplase. TNK also reduces the time required to recanalize the occluded vessel.Advance in antithrombotic therapy: Dabigatran did not prove efficacy over aspirin among east Asian patients with ESUS (Embolic Stroke of Undetermined Source). Sub–group analysis of CSPS.com study revealed that add–on effect of cilostazol is greater with patients treated with clopidogrel than those with aspirin. Dual antiplatelet therapy (DAPT) using cilostazol might be a potential solution to the genetic polymorphisms in CYP2C19 poor metabolizer.cover poor metabolizer. Cilostazol based DAPT is effective for non–cardioembolic ischemic stroke patients with intracranial arterial stenosis.Blood pressure control: Hypertension is the most powerful risk factor of stroke, even for the patients with ischemic stroke. A meta–analysis of Boncorago et al. revealed that anti–hypertensive therapy reduces the risk of ischemic stroke/TIA (HR 0.79, 95%CI 0.66–0.94). A post–hoc analysis of ATACH–2 reaffirmed that the blood pressure drop should not exceed 90mmHg to avoid acute kidney injury.New desease associated with COVID–19: It is the Thrombosis with Thrombocytopenia Syndrome (TTS). Similar to Heparin–induced Thrombocytopenia, heparin aggravates TTS. Intravenous immunoglobulin and non–heparin anticoagulants should be started as soon as possible.

著者

戸田 達史

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.39, no.4, pp.477, 2022 (Released:2022-12-27)

著者

伊藤 悟

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.34, no.3, pp.215-218, 2017 (Released:2017-10-14)

参考文献数

7

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In the inflammatory disease of the central nervous system, there are meningitis that inflammation is limited in a subarachnoid cavity and encephalitis that inflammation also occur in brain. Because of the high mortality rate and severe aftereffect particularly in encephalitis, early diagnosis and start of therapy are important. The diagnosis of encephalitis is made on the basis of clinical symptoms and cerebrospinal fluid abnormalities. Many cases often need to receive general care in the intensive care unit because of the serious condition. Bacteria, virus, tuberculosis, fungus, parasitic worm and prion protein are representative as causes of infectious encephalitis. Most of the non–infectious encephalitis is caused by autoimmune disease, including connective tissue disease, Behçet's disease, sarcoidosis, paraneoplasic encephalopathy and Hashimoto disease. According to fundamental differences for medical treatment, it is very important to distinguish infectiousness and non–infectiousness encephalitis. I explain the main point of the differential diagnosis, mainly on neurological examination, findings of radiological imaging and an examination of cerebrospinal fluid.

著者

本橋 紀夫 青木 吉嗣

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.430-434, 2022 (Released:2022-12-27)

参考文献数

31

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Muscular dystrophy is genetic muscle diseases following with progressive muscle weakness. Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy, and the gene responsible for DMD was identified by using a positional cloning technique in 1986. Since the discovery of DMD gene, our understanding on the structure of dystrophin protein, dystrophin–associated protein complexes or pathogenesis has dramatically improved. These knowledges obtained by DMD research have provided the basis for potential therapies, and have particularly contributed to the development of therapies targeting mRNA and genomic DNA. Exon–skipping therapy using antisense nucleic acid (AON) targeting pre–mRNA has been successfully correct out–of–frame mRNAs to produce in–frame transcripts by removing an exon during splicing, with the restoration of functionally preserved dystrophin protein, and is currently approved in US and Japan. Recent advances in genetic analysis techniques including next–generation sequencing (NGS) have made it possible to identify many causative genes and proteins, to diagnose multiple types of muscular dystrophies and to develop potential therapies. In addition to DMD, novel technologies have contributed to the elucidation of pathogenesis in several muscular diseases including myotonic dystrophy (DM), Fukuyama congenital muscular dystrophy (FCMD), GNE myopathy and MELAS, paving the way for clinical research and drug discovery. We hope that further innovation will encourage the progress of basic research, and contribute to breakthrough for the development of therapies for non–curable diseases.