著者

北川 一夫

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.33, no.3, pp.382-386, 2016 (Released:2016-11-10)

参考文献数

12

被引用文献数

3

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Cryptogenic stroke is one–fourth among cerebral infarction, but most of them could be ascribed to embolic stroke. ESUS was proposed for unifying embolic stroke of undetermined sources by Hart et al. in 2014. The etiologies underlying ESUS included minor–risk potential cardioembolic sources, covert paroxysmal atrial fibrillation, cancer–associated coagulopathy and embolism, arteriogenic emboli, and paroxysmal embolism. Extensive evaluation including transesophageal echocardiography and cardiac monitoring for long time could identify the etiology of these patients. Although anti–platelet drug is recommended in ESUS in the current guideline, clinical trials are ongoing to determine the efficacy of non–vitamin K antagonist oral anticoaglulant in ESUS patients.

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.36, no.3, pp.250-253, 2019 (Released:2019-11-25)

参考文献数

18

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Migraine is a common neurological disorder, negatively affecting activities of daily living. Patients with migraine frequently show daytime sleepiness and insomnia. Sleep disturbances worsen headache, while sleep ameliorates headache. We have previously reported increased prevalence of restless legs syndrome and dream enacting behavior in migraine patients. Hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, are characterized by excessive daytime sleepiness not caused by disturbed nocturnal sleep or impaired circadian rhythms. Previous studies showed increased prevalence of migraine in patients with narcolepsy compared with healthy controls. In our multicenter study, migraine was more frequently observed in patients with narcolepsy or idiopathic hypersomnia compared with healthy controls. These findings may suggest pathophysiological similarities between migraine and hypersomnia disorders.

著者

平田 雅之

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.33, no.3, pp.399-404, 2016 (Released:2016-11-10)

参考文献数

19

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Research and development of brain-machine interfaces (BMIs) are now ongoing to support severely disabled people due to intractable neurological disease, spinal cord injury and stroke etc. Implantable brain-machine interfaces using intracranial electrodes require surgical intervention. However, it may achieve higher performance. Here I describe the trends of domestic and international BMI research for clinical application.In USA, clinical research of BMIs using micro–needle electrode array are ongoing in University of Pittsburgh and in Brown University. They reported that three dimensional real time control of a robotic arm and writing using a mouse cursor were successfully performed by severely disabled people. We demonstrated that, real time control of a robotic arm was successfully performed by patients with intractable epilepsy or intractable pain whom intracranial electrodes were temporarily implanted. And most recently we demonstrated that a severely disabled patient with ALS successfully controlled a robotic arm and writing.A fully implantable device is another key to clinical application for implantable BMIs. Development of 40 to 100 channel implantable devices are ongoing in USA, France and Germany. However clinical application of these implantable devices has not been reported yet. We developed a 128–ch electrocorticographic fully implantable device using a contour fitting 3D cortical electrodes and completed chronic implantation in monkey for 6 months. Translational research with companies is indispensable for clinical application. We have to establish a business model and perform strategic pharmaceutical affairs based on it.From technological point of view, implantable BMIs will be clinically applied in the next 5 to 10 years. Now it's time to think well about its application.

著者

神林 崇 今西 彩 富永 杜絵 石戸 秀明 入鹿山 容子 韓 庫銀 木村 昌由美 近藤 英明

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.503-507, 2021 (Released:2022-04-28)

参考文献数

7

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Even though we are currently in the midst of pandemic from coronavirus infection, the new influenza (H1N1) epidemic in 2009–2010 was unforgettable. Concurrently with the H1N1, narcolepsy surged in post–affected children in China. In Northern Europe, narcolepsy surged in children after H1N1 vaccination. Although there were many cases of H1N1 in Japan, there was no change in the incidence of narcolepsy because anti–influenza drugs prevented the disease from becoming more severe and the vaccine did not contain an adjuvant. It has recently become clear that the Spanish flu that prevailed about 100 years ago was also H1N1. Economo's encephalitis lethargica, which was prevalent at the same time, is thought to be autoimmune encephalitis rather than H1N1–induced influenza encephalitis. It has been reported that Economo's encephalitis caused damage to the hypothalamus, including the orexin system, resulting in lethargic symptoms.Since the 2010s, the number of patients with neurodevelopmental disorders (ADHD, ASD) has been increasing among the patients who complain of hypersomnolence. Consideration of the course of symptoms revealed that the patient was originally below the threshold of the diagnostic criteria for neurodevelopmental disease, that hypersomnolence occurred from around adolescence, and that the case also met the criteria for neurodevelopmental disease. Although hypersomnolence was not noticeable in early childhood and inattention was the main symptom, the diagnostic criteria were not met. Hypersomnolence, on the other hand, increased from around adolescence, was added, and attention deficit was exacerbated. Therefore, it is considered that there are many cases that satisfy both the diagnosis of ADHD and central hypersomnia. ADHD characteristics such as attention deficit, hyperactivity, and poor impulsivity may be observed in children who have recovered from Economo's encephalitis and are called post–encephalitis behavioral disorders. The pathophysiology of Economo's encephalitis is presumed to be a disorder of the hypothalamus, including the orexin system, but it is possible that the disorder remained even after recovery.We believe that impaired attention, and restlessness caused by the hypersomnolence in neurodevelopmental disorders can be explained by dysfunctions of the orexin system and its arousal system. H1N1 morbidity may trigger neurodevelopmental disorders accompanied by hypersomnolence.

著者

清水 潤 前田 明子

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.141-145, 2020 (Released:2020-08-31)

参考文献数

9

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The idiopathic inflammatory myopathies (IIMs) are a group of heterogenous conditions showing immune–mediated muscle damage. Increasing numbers of myositis–specific autoantibodies (MSAs) or myositis–associated autoantibodies (MAAs) have been shown to be associated with a majority of patients with IIMs year after year. It has been known that there are associations between these autoantibodies, especially MSAs, and characteristic clinical features. Anti–mitochondrial antibody (AMA) is a characteristic marker of primary biliary cirrhosis. We previously studied clinical features of IIMs associated with AMA and reported. In the report, we found that IIMs associated with AMA frequently include patients with a clinically chronic disease course, muscle atrophy, cardiopulmonary involvement and granulomatous inflammation. After we reported, several case repots mainly in Japanese literature and four case series in English literature have been reported on clinicopathological features of IIMs associated with AMA. Of the four case series, one is negative, one is partly supportive, and two are supportive to our findings. In this review, we summarized the findings of four case series in English literature in comparison with our previous report. We also discussed the reasons of the differences in findings among reports. Considering growing numbers of literature supporting association between AMAs and characteristic clinical features ; chronic disease course, muscle atrophy, and cardiac involvement, we believe AMA should be added to the growing list of myositis–associated antibodies.

著者

坪井 義夫 梅本 丈二 野田 雅子

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.1, pp.27-30, 2022 (Released:2022-06-15)

参考文献数

8

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The amount of weight loss in patients with Parkinson disease (PD) documented in the literature varies from 52 to 65%. As reduced energy input, hyposmia, cognitive impairment, depression, gastrointestinal dysfunction, and dysphagia are thought to be causally related to weight loss. On the other hand, weight loss and malnutrition are also the result of a negative energy balance, which means that energy expenditure exceeds intake caused by tremor, rigidity, and dyskinesia. To predict total energy expenditure, a calculation method to multiply basal energy expenditure by 1.1–1.3, or PD coefficient which is appropriate to the degree of rigidity and tremor has been proposed. For nutrition therapy to replenish energy in PD patients, functional food including medium–chain triglyceride supplements may be helpful.

著者

西上 智彦

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.505-507, 2019 (Released:2020-04-24)

参考文献数

16

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Central sensitization (CS) is defined as an amplification of neural signaling within the central nervous system that elicits pain hypersensitivity and increased responsiveness of nociceptive neurons in the central nervous system to their normal or subthreshold afferent input. Evaluation of central sensitization is assessed by Quantitative Sensory Testing (QST), Brain imaging, and self–administered questionnaire. In the case of knee osteoarthritis, a pressure pain monitor (AlgoMed, Medoc) is used to compress the non–painful forearm at 1kg/s. The pressure felt by the subject as NRS 1 is measured three times at 30–second intervals, and the mean of three times is adopted. The Wind Up phenomenon is evaluated using a method called Temporal Summation (TS). The TS is assessed by differences in the initial and final pain intensity of multiple repeated stimuli, such as pressure and heat stimuli. Decreased descending pain control systems are assessed by the degree to which pain is reduced when noxious stimuli are added outside of pain sites. TS and CPM are used as indicators of central sensitization at the research level, but are rarely used clinically. The reasons for this include the high cost of equipment for evaluation and the time required for measurement. Central Sensitization Inventory (CSI) has been developed as an evaluation of CSS, and high validity and reliability have been reported.CSI consists of Part A (CSI score), which questions the common health–related symptoms of CSS, and Part B, which questions the presence or absence of a history of disease characteristic of CSS.

著者

赤松 恵 山下 雄也 郭 伸

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.2, pp.86-94, 2017 (Released:2017-07-25)

参考文献数

36

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Both TAR DNA binding protein of 43kDa (TDP–43) pathology and failure of RNA editing at the glutamine/arginine (Q/R) site of GluA2, a subunit of the α–amino–3–hydroxy–5–methyl–4–isoxazole propionic acid (AMPA) receptor, are the characteristic etiology–linked molecular abnormalities that concomitantly occur in the motor neurons of the majority of patients with amyotrophic lateral sclerosis (ALS), the most common adult–onset fatal motor neuron disease. Adenosine deaminase acting on RNA 2 (ADAR2) specifically catalyzes RNA editing at the Q/R site of GluA2, and conditional ADAR2 knockout mice (ADAR2flox/flox/VAChT–Cre.Fast ; AR2 mice) exhibit a progressive ALS phenotype with TDP–43 pathology–like TDP–43 mislocalization in the ADAR2–lacking motor neurons. Because Ca2+–permeable AMPA receptor–mediated mechanism underlies death of motor neurons in the AR2 mice, amelioration of exaggerated Ca2+ influx by AMPA receptor antagonists may be a potential ALS therapy. Here we showed that oral perampanel, a selective non–competitive AMPA receptor antagonist, significantly prevented progression of ALS phenotype and death of motor neurons with effective normalization of TDP–43 pathology in the AR2 mice. Given that perampanel has already been approved as an anti–epileptic drug, perampanel would be a potential candidate ALS drug.

著者

細井 昌子 扇谷 昌宏 加藤 隆弘

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.35, no.6, pp.S168, 2018 (Released:2018-11-12)

著者

黒羽 泰子 長谷川 直哉 長谷川 有香 谷 卓 高橋 哲哉 松原 奈絵 高橋 幸利 小池 亮子

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.5, pp.543-546, 2018 (Released:2018-04-05)

参考文献数

11

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症例は48歳男性.37歳,全身けいれん,失語で発症し,左側大脳半球のT2高信号域を認め,carbamazepine, sodium valproateを開始した.42歳,右半身不全麻痺が出現し,48歳,二次性全般化発作が頻回となり,失語,歩行障害が進行した.頭部MRI上,左側大脳半球が高度に萎縮し,脳波で左側前頭極に焦点のある鋭波を認めた.血清,髄液で抗GluN2B抗体が陽性であった.一側性大脳半球萎縮,焦点性てんかん発作があり,知的退行,運動障害が進行性であるため,Rasmussen脳炎と診断し,ステロイドパルス療法を施行した.治療後,二次性全般化発作の頻度が減り,自立歩行も可能となった.Rasmussen脳炎は,主に小児期に発症する自己免疫性てんかんで,早期治療介入が予後を改善する.本症例は,長期経過後の導入にもかかわらずステロイドパルス療法が有効であった.成人発症の緩徐進行性難治性てんかんでは,同疾患の可能性も疑い,長期経過例でも免疫療法を検討する必要があると思われる.

著者

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.33, no.4, pp.542-545, 2017 (Released:2017-02-28)

参考文献数

41

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Articles in 2015 on treatments for autonomic nervous system disorders were reviewed.1. Orthostatic hypotension (OH) : Droxidopa had an improvement in some clinical symptoms due to OH in patients with Parkinson's disease, multiple system atrophy, pure autonomic failure and nondiabetic autonomic neuropathy.2. Constipation : Polyethylene glycol and lubiprostone were effective for the constipation due to slow colonic transit in PD. Management of constipation secondary to defecatory dysfunction due to pelvic floor dyssynergia could be done by levodopa or apomorphine injections, botulinum toxin type A injection into the puborectalis muscle.3. Postural tachycardia syndrome : Inspiratory resistance through an impedance threshold device improved heart rate control in patients with postural tachycardia syndrome during upright posture.4. Vasovagal syncope : Non–pharmacologic treatments including physical counterpressure maneuver and tilt–training and pharmacologic treatments with beta–blocker, fludrocortisone, midodrine and serotonin transporter inhibitors were effective in patients with vasovagal syncope.5. Urinary disturbance : β3–adrenoceptor agonist mirabegron improved the symptoms of overactive bladder (OAB). Antimuscarinics such as solfenacin, imidafenacin, fesoterodine or oxybutynin patch, provided an improvement of OAB. Treatment with solifenacin plus tamsulosin improved the storage and voiding symptoms. Combination treatment with mirabegron and solifenacin improved OAB symptoms. Accupuncture was safe with significant improvements of overactive bladder symptoms. BoNT/A and A/Ona showed benefits in treatment of refractory OAB. Percutaneous tibial nerve stimulation and sacral nerve stimulation showed effectiveness for treatment of OAB.6. Hyperhidrosis : Oxybutynin for treating plantar hyperhidrosis and topical glycopyrrolate for treatment of facial hyperhidrosis were effective. A combination of BoNT/A, B and anticholinergics improved compensatory hyperhidrosis after sympathectomy. Video–assisted thoracic sympathicotomy for the treatment of palmar and axillary hyperhidrosis showed the long–term effectiveness. Sympathotomy by clamping at T3 was less effective in reducing the primary symptom of postoperative palmar sweating, but induced less compensatory sweating than did sympathotomy by cutting at T3. Tumescent suction curettage was an effective and safe treatment for axillary hyperhidrosis.

著者

堺 竜介 甲田 亨 馬場 孝輔 奥野 龍禎 中辻 裕司 望月 秀樹

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.34, no.1, pp.47-50, 2017 (Released:2017-05-31)

参考文献数

9

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症例は69歳の男性である.右耳介の発赤腫脹の後,活動性の低下,パーキンソニズムを呈し,頭部MRIにて両側対称性に大脳基底核に高信号を認めた.その後,精神症状が亜急性に進行し,髄液中の細胞と蛋白の増加があり,造影効果も認め,自己免疫性脳炎と考えてステロイドパルス療法を実施した.改善を認め転院となったが,その後辺縁系を含め広範囲に病変を呈し当院へ再入院となった.ステロイドパルス療法を実施し症状,頭部MRI画像での改善が再度得られた.後に抗N–methyl–D–aspartate(NMDA)受容体抗体が陽性と判明,経過を通じて悪性腫瘍の合併も明らかではなく非典型的な経過ではあるが,抗NMDA受容体脳炎と考えられた.パーキンソニズムで発症し,病初期の画像は基底核病変のみで,緩徐に進行する脳炎でも抗NMDA受容体脳炎を考慮し,精査する必要があると考えられる.

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.4, pp.573-609, 2017 (Released:2017-02-28)

著者

谷口 洋 坂本 剛 鈴木 重明

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.661-665, 2021 (Released:2022-04-28)

参考文献数

16

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The effect of immune checkpoint inhibitors has become clear, and its indication is expanding. Contrary to its effects, immune related adverse events (irAE) have become a problem. Neurologic irAE are relatively rare, yet potentially fatal. Neurologists should make appropriate diagnoses and treatments.

著者

清水 潤

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.537-542, 2020 (Released:2021-05-27)

参考文献数

20

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The idiopathic inflammatory myopathies (IIMs, also known as myositis) are a heterogeneous group of autoimmune disorders involving skeletal muscle. IIMs are mainly characterized with associated clinical complications, pathological findings, and myositis autoantibodies. It has been known that about 70 percent of patients have myositis autoantibodies ; myositis–specific autoantibodies (MSAs) or myositis–associated autoantibodies (MAAs). Because a number of MSAs/MAAs correlate with specific clinical features of patients with IIMs, and only one of MSAs is detected in individual patients, it is presumed that autoantibodies or their target molecules correlate with underlying pathomechanisms of IIMs.For the treatment of IIM, high–dose oral corticosteroid therapy is used for the first–line therapy mainly based on experience. As second–line therapies, immunosuppressants are used as steroid–sparing agents or when the disease is refractory. Intravenous immunoglobulin therapy is considered in the patients who failed to respond to first–line therapy. Therefore, at present, most of the immunotherapies applied to IIMs patients are nonspecific in spite of heterogeneity of underlying pathological mechanisms. Currently, numerous synthetic and biological immunosuppressive agents are available to treat rheumatic diseases including IIMs. To develop more specific therapies targeting underlying pathogenic pathways, further studies on better classification of IIMs and further therapeutic studies on subsets of classified IIM patients should be necessary.

著者

國本 雅也

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.35, no.4, pp.432-436, 2018 (Released:2019-04-22)

参考文献数

8

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Orthostatic hypotension (OH), anhidrosis, and pupil abnormalities are clinically common autonomic symptoms. Subjective symptoms of orthostatic hypotension include lightheadedness, fainting, and pale vision. The degree of OH can be measured at the bedside in patients lying supine or standing, using a sphygmomanometer. The pathogenesis of OH can be indicated by assessing positionally related changes in blood pressure and heart rate. Anhidrosis can be indicated by measuring body temperature under warm or hot conditions. Sweating that is insufficient to compensate for increases in body temperature produces unpleasant feelings and abnormally increased body temperature. A poor ability to sweat can be estimated simply by touching dry skin and more precisely determined by generalized thermal sweating tests using the Minor method. Pupil abnormalities can be identified by close examination of the eyes under light and dark conditions. Myosis, ptosis, and anhidrosis in the affected side of a face are the triadic symptoms of Horner syndrome, in which the sympathetic tract from the upper thoracic spinal cord to the eye is disturbed. Remarkable mydriasis can be determined under conditions of light in patients with Adie syndrome, in whom the accommodation reflex remains preserved. These symptoms can be easily assessed at the bedside and should be interpreted appropriately to solve clinical problems associated with the autonomic nervous system.

著者

菊池 友和 山口 智 鈴木 真理 荒木 信夫

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.33, no.3, pp.480-483, 2016 (Released:2016-11-10)

参考文献数

12

被引用文献数

1

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1ヶ月以上薬物療法を行い効果の得られなかった反復発作性緊張型頭痛(frequent episodic tension–type headache:FTTH)と慢性TTH(chronic TTH:CTTH)に分類し,鍼治療を行い,Visual Analogue Scale(VAS)による自覚症状の評価が50%以上改善したのを有効として,両群の治療回数・期間について検討した.対象は,TTH221例.FTTH82例(男28,女54,平均年齢52.7歳)とCTTH139例(男44,女95,平均年齢50.4歳)である.FTTHの有効率80.1%,CTTHの有効率59.9%であり,FTTH群の方が有意に高値であった(p<0.01).回数・期間はFTTH 2.8回14.9日,CTTH8.9回35.9日であり,FTTHの方が少ない回数で短期間に改善した(p<0.01).FTTHは,鍼治療を3回または2週間継続し,さらにCTTHは9回または5週間継続し効果判定する必要性が示された.

著者

前田 哲也

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.253-256, 2017 (Released:2017-10-14)

参考文献数

12

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People living with Parkinson's disease are stuck in the driving issues in Japan. Parkinson's disease is the most common movement disorder, in which dopamine replacement therapy (DRT) can relieve the severity of motor symptoms. There are two independent aspects in the driving problems, such as driving skills associating with motor deficits and sudden onset of sleep at the wheel associating with DRT. Instead of on road assessment of driving fitness, a driving simulator is increasingly used for clinical research and practice, which is expected supplying novel practical evidences. In contrast, DRT associating sleep problems are clinically delicate because DRT is essential for treatment of Parkinson's disease and is uneasy to be withdrawn even if sudden onset sleepiness was induced. Alternatively, people living with Parkinson's disease who need to drive in their daily lives are not so rare. There is neither legal restrictions in their driving nor practical guidelines of driving in Japan. This review summarizes such conflicting problems of the driving issues surrounding Parkinson's disease in Japan.

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.5, pp.769-812, 2020 (Released:2021-07-21)

著者

井上 道雄 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.37, no.2, pp.123-128, 2020 (Released:2020-08-31)

参考文献数

34

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Dermatomyositis (DM) is characterized clinically by myositis and a unique set of skin eruptions and myopathologically by perifascicular atrophy and capillary C5b–9 depositions. In DM muscles, a set of proteins known to be induced by type I interferon, including myxovirus resistance protein A (MxA), are expressed. Sarcoplasmic MxA on immunohistochemistry is highly specific and sensitive for DM, and thus a useful diagnostic marker. In addition, an increasing number of reports have been recently describing the efficacy of JAK inhibitors, which block the type I interferon pathway, in patients with refractory DM. Therefore, DM can be regarded as muscle type I interferonopathy. Five DM–specific autoantibodies (DMSA) so far identified are against : TIF1–γ, MDA5, Mi–2, NXP–2, and SAE. In our cohort, 97% of DM cases confirmed by the sarcoplasmic MxA expression on muscle biopsy had one of DMSAs, suggesting that DMSA is a surrogate marker of muscle type I interferonopathy. Apparently, each DMSA is associated with a relatively specific phenotype. For example, anti–TIF1–γ autoantibodies are associated with malignancy and anti–MDA with clinically amyopathic DM. This indicates that DM may well be further subclassified by DMSA.Antisynthetase syndrome (ASS) is characterized by the presence of anti–aminoacyl t–RNA synthetases (ARS). So far, 10 anti–ARS antibodies have been identified, including Anti–Jo–1, PL–7, EJ, OJ, PL–12, and KS autoantibodies. ASS was previously classified as DM because patients typically develop skin rash such as mechanic's hand, in addition to myositis and chronic idiopathic lung disease. However, a recently proposed classification of idiopathic inflammatory myopathies (IIMs) based upon pathological and serological features, in addition to clinical information, includes ASS as a distinct subtype of IIM. Mounting evidence indicates the correlation between autoantibodies and phenotypes also in ASS. Anti–Jo–1, PL–7, EJ, OJ autoantibodies are more closely associated with myositis rather than interstitial lung disease while anti–PL–12, KS autoantibodies are more closely correlated with interstitial lung disease. ASS is pathologically characterized by perifascicular necrosis and perifascicular pathology. Perifascicular necrosis may superficially resemble perifascicular atrophy, however, MxA is not expressed in myofibers in ASS, indicating ASS and DM have different pathomechanisms. Of note, anti–OJ autoantibodies, which are seen in 27% of ASS patients who received muscle biopsy, cannot be detected by line blot assay or ELISA.