motor neuron disease

運動ニューロン疾患

WordNet

1. an impairment of health or a condition of abnormal functioning
2. machine that converts other forms of energy into mechanical energy and so imparts motion
3. a nonspecific agent that imparts motion; "happiness is the aim of all men and the motor of all action"
4. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological
5. the act of driving an automobile

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. (電気の)『モーター』,電動機 / エンジン,(特に)内燃機関 / 《英》自動車(motorcar) / 《名詞の前にのみ用いて》 / モーターの;エンジンの;自動車の / モーター(エンジン)による,自動車による / (神経・筋肉について)運動の / 自動車で行く / …‘を'自動車で運ぶ
4. 病気にかかった / 病的な,不健全な(morbid)
5. 自動車運転〈技術〉,ドライブ
6. 神経単位,神経細胞,ニューロン,ノイロン

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/07/19 10:49:21」(JST)

wiki en

[Wiki en表示]

This article is about a category of neurological disorders. It is not to be confused with motor neurone disease which is another name for amyotrophic lateral sclerosis.

Motor neuron disease
spinal diagram
Classification and external resources
Specialty	Neurology
ICD-10	G12.2
ICD-9-CM	335.2
DiseasesDB	8358
MeSH	D016472

A motor neuron disease (MND) is any of five neurological disorders that selectively affect motor neurons, the cells that control voluntary muscles of the body. These five conditions are amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy. They are neurodegenerative in nature and cause increasing disability and eventually, death.

Terminology

Technically the term "motor neuron disease" includes five diseases: amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy.

In the United States the term is often used interchangeably with ALS. In the United Kingdom "motor neurone disease" may be used to mean ALS.

While MND refers to a specific subset of similar diseases, there are numerous other diseases of motor neurons that are referred to collectively as "motor neuron disorders", for instance disease belonging to spinal muscular atrophies. However, they are not classified as "motor neuron diseases" by the tenth International Statistical Classification of Diseases and Related Health Problems (ICD-10) which is the definition followed in this article.

Classification

Motor neuron diseases affect either upper motor neurons (UMN) or lower motor neurons (LMN), or both:

Type	UMN degeneration	LMN degeneration
Amyotrophic lateral sclerosis (ALS)	Yes	Yes
Primary lateral sclerosis (PLS)	Yes	No
Progressive muscular atrophy (PMA)	No	Yes
Progressive bulbar palsy (PBP)	No	Yes, bulbar region
Pseudobulbar palsy	Yes, bulbar region	No

References

External links

• Motor neuron diseases at NINDS

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 筋萎縮性側索硬化症およびその他の運動ニューロン疾患の臨床的特徴 clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease
• 2. 筋萎縮性側索硬化症およびその他の運動ニューロン疾患の診断 diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease
• 3. 筋萎縮性側索硬化症の疫学および病因 epidemiology and pathogenesis of amyotrophic lateral sclerosis
• 4. 家族性筋萎縮性側索硬化症 familial amyotrophic lateral sclerosis
• 5. 筋萎縮性側索硬化症の病態修飾療法 disease modifying treatment of amyotrophic lateral sclerosis

English Journal

• Dysfunctions within limbic-motor networks in amyotrophic lateral sclerosis.

• Passamonti L, Fera F, Tessitore A, Russo A, Cerasa A, Gioia CM, Monsurrò MR, Migliaccio R, Tedeschi G, Quattrone A.SourceConsiglio Nazionale delle Ricerche (CNR), Istituto di Scienze Neurologiche (ISN), Unità di Ricerca Neuroimmagini, Catanzaro, Italia. Electronic address: luca.passamonti@cnr.it.
• Neurobiology of aging.Neurobiol Aging.2013 Nov;34(11):2499-509. doi: 10.1016/j.neurobiolaging.2013.05.016. Epub 2013 Jun 25.
• Previous studies have shown that affective symptoms are part of the clinical picture in amyotrophic lateral sclerosis (ALS), the most common motor neuron disorder in elderly people. Diffuse neurodegeneration of limbic regions (e.g., prefrontal cortex [PFC], amygdala) was demonstrated in ALS post-mor
• PMID 23806980

• Glial A30P alpha-synuclein pathology segregates neurogenesis from anxiety-related behavior in conditional transgenic mice.

• Marxreiter F, Ettle B, May VE, Esmer H, Patrick C, Kragh CL, Klucken J, Winner B, Riess O, Winkler J, Masliah E, Nuber S.SourceDepartment of Molecular Neurology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nuremberg, 91054 Erlangen, Germany.
• Neurobiology of disease.Neurobiol Dis.2013 Nov;59:38-51. doi: 10.1016/j.nbd.2013.07.004. Epub 2013 Jul 16.
• In Parkinson's disease (PD) patients, alpha-synuclein (α-syn) pathology advances in form of Lewy bodies and Lewy neurites throughout the brain. Clinically, PD is defined by motor symptoms that are predominantly attributed to the dopaminergic cell loss in the substantia nigra. However, motor deficit
• PMID 23867236

• Chronic treatment with lithium does not improve neuromuscular phenotype in a mouse model of severe spinal muscular atrophy.

• Dachs E, Piedrafita L, Hereu M, Esquerda JE, Calderó J.SourceUnitat de Neurobiologia Cel·lular, Departament de Medicina Experimental, Facultat de Medicina, Universitat de Lleida and Institut de Recerca Biomèdica de Lleida (IRBLLEIDA), Av. Rovira Roure 80, 25198 Lleida, Catalonia, Spain.
• Neuroscience.Neuroscience.2013 Oct 10;250:417-33. doi: 10.1016/j.neuroscience.2013.07.026. Epub 2013 Jul 19.
• Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by defective levels of the survival motor neuron (SMN) protein. SMA causes spinal motoneuron (MN) loss, and progressive muscle weakness and paralysis. Currently, there is no effective therapy to cure this disease.
• PMID 23876328

Japanese Journal

• 軟口蓋麻痺を合併した運動ニューロン病患者の腹腔鏡手術にair-Q blockerを用いた1症例

• 桑村 歩,駒澤 伸泰,松波 小百合 [他]
• 麻酔 64(8), 830-832, 2015-08
• NAID 40020554894

• 近位筋優位遺伝性運動感覚ニューロパチーの2症例

• 森 千晃,齋藤 朋子,齊藤 利雄,藤村 晴俊,佐古田 三郎
• 臨床神経学 55(6), 401-405, 2015
• … 性の近位筋優位筋力低下・筋萎縮，四肢遠位のしびれ，四肢腱反射消失を呈し，神経伝導検査で感覚神経が導出不能であった男性2例を報告した．1例は祖父母が沖縄県，もう1例は両親が滋賀県出身で，常染色体優性遺伝と考えられる家族歴を有していた．また，1例では四肢や体幹に有痛性筋けいれんが頻発していた．近位筋優位遺伝性運動感覚ニューロパチー（hereditary motor and sensory neuropathy with proximal dominant …
• NAID 130005083891

• Risk of Amyotrophic Lateral Sclerosis in Patients With Diabetes: A Nationwide Population-Based Cohort Study

• Sun Yu,Lu Chien-Jung,Chen Rong-Chi,Hou Wen-Hsuan,Li Chung-Yi
• Journal of Epidemiology 25(6), 445-451, 2015
• … hazard ratios (HRs) of ALS (ICD-9-CM 335.20) in relation to diabetes using a Cox proportional hazard regression model, with adjustment for potential confounders, including sex, age, geographic area, urbanization status, Charlson Comorbidity Index, frequency of medical visit, and histories of hypertension, hyperlipidemia, and chronic obstructive pulmonary disease.<BR>Results: Over a 9-year period, 255 diabetic and 201 non-diabetic subjects developed ALS, corresponding to incidence densities of 7.42 and 5.06 per 100 000 person-years, …
• NAID 130005074467

Related Links

• Amyotrophic lateral sclerosis - Wikipedia, the free encyclopedia

Amyotrophic lateral sclerosis (ALS), also referred to as motor neuron disease in British English, is the most common form of the motor neuron diseases. The condition is sometimes called Lou Gehrig's disease in North America, after the New ...

Related Pictures

---

★リンクテーブル★

リンク元	「脊髄性筋萎縮症」「運動ニューロン疾患」「lateral sclerosis」「motor system disease」「anterior horn cell disease」
拡張検索	「secondary motor neuron disease」「motor neuron diseases」
関連記事	「disease」「motor」「neuro」

「脊髄性筋萎縮症」

　　[★]

英

spinal muscular atrophy, SMA

同？

脊髄進行性筋萎縮症 progressive spinal muscular atrophy

関

筋萎縮、運動ニューロン疾患, motor neuron disease, MND

運動ニューロン病

BET.439

• 上位運動ニューロン and/or 下位運動ニューロンが選択的に傷害される疾患の総称

上位運動ニューロン障害	原発性側索硬化症 PLS
上位+下位運動ニューロン障害	筋萎縮性側索硬化症 ALS		孤発性/家族性
下位運動ニューロン障害	脊髄性筋萎縮症 SMA	SMA type 1, infantile SMA, Werdnig-Hoffmann disease ウェルドニッヒ・ホフマン病 WH	AR
		SMA type 2, intermediate SMA
		SMA type 3, juvenile SMA, Kugelberg-Welander disease クーゲルベルク・ウェランダー病 KW	AR
	球脊髄性筋萎縮症 BSMA Kennedy-Alter-Sung disease ケネディ・オルター・スン症候群 KAS		XR、CAGリピート

遺伝形式

• 常染色体劣性遺伝

原因遺伝子

• 5q13.2に座乗するSMN1遺伝子の変異。

症状

• 下位運動ニューロンの脱落・変性
• 近位筋優位の筋萎縮　　　←　　　神経原性の筋萎縮なのに・・・　　(cf. 筋萎縮)

参考

uptodate

• 1. [charged] 脊髄性筋萎縮症 - uptodate [1]

OMIM

• 1. SPINAL MUSCULAR ATROPHY, TYPE I; SMA1, Gene map locus 5q12.2-q13.3

http://omim.org/entry/253300

• 2. SPINAL MUSCULAR ATROPHY, TYPE II; SMA2, Gene map locus 5q12.2-q13.3

http://omim.org/entry/253550

• 3. SPINAL MUSCULAR ATROPHY, TYPE III; SMA3, Gene map locus 5q12.2-q13.3, 5q12.2-q13.3

http://omim.org/entry/253400

• 4. SPINAL MUSCULAR ATROPHY, TYPE IV; SMA4, Gene map locus 5q12.2-q13.3

http://omim.org/entry/271150

「運動ニューロン疾患」

　　[★]

英

motor neuron disease, MND

関

筋萎縮

• 神経系 091102IV

運動ニューロン病(BET.439)

• 上位運動ニューロン and/or 下位運動ニューロンが選択的に傷害される疾患の総称

上位運動ニューロン障害	原発性側索硬化症 PLS
上位+下位運動ニューロン障害	筋萎縮性側索硬化症 ALS
下位運動ニューロン障害	脊髄性筋萎縮症 SMA	SMA type 1, infantile SMA, Werdnig-Hoffmann disease
		SMA type 2, intermediate SMA
		SMA type 3, juvenile SMA, Kugelberg-Welander disease
	球脊髄性筋萎縮症 BSMA Kennedy-Alter-Sung disease

「lateral sclerosis」

　　[★] 側索硬化症

関

anterior horn cell disease、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease

「motor system disease」

　　[★]

• 運動系疾患

関

anterior horn cell disease、lateral sclerosis、lower motor neuron disease、motor neuron disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease

「anterior horn cell disease」

　　[★]

• 前角細胞疾患

関

lateral sclerosis、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、secondary motor neuron disease、upper motor neuron disease

「secondary motor neuron disease」

　　[★]

二次運動ニューロン病

関

anterior horn cell disease、lateral sclerosis、lower motor neuron disease、motor neuron disease、motor system disease、primary lateral sclerosis、upper motor neuron disease

「motor neuron diseases」

　　[★] 運動ニューロン疾患

「disease」

　　[★]

• n.

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

• 注意

disease ≠ illness ≠ disorder

　 　 　

「motor」

　　[★]

• adj.

• 運動の

• n.

• モーター

関

exercise、kinesis、locomote、locomotive、motility、motion、move、movement

　 　 　

「neuro」

　　[★]

• comb form.

• ニューロ、神経

関

nerve、nervi、nervous、nervus、neural、neuron