マリー・シュトリュンペル病
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- 1. シャルコー・マリー・トゥース病：マネージメントおよび予後charcot marie tooth disease management and prognosis [show details]
… Charcot-Marie-Tooth disease (CMT) consists of a spectrum of disorders caused by mutations in various genes whose protein products are expressed in myelin and/or axonal structures within peripheral nerves…
- 2. シャルコー・マリー・トゥース病：遺伝的特性、臨床的特徴、診断charcot marie tooth disease genetics clinical features and diagnosis [show details]
…neuropathies were designated by eponyms that had the connotation of specific clinical features (eg, Charcot-Marie-Tooth disease [CMT] or Dejerine-Sottas disease). However, phenotypic variability resulted in substantial …
- 3. 遺伝性ニューロパチーの概要overview of hereditary neuropathies [show details]
…neuropathies were designated by eponyms that had the connotation of specific clinical features (eg, Charcot-Marie-Tooth [CMT] disease or Dejerine-Sottas disease). However, phenotypic variability resulted in substantial …
- 4. 神経筋疾患の超音波診断diagnostic ultrasound in neuromuscular disease [show details]
…Charcot-Marie-Tooth disease, in which palpably enlarged nerves were confirmed to be of increased size with ultrasound . The extent and degree of enlargement, which are greatest in Charcot-Marie-Tooth type …
- 5. 末梢神経疾患および筋疾患の鑑別診断differential diagnosis of peripheral nerve and muscle disease [show details]
…overcome weakness in foot dorsiflexors, can be seen in patients with polyneuropathy such as Charcot-Marie-Tooth disease. A waddling gait is characteristic of patients with proximal muscle weakness, as with …
English Journal
- The Relationship of Ankylosing Spondylitis and Subclinical Atherosclerosis: A Systemic Review and Meta-Analysis.
- Bai R, Zhang Y, Liu W, Ma C, Chen X, Yang J, Sun D.
- Angiology. 2019 Jul;70(6)492-500.
- Cardiovascular disease has become the first cause of death for patients with ankylosing spondylitis (AS). However, the relevant data is limited and the relationship between AS and subclinical atherosclerosis remains questionable. Therefore, we assessed the relationship between subclinical atheroscle
- PMID 30497278
- Effect of cigarette smoking on sexual functions, psychological factors, and disease activity in male patients with ankylosing spondylitis.
- Aykurt Karlıbel İ, Dülger S, Kasapoğlu Aksoy M, Güzelsoy M, Türkoğlu AR, Altan L, Yıldız T.
- The aging male : the official journal of the International Society for the Study of the Aging Male. 2019 Jun;22(2)109-115.
- This study aims to investigate the effect of smoking on sexual functions in AS patients. A total of 67 male AS patients with a median age of 34 years (range: 18-57) reporting sexual activity at least for the past 4 weeks period were included. Patients were divided into smokers (Group 1, n = 47
- PMID 29863428
- Genetic association of non-MHC region with ankylosing spondylitis in a Chinese population.
- Liu J, Pu W, Li Y, Ma Y, Zhu Q, Wan W, Yang C, Wang X, Chen X, Zhou X, Reveille JD, Jin L, Zou H, Wang J.
- Annals of the rheumatic diseases. 2019 Jun;78(6)852-853.
- PMID 30552171
Related Links
- Looking for online definition of Marie-Strumpell disease in the Medical Dictionary? Marie-Strumpell disease explanation free. What is Marie-Strumpell disease? Meaning of Marie-Strumpell disease medical term. What does Marie ...
- arthritis of the spine, resembling rheumatoid arthritis, which may progress to bony ankylosis with ossification of the anterior and posterior longitudinal ligaments; the disease is more common in males, often with the rheumatoid factor ...
- Marie-Strümpell disease: A synonym for ankylosing spondylitis. Named for the French neurologist Pierre Marie (1853-1940) and the German neurologist whose full name was Ernst Adolf Gustav Gottfried von Str¨mpell ...
★リンクテーブル★
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- 英
- ankylosing spondylitis AS
- 同
- リウマチ性脊椎炎、ベヒテレフ病、マリー-シュトリュンペル病 Marie-Struempell disease
- 関
- 血清反応陰性脊椎関節症
概念
- リウマトイド因子陰性で脊椎と仙腸関節が侵される疾患。
原因
疫学
- 発生頻度は0.04%程度で家族内発生が多い。
- 好発年齢：10歳台後半～20歳代台
- 男女比：90%男性。 男:女=9:1-5:1
病態
- 靭帯付着部などの関節辺縁に限局した骨炎が生じ、軟骨下骨や線維軟骨が肉芽組織に置換され、炎症が収まるのに伴いこの肉芽組織が骨化し、骨強直を来す。
- 踵骨部・坐骨結節に腱付着部症、仙腸関節である。
症状
- 腰痛、殿部や背部の痛み。
- 運動制限
- 深呼吸時の胸痛、関節痛、アキレス腱痛
合併症
検査
血液検査
- リウマチ因子：陰性
- HLA-B27：90%の症例で陽性
- 赤沈：亢進
X線像
- 初期は仙腸関節部の両側性の辺縁不整。びらんが出現すると関節裂隙が開大して見える。次いで、びらんの周辺に硬化像が認められ、関節裂隙は狭小化し、最終的に硬直に至る(骨性強直)。
- 脊椎：前縦靭帯の椎体付着部からの骨化(椎体骨棘形成 syndesmophyte) → 側面像での椎体の方形化 squaring → 竹様脊柱 bamboo spine
診断
診断基準(東京都特殊疾病(難病)息者診断手引き, 1990年)
- 1.腰痛(最低3カ月以上,運動で軽快し,安静による効果なし)
- 2.腰椎の可動制限(失状および前額面)
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- 後腸骨棟の高さで,垂直に測定した10cmの間隔が前屈で何cm伸延したかを計測：異常:5cm以下
- 腋窩正中腺状,任意に引かれた20cmの線が側屈で何cm伸延したかを計測：異常:5cm以下
- 胸郭拡張測定検査
- 第四肋間の高さで,最大呼気時の胸囲と最大吸気時の胸囲との差を計測：異常: 2.5cm以下
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- (1)両側仙腸開節炎 2-3度
- (2)片側仙腸関節炎 3-4度
- 0度:正常
- 1度:疑い
- 2度:軽度(小さな限局性の侵食像や硬化像)
- 3度:中等度(侵食像や硬化像の拡大,関節裂隙狭小)
- 4度:強直
- 確実例:主要症状1. 2, 3のうち1項目以上+必要検査1の(1)
- 疑い例:主要症状なし+必要検査1の(1)あるいは(2)
- 付記: HLA-B27の成績を記載のこと
治療
- 運動療法
- 薬物療法：NSAIDs。DMARDsも使われうる。
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- 英
- Marie-Struempell disease
- 関
- 強直性脊椎関節炎
[★]
- 英
- Marie-Struempell disease
- 関
- 強直性脊椎炎
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- 疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)
- 特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)
- something that is very wrong with people's attitudes, way of life or with society.
- 関
- ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness
- disease ≠ illness ≠ disorder
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急性流行性白質脳炎。シュトリュンペル病