著者
髙嶋 博
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.4, pp.472-473, 2018 (Released:2018-02-20)
参考文献数
15
著者
髙畑 克徳 髙嶋 博
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.1, pp.9-18, 2016 (Released:2016-05-20)
参考文献数
19
被引用文献数
1

Autoimmune encephalopathies are clinically and immunologically heterogeneous disorders. Over time, many different types of autoimmune encephalopathy have been discovered. In such clinical situations, we often recognize that patients with autoimmune encephalopathy are often misdiagnosed as exhibiting functional psychogenic movement, conversion, or somatoform disorders. We clinically analyzed 63 patients (14 males and 49 females; age range, 15–79 years) diagnosed with autoimmune encephalopathy in our hospital from 2013 to 2015. Throughout this period we diagnosed almost no conversion disorders in our department. These patients were diagnosed using the diagnostic criteria for each disease, following clinical features showing neurological symptoms of brain origin, responsiveness to immunosuppressive therapy, the existence of known pathological antibodies, and/or history of human papilloma virus (HPV) vaccination. Fourty–two patients showed motor disturbance (weakness, paresis of extremities, or slower pinching) and 35/42 (83.3%) patients showed give–way weakness, indicating disruption of continuous muscle contraction. Fourty–four patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or fast neurologic pain. Surprisingly, most pain was distributed in manner that was not explainable anatomically, while some patients also showed patchy, stocking–glove, or localized pain. Seventeen patients exhibited involuntary movements such as tremor entrainment, dystonia, or coarse involuntary movement. In most patients, such motor, sensory, or involuntary movements were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immune adsorption therapy. We observed memory loss, PNES (psychogenic non–epileptic seizure), dissociative amnesia, hyperventilation, opsoclonus, epilepsy, or autonomic symptoms amongst our patients. Although give–way weakness, anatomically unexplainable pain/abnormal sensation, and strange involuntary movements were thought to be psychogenic, the presence of one of these three symptoms was indicative of autoimmune encephalopathy. As autoimmune encephalitis exhibits diffuse involvement with the whole brain, these symptoms were entirely understandable. Except for the presence of organic disease, most patients were classified into somatoform disorders (DSM–IV, ICD–10) or functional movement disorders. Without first excluding autoimmune encephalopathy, we propose that physicians should not diagnose somatoform disorders. Since autoimmune encephalopathy patients often possess so–called psychogenic signs, it is possible that such signs might be generated by autoimmune encephalopathy instead of somatoform disorders. In conclusion, we propose that give–way weakness and anatomically unexplainable pain/abnormal sensation are key symptoms of autoimmune encephalopathy. We hope that many patients with autoimmune encephalopathy will now be identifiable using our new neurological examination and that each patient can be given an exact diagnosis and therefore be administered with the appropriate treatments.
著者
髙嶋 博
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.160-162, 2017 (Released:2017-10-14)
参考文献数
5

Autoimmune encephalopathies are clinically and immunologically heterogeneous disorders. Many different types of autoimmune encephalopathy have been discovered, and most common type may be Hashimoto encephalopathy in it. In clinical situations, we often recognize that patients with autoimmune encephalopathy are often misdiagnosed as exhibiting functional psychogenic movement, conversion, or somatoform disorders. We clinically analyzed 63 patients with autoimmune encephalopathy. Two–thirds of patients showed motor disturbance mostly with give–way weakness. About 70% of patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or fast neurologic pain. Most pain was distributed in manner that was not explainable anatomically. 27% of patients exhibited involuntary movements such as tremor entrainment, dystonia, or coarse involuntary movement. We observed memory loss, PNES (psychogenic non–epileptic seizure), dissociative amnesia, hyperventilation, opsoclonus, epilepsy, or autonomic symptoms amongst our patients. Although give–way weakness, anatomically unexplainable pain, and strange involuntary movements were thought to be psychogenic, the presence of one of these three symptoms was indicative of autoimmune encephalopathy. As autoimmune encephalitis exhibits diffuse involvement with the whole brain, these symptoms were entirely understandable. Except for the presence of organic disease, most patients were classified into somatoform disorders or functional movement disorders. Without first excluding autoimmune encephalopathy, physicians should not diagnose somatoform disorders.
著者
牧 美充 髙嶋 博
出版者
医学書院
雑誌
BRAIN and NERVE-神経研究の進歩 (ISSN:18816096)
巻号頁・発行日
vol.69, no.10, pp.1131-1141, 2017-10-01

多くの橋本脳症の患者がgive-way weaknessや解剖学的には説明しづらい異常感覚を呈していることをわれわれは見出した。それらは身体表現性障害(いわゆるヒステリー)で特徴的とされる身体症状に類似しており,脳梗塞のような局所的な障害で引き起こされる症状とは切り離されて考えられてきた。そのような神経症候が出現するためには,びまん性,多巣性に濃淡を持った微小病変を蓄積させることができる自己免疫性脳症のような病態を想定する必要がある。このような考え方で,われわれは「びまん性脳障害による神経症候」という新しい診断概念に到達し,実臨床では多くの患者を見出している。今回,抗ガングリオニックアセチルコリン受容体抗体関連脳症,子宮頸がんワクチン接種後に発生した脳症,またはスティッフ・パーソン症候群でも同様の症候がみられることを報告する。自己免疫性脳症の臨床では,抗体の存在だけでなく,自己免疫性脳症による「びまん性脳障害」という概念が重要であり,この新しい診断概念を用いることで診断が困難な自己免疫性脳症の軽症例であっても容易に診断が可能となる。
著者
樋口 雄二郎 髙嶋 博
出版者
一般社団法人 日本内科学会
雑誌
日本内科学会雑誌 (ISSN:00215384)
巻号頁・発行日
vol.104, no.7, pp.1470-1478, 2015-07-10 (Released:2016-07-10)
参考文献数
9

遺伝性ニューロパチーは,運動・感覚または自律神経障害を呈する疾患の総称であり,臨床的にも遺伝的にも非常に多様である.これまでに70以上の原因遺伝子が同定されており,分子病態は細胞内の様々な小器官に複雑に関与していることがわかっている.遺伝性ニューロパチーの遺伝子診断は次世代ゲノムシークエンサー(next-generation sequencer:NGS)を用いたターゲットリシークエンスやエクソーム解析が非常に有用であり,診断確定だけでなく患者の予後予測や治療法の確立につながる.また,分子病態ごとに様々な治療法が開発されており,モデル動物では有効性が確認されている薬剤もあり,なかでもクルクミン治療は我が国でヒトへの臨床試験が計画されている.
著者
髙嶋 博
出版者
日本神経治療学会
雑誌
神経治療学
巻号頁・発行日
vol.34, no.4, pp.472-473, 2018
著者
永田 龍世 稲森 由恵 髙田 良治 池田 賢一 渡邊 修 髙嶋 博
出版者
日本神経学会
雑誌
臨床神経学 (ISSN:0009918X)
巻号頁・発行日
vol.54, no.2, pp.146-150, 2014-02-01 (Released:2014-02-28)
参考文献数
16

症例は80歳男性である.両側性末梢性顔面神経麻痺,四肢筋力低下,四肢末梢・会陰部の感覚障害,尿閉,便秘,四肢腱反射消失をみとめた.バゾプレシン分泌過剰症(syndrome of inappropriate antidiuretic hormone secretion; SIADH),脳脊髄液細胞増多・蛋白上昇をみとめ,セフトリアキソンおよびステロイドパルス治療にて神経症状はすみやかに改善した.脳脊髄液CXCL-13上昇,血清抗ボレリアIgM抗体陽性より神経ボレリア症と診断した.経過中,SIADHの再燃と全身状態の悪化をみとめ,骨髄検査でT細胞型悪性リンパ腫が判明した.近年,ボレリア感染とリンパ腫の関連が報告されており,本例においてもボレリア感染がリンパ腫の発症機序に関連した可能性が考えられた.
著者
浜田 恭輔 武井 藍 﨑山 佑介 森山 宏遠 橋口 昭大 髙嶋 博
出版者
日本神経学会
雑誌
臨床神経学 (ISSN:0009918X)
巻号頁・発行日
vol.58, no.1, pp.30-34, 2018 (Released:2018-01-26)
参考文献数
15

症例は43歳男性である.緩徐に進行する構音障害と歩行失調,幼児退行などの精神症状,脳幹・小脳の萎縮性所見より脊髄小脳変性症が疑われ入院した.口内炎,陰部潰瘍,毛囊炎様皮疹,HLA-B51をみとめ,髄液IL-6高値より慢性進行型神経ベーチェット病と診断した.ステロイド,メトトレキサートの治療効果に乏しく,インフリキシマブで髄液IL-6の減少が得られたが症状は改善しなかった.本症例は広範に不可逆性の脳組織障害が生じた難治例と考えられた.脳幹の萎縮が進行する前に免疫治療を介入すべき疾患であり,精神症状と運動失調症がみられる症例では診断に有用である髄液IL-6を測定することが望ましい.
著者
荒田 仁 髙嶋 博
出版者
一般社団法人 日本内科学会
雑誌
日本内科学会雑誌 (ISSN:00215384)
巻号頁・発行日
vol.106, no.8, pp.1542-1549, 2017-08-10 (Released:2018-08-10)
参考文献数
9

一部の自己免疫性脳症と精神疾患は臨床徴候が類似することが多く,しばしば誤って診断されている.従来の神経診察法のみで正確に診断することは難しく,脳がびまん性に障害された場合の神経徴候を理解するという視点が必要である.見極めるためには詳細な問診と神経診察が重要であり,SPECT(single photon emission computed tomography),甲状腺自己抗体ならびに抗GluR抗体測定が診断に有用である.
著者
吉村 道由 荒田 仁 出口 尚寿 髙嶋 博
出版者
The Japanese Society of Internal Medicine
雑誌
日本内科学会雑誌 (ISSN:00215384)
巻号頁・発行日
vol.103, no.8, pp.1876-1884, 2014

しびれは個々でその定義が異なり,鑑別も非常に多い.高齢者においては,年齢に伴う動脈硬化性変化や骨性変化によりきたす疾患も多くみられる.診断をするにあたっては既往症や基礎疾患などの背景と伴に,発症様式,性状,部位なども重要となる.しびれのメカニズムを述べるとともに,末梢性,中枢性,部位別に分類してそれぞれの代表的な疾患の特徴や,診断をすすめるうえでの検査について述べる.
著者
髙畑 克徳 髙嶋 博
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.1, pp.9-18, 2016

Autoimmune encephalopathies are clinically and immunologically heterogeneous disorders. Over time, many different types of autoimmune encephalopathy have been discovered. In such clinical situations, we often recognize that patients with autoimmune encephalopathy are often misdiagnosed as exhibiting functional psychogenic movement, conversion, or somatoform disorders. We clinically analyzed 63 patients (14 males and 49 females; age range, 15–79 years) diagnosed with autoimmune encephalopathy in our hospital from 2013 to 2015. Throughout this period we diagnosed almost no conversion disorders in our department. These patients were diagnosed using the diagnostic criteria for each disease, following clinical features showing neurological symptoms of brain origin, responsiveness to immunosuppressive therapy, the existence of known pathological antibodies, and/or history of human papilloma virus (HPV) vaccination. Fourty–two patients showed motor disturbance (weakness, paresis of extremities, or slower pinching) and 35/42 (83.3%) patients showed give–way weakness, indicating disruption of continuous muscle contraction. Fourty–four patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or fast neurologic pain. Surprisingly, most pain was distributed in manner that was not explainable anatomically, while some patients also showed patchy, stocking–glove, or localized pain. Seventeen patients exhibited involuntary movements such as tremor entrainment, dystonia, or coarse involuntary movement. In most patients, such motor, sensory, or involuntary movements were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immune adsorption therapy. We observed memory loss, PNES (psychogenic non–epileptic seizure), dissociative amnesia, hyperventilation, opsoclonus, epilepsy, or autonomic symptoms amongst our patients. Although give–way weakness, anatomically unexplainable pain/abnormal sensation, and strange involuntary movements were thought to be psychogenic, the presence of one of these three symptoms was indicative of autoimmune encephalopathy. As autoimmune encephalitis exhibits diffuse involvement with the whole brain, these symptoms were entirely understandable. Except for the presence of organic disease, most patients were classified into somatoform disorders (DSM–IV, ICD–10) or functional movement disorders. Without first excluding autoimmune encephalopathy, we propose that physicians should not diagnose somatoform disorders. Since autoimmune encephalopathy patients often possess so–called psychogenic signs, it is possible that such signs might be generated by autoimmune encephalopathy instead of somatoform disorders. In conclusion, we propose that give–way weakness and anatomically unexplainable pain/abnormal sensation are key symptoms of autoimmune encephalopathy. We hope that many patients with autoimmune encephalopathy will now be identifiable using our new neurological examination and that each patient can be given an exact diagnosis and therefore be administered with the appropriate treatments.