WordNet

not performing or scheduled for duties; "Hes off every Tuesday"
at a distance in space or time; "the boat was 5 miles off (or away)"; "the party is still 2 weeks off (or away)"; "away back in the 18th century" (同)away
(of events) no longer planned or scheduled; "the wedding is definitely off" (同)cancelled
in an unpalatable state; "sour milk" (同)sour, turned
below a satisfactory level; "an off year for tennis"; "his performance was off"
no longer on or in contact or attached; "clean off the dirt"; "he shaved off his mustache"
not in operation or operational; "the oven is off"; "the lights are off"
any state or process known through the senses rather than by intuition or reasoning
a remarkable development
the near or foreseeable future; "there was a wedding in the offing"
the part of the sea that can be seen from the shore and is beyond the anchoring area; "there was a ship in the offing"

PrepTutorEJDIC

《分離》(接触しているところ)『から』,から離れて,からはずれて / 《位置》…『から離れたところで』(『に』) / (本来の状態など)からはずれて,…が狂って,がなくなって / (本道など)からそれて,から横道にはいったところに / 《eat, dineなどと共に》…からとって[食べる] / 《『from off』の形で》《話》…から / (ある値段)から割り引いて / 《話》…をやめて,慎んで《+『名』(do『ing』)》 / (仕事義務など)から離れて,を休んで / (人)をたよって,にやっかいになって / (1)《運動・移動を表す動詞と共に》『離れて』,向こうへ / 《状態の動詞と共に》『離れて』 / 《瞬間の動作を表す動詞と共に》『離して』 / 『去って』,出かけて / (電気・ガス・水道などが)『切れて』 / (本来の位置・状態などから)『はずれて』 / (仕事・勤務などを)『旧んで』 / (時間的・空間的に)『離れて』 / 《動作の完了》『すっかり』,完全に / 割り引いて / (中心・的などから)『それた』,『離れた』,はずれた / 『時期はずれの』,(時期などが)いつもほどよくない,シーズン外の / (状態が)水準以下の,いつも調子(状態)でない / 《補語にのみ用いて》(予定・用意されたことが)取りやめになって / 《補語にのみ用いて》(電気器具などが)電源が切れて;(ガス・水道が)切れて,止まって / (人が)…の暮らしぶりで / 勤務のない,休みの / 取れて,ぬげて,はずれて / (馬・車の1対のうち)右側の / 《補語にのみ用いて》(走者が)すでに走り出して / 《おもに英》《補語にのみ用いて》(食物などが)いたんで食べられない;飲めない / 《英》《補語にのみ用いて》(食堂などでメニューには載っているが料理が)もう品切れで / 《英》《補語にのみ用いて》(人の態度が)誠意のない,不親切な,ひどい / 《名詞の前にのみ用いて》《the ~》(クリケットで)打者の右前方の
《所有・所属》…『の』,…のものである,…に属する・《材料・要素》…『でできた』,から成る・《部分》…『の』[『中の』] ・《数量・単位・種類を表す名詞に付いて》…の・《原因・動機》…『で』,のために(because of) ・《主格関係》…『の』,による,によって・《目的格関係》…『を』,の・《同格関係》…『という』・《関係・関連》…『についての』[『の』],の点で・《抽象名詞などと共に》…の[性質をもつ] ・《『It is』+『形』+『of』+『名』+『to』 doの形で,ofの後の名詞を意味上の主語として》・《分離》…『から』・《起原・出所》…『から』[『の』](out of) ・《『名』+『of』+『a』(『an』)+『名』の形で》…のような・《『名』+『of』+『mine』(『yours, his』など独立所有格)の形で》…の…・《時》(1)《副詞句を作って》…に《形容詞句を作って》…の・《時刻》《米》…前(to,《米》before)
『現象』 / 驚くべきこと(物),非凡な人
(陸から見える)沖,沖合い
疲労させる,消耗させる

UpToDate Contents

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1. パーキンソン病における症状の変動およびジスキネジア motor fluctuations and dyskinesia in parkinson disease
2. パーキンソン病の薬理学的治療 pharmacologic treatment of parkinson disease

English Journal

High prevalence of gastroesophageal reflux disease in Parkinson's disease: a questionnaire-based study.

Maeda T, Nagata K, Satoh Y, Yamazaki T, Takano D.Author information Department of Neurology, Research Institute for Brain and Blood Vessels, 6-10 Senshu-Kubota-Machi, Akita 010-0874, Japan.AbstractThe aim of this study is to investigate the frequency and clinical features of gastroesophageal reflex disease (GERD) in Parkinson's disease (PD). Consecutively recruited PD patients and controls were questioned about heartburn and GERD with a questionnaire. In PD patients, disease duration and severity, quality of life, and nonmotor symptoms were also examined and then the clinical features of GERD were analyzed. A total of 102 patients and 49 controls were enrolled and 21 patients and 4 controls had heartburn, significantly frequent in PD. The prevalence rate of GERD was 26.5% in PD and the odds ratio was 4.05. Heartburn, bent forward flexion, and wearing-off phenomenon were frequent, and scores of UPDRS, total and part II, PD questionnaire-39, and nonmotor symptom scale were significantly higher in PD patients with GERD than without GERD. Multiple logistic regression analysis revealed statistical significance in UPDRS part II and nonmotor symptom scale. This study suggests that GERD is prevalent in PD. Deterioration of daily living activities and other nonmotor symptoms can imply the presence of GERD. Because clinical symptoms of GERD are usually treatable, the management can improve the patient's quality of life. Increased attention should be given to detect GERD in PD.
Parkinson's disease.Parkinsons Dis.2013;2013:742128. doi: 10.1155/2013/742128. Epub 2013 Feb 11.
The aim of this study is to investigate the frequency and clinical features of gastroesophageal reflex disease (GERD) in Parkinson's disease (PD). Consecutively recruited PD patients and controls were questioned about heartburn and GERD with a questionnaire. In PD patients, disease duration and seve
PMID 23476890

A twelve-year follow-up study on a case of early-onset parkinsonism preceding clinical manifestation of Gaucher disease.

Machaczka M, Arce MP, Rucinska M, Yoshitake T, Kehr J, Jurczak W, Skotnicki AB, Månsson JE, Tylki-Szymanska A, Svenningsson P.Author information Hematology Center Karolinska, M54, Karolinska University Hospital Huddinge, 141 86, Stockholm, Sweden, maciej.machaczka@ki.se.AbstractMutations in the glucocerebrosidase gene (GBA1) cause Gaucher disease (GD) and are the most common genetic risk factor for the development of Parkinson's disease (PD). Here, we present a 12-year follow-up study of a male with GD and PD (diagnosed 24years ago), which PD preceded the clinical manifestation of GD by 12years. The patient is a compound heterozygote for mutations c.115+1G>A and c.1226A>G (IVS2 + 1/N370S) in the GBA1 gene. Imiglucerase had a beneficial effect on GD, but not on PD. Treatment with L-dopa and other PD drugs showed temporary efficacy but 2years later significant wearing-off phenomenon and dyskinesias appeared. Unilateral pallidotomy was performed with transient benefit. Cognitive decline appeared later and developed in to akinetic mutism. A lumbar puncture was performed to characterize the biochemical profile of cerebrospinal fluid (CSF). Analyses of monoamine metabolites levels in the CSF, determined by reverse-phase high-performance liquid chromatography, revealed remarkably low levels of all studied monoamine metabolites (HVA, DOPAC, 5-HIAA, MHPG). These data indicate that PD associated with GBA1 mutations may not only affect dopaminergic neurons, but also noradrenergic and serotonergic neurons. Of note, normal levels of P-tau, total tau and β-amyloid (1-42) were detected on ELISA assay. Thus, the cognitive decline, akinetic mutism and moderate cortical atrophy found on the CT scan were not paralleled by any changes of dementia markers in CSF. This single case study extends the follow-up period and adds novel CSF information; however additional data on other patients with both PD and GD may help put our observations in its ultimate proper context.
JIMD reports.JIMD Rep.2012;3:53-7. doi: 10.1007/8904_2011_48. Epub 2011 Sep 20.
Mutations in the glucocerebrosidase gene (GBA1) cause Gaucher disease (GD) and are the most common genetic risk factor for the development of Parkinson's disease (PD). Here, we present a 12-year follow-up study of a male with GD and PD (diagnosed 24years ago), which PD preceded the clinical manifest
PMID 23430873

Japanese Journal

Wearing-off Questionnaires(WOQ-19とWOQ-9)の言語的妥当性を担保した日本語版の作成

近藤智善,高橋一司
Brain and nerve : 神経研究の進歩 63(11), 1285-1292, 2011-11
NAID 40019061153

Two cases of Parkinson's disease with the dissection of the motor function and blood levodopa levels

Kawamura Kazuyuki,Arii Takaharu,Nagai Masahiro [他]
Journal of Tokushima National Hospital 2, 40-43, 2011-03
NAID 40019066434

「パーキンソン病」

　　[★]

英: Parkinson disease Parkinson's disease PD
同: Parkinson病; 振戦麻痺 paralysis agitans shaking palsy、特発性パーキンソニズム idiopathic parkinsonism
関: 症候性パーキソニズム、定位脳手術

疫学

50-60歳のに発症
有病率は10万人対約100人

病因

不明

病理

黒質線条体系のドーパミン欠如が成因に関与している (SPC.9)
黒質緻密部メラニン含有細胞の変性萎縮減少と残像神経細胞の細胞内にレビー小体が出現する。レビー小体 Lewy小体
障害部位およびレビー小体の出現部位：黒質緻密部、迷走神経背側運動核、交感神経心臓枝の節後線維、嗅球、青斑核、縫線核、マイネルト基底核、扁桃核。 (HBN.938)
時にレビー小体が大脳皮質に出現。(HBN.938)

病態生理

at 黒質

脳の神経細胞は加齢により減少。

パーキンソン病では減少が早く著しい

↓　

黒質ドパミン神経細胞が変性脱落し、ドパミンを十分合成できない。

線条体コリン神経が優位

症状

1. 運動症状　3大徴候

安静時振戦 rest tremor

固縮 rigidity

無動 akinesia ←　動作緩慢 bradykinesia　と記述される場合もある (HIM.2550)

姿勢保持反射障害 postual instability

2. 精神障害　認知症、うつ病、神経心理学障害
3. 自律神経障害　便秘、起立性低血圧、流涎(ﾘｭｳｾﾞﾝ)、排尿障害、脂漏

症状の頻度

振戦　　　58.2%
歩行障害　24.0%
動作緩慢　20.9%

症状の初発

振戦　＞　歩行障害、固縮、無動
すくみ足や姿勢反射障害で発症することはない

病因

若年性Perkinson病、家族性慢性優性遺伝型Perkinson病
1. Parkin遺伝子の異常、1998年

chr6にあるパーキン遺伝子の変異
パーキンはユビキチンリガーゼの一種
パーキンの変異でパエル受容体が異常蓄積

2. α-synclein遺伝子の異常、1998年

chr4上のα-synuclein gene
リン酸、αシヌクレイン異常蓄積
ドパミン神経細胞師を引き起こす

薬剤性：黒質線条体ドーパミン神経の脱落

除草剤パラコート、園芸用殺虫剤マンネブ、天然系殺虫剤ロテノン

治療

薬物療法

パーキンソン病治療薬

薬物治療の副作用

wearing-off phenomenon：レボドパによって生じる

脳深部刺激療法

その他

症状

these various motor impediments and tremors characteristically begin in one limb (more often the left) and spread to one side and later to both sides until the patient is quite helpless.(APN.1036) 片側性で始まる