n.

脱力感、脱力。衰弱

WordNet

the condition of being financially weak; "the weakness of the dollar against the yen"
the property of lacking physical or mental strength; liability to failure under pressure or stress or strain; "his weakness increased as he became older"; "the weakness of the span was overlooked until it collapsed"
a penchant for something even though it might not be good for you; "he has a weakness for chocolate"
desert (a cause, a country or an army), often in order to join the opposing cause, country, or army; "If soldiers deserted Hitlers army, they were shot" (同)desert
an imperfection in an object or machine; "a flaw caused the crystal to shatter"; "if there are any defects you should send it back to the manufacturer" (同)fault, flaw
an imperfection in a bodily system; "visual defects"; "this device permits detection of defects in the lungs"
a failing or deficiency; "that interpretation is an unfortunate defect of our lack of information" (同)shortcoming
(sports) a serve that is illegal (e.g., that lands outside the prescribed area); "he served too many double faults"
responsibility for a bad situation or event; "it was Johns fault"
(geology) a crack in the earths crust resulting from the displacement of one side with respect to the other; "they built it right over a geological fault"; "he studied the faulting of the earths crust" (同)faulting, geological fault, shift, fracture, break
(electronics) equipment failure attributable to some defect in a circuit (loose connection or insulation failure or short circuit etc.); "it took much longer to find the fault than to fix it"

PrepTutorEJDIC

〈U〉(体・心・性格か)『弱いこと』《+of+名》 / 〈C〉『弱点』,短所 / 〈C〉(特に良くないものに対する)特別な好み,愛好《+for+名》
〈C〉『欠点』,弱点,欠陥 / 〈U〉〈C〉《古》不足,欠乏 / (国・党・主義などから)離脱する,裏切って出る《+『from』+『名』》;離反して(…に)転向する《+『to』+『名』》
〈C〉『欠点』,短所(defect) / 〈C〉『誤り』,落ち度 / 〈U〉(過失の)『責任』,罪 / 〈C〉断層(地層の食い違い) / 〈C〉フォールト(テニスなどでサーブの失敗) / …‘の'あら捜しをする / あやまちを犯す / 断層を生じる

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/09/09 15:00:32」(JST)

wiki en

[Wiki en表示]

"Asthenia" redirects here. The tortrix moth genus is considered a junior synonym of Epinotia.

Weakness or asthenia is a symptom of a number of different conditions.^[1] The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, including muscular dystrophy and inflammatory myopathy. It occurs in neuromuscular junction disorders, such as myasthenia gravis.

Differential diagnosis

True vs. perceived weakness

True weakness (or neuromuscular) describes a condition where the force exerted by the muscles is less than would be expected, for example muscular dystrophy.
Perceived weakness (or non-neuromuscular) describes a condition where a person feels more effort than normal is required to exert a given amount of force but actual muscle strength is normal, for example chronic fatigue syndrome.^[2]

In some conditions, such as myasthenia gravis, muscle strength is normal when resting, but true weakness occurs after the muscle has been subjected to exercise. This is also true for some cases of chronic fatigue syndrome, where objective post-exertion muscle weakness with delayed recovery time has been measured and is a feature of some of the published definitions.^[3]^[4]^[5]^[6]^[7]^[8]

Asthenia vs. myasthenia

Asthenia (Greek: ἀσθένεια, lit. lack of strength but also disease) is a medical term referring to a condition in which the body lacks or has lost strength either as a whole or in any of its parts. It denotes symptoms of physical weakness and loss of strength. General asthenia occurs in many chronic wasting diseases (such as tuberculosis and cancer), sleep disorders or chronic disorders of the heart, lungs or kidneys, and is probably most marked in diseases of the adrenal gland. Asthenia may be limited to certain organs or systems of organs, as in asthenopia, characterized by ready fatiguability. Asthenia is also a side effect of some medications and treatments, such as Ritonavir (a protease inhibitor used in HIV treatment), vaccines such as the HPV vaccine Gardasil^[9] and fentanyl patches (an opioid used to treat pain).

Differentiating psychogenic (perceived) asthenia and true asthenia from myasthenia is often difficult, and in time apparent psychogenic asthenia accompanying many chronic disorders is seen to progress into a primary weakness.

Myasthenia (my- from Greek μυο meaning "muscle" + -asthenia ἀσθένεια meaning "weakness"), or simply muscle weakness, is a lack of muscle strength. The causes are many and can be divided into conditions that have either true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, including muscular dystrophy and inflammatory myopathy. It occurs in neuromuscular diseases, such as myasthenia gravis.

Types

Muscle fatigue can be central, neuromuscular, or peripheral muscular. Central muscle fatigue manifests as an overall sense of energy deprivation, and peripheral muscle weakness manifests as a local, muscle-specific inability to do work.^[10]^[11] Neuromuscular fatijue can be either central or peripheral.

Central fatigue

The central fatigue is generally described in terms of a reduction in the neural drive or nerve-based motor command to working muscles that results in a decline in the force output.^[12]^[13]^[14] It has been suggested that the reduced neural drive during exercise may be a protective mechanism to prevent organ failure if the work was continued at the same intensity.^[15]^[16] The exact mechanisms of central fatigue are unknown, though there has been a great deal of interest in the role of serotonergic pathways.^[17]^[18]^[19]

Neuromuscular fatigue

Nerves control the contraction of muscles by determining the number, sequence, and force of muscular contraction. When a nerve experiences synaptic fatigue it becomes unable to stimulate the muscle that it innervates. Most movements require a force far below what a muscle could potentially generate, and barring pathology, neuromuscular fatigue is seldom an issue.

For extremely powerful contractions that are close to the upper limit of a muscle's ability to generate force, neuromuscular fatigue can become a limiting factor in untrained individuals. In novice strength trainers, the muscle's ability to generate force is most strongly limited by nerve’s ability to sustain a high-frequency signal. After an extended period of maximum contraction, the nerve’s signal reduces in frequency and the force generated by the contraction diminishes. There is no sensation of pain or discomfort, the muscle appears to simply ‘stop listening’ and gradually cease to move, often lengthening. As there is insufficient stress on the muscles and tendons, there will often be no delayed onset muscle soreness following the workout. Part of the process of strength training is increasing the nerve's ability to generate sustained, high frequency signals which allow a muscle to contract with their greatest force. It is this "neural training" that causes several weeks worth of rapid gains in strength, which level off once the nerve is generating maximum contractions and the muscle reaches its physiological limit. Past this point, training effects increase muscular strength through myofibrillar or sarcoplasmic hypertrophy and metabolic fatigue becomes the factor limiting contractile force.

Peripheral muscle fatigue

Peripheral muscle fatigue during physical work is considered^{[by whom?]} an inability for the body to supply sufficient energy or other metabolites to the contracting muscles to meet the increased energy demand. This is the most common case of physical fatigue—affecting a national^[where?] average of 72% of adults in the work force in 2002. This causes contractile dysfunction that manifests in the eventual reduction or lack of ability of a single muscle or local group of muscles to do work. The insufficiency of energy, i.e. sub-optimal aerobic metabolism, generally results in the accumulation of lactic acid and other acidic anaerobic metabolic by-products in the muscle, causing the stereotypical burning sensation of local muscle fatigue, though recent studies have indicated otherwise, actually finding that lactic acid is a source of energy.^[20]

The fundamental difference between the peripheral and central theories of muscle fatigue is that the peripheral model of muscle fatigue assumes failure at one or more sites in the chain that initiates muscle contraction. Peripheral regulation therefore depends on the localized metabolic chemical conditions of the local muscle affected, whereas the central model of muscle fatigue is an integrated mechanism that works to preserve the integrity of the system by initiating muscle fatigue through muscle derecruitment, based on collective feedback from the periphery, before cellular or organ failure occurs. Therefore, the feedback that is read by this central regulator could include chemical and mechanical as well as cognitive cues. The significance of each of these factors will depend on the nature of the fatigue-inducing work that is being performed.

Though not universally used, "metabolic fatigue" is a common alternative term for peripheral muscle weakness, because of the reduction in contractile force due to the direct or indirect effects of the reduction of substrates or accumulation of metabolites within the myocytes. This can occur through a simple lack of energy to fuel contraction, or through interference with the ability of Ca²⁺ to stimulate actin and myosin to contract.

Lactic acid hypothesis

It was once believed that lactic acid build-up was the cause of muscle fatigue.^[21] The assumption was lactic acid had a "pickling" effect on muscles, inhibiting their ability to contract. The impact of lactic acid on performance is now uncertain, it may assist or hinder muscle fatigue.

Produced as a by-product of fermentation, lactic acid can increase intracellular acidity of muscles. This can lower the sensitivity of contractile apparatus to calcium ions (Ca²⁺) but also has the effect of increasing cytoplasmic Ca²⁺ concentration through an inhibition of the chemical pump that actively transports calcium out of the cell. This counters inhibiting effects of potassium ions (K⁺) on muscular action potentials. Lactic acid also has a negating effect on the chloride ions in the muscles, reducing their inhibition of contraction and leaving K⁺ as the only restricting influence on muscle contractions, though the effects of potassium are much less than if there were no lactic acid to remove the chloride ions. Ultimately, it is uncertain if lactic acid reduces fatigue through increased intracellular calcium or increases fatigue through reduced sensitivity of contractile proteins to Ca²⁺.

Pathophysiology

Muscle cells work by detecting a flow of electrical impulses from the brain, which signals them to contract through the release of calcium by the sarcoplasmic reticulum. Fatigue (reduced ability to generate force) may occur due to the nerve, or within the muscle cells themselves. New research from scientists at Columbia University suggests that muscle fatigue is caused by calcium leaking out of the muscle cell. This makes less calcium available for the muscle cell. In addition, the Columbia researchers propose that an enzyme activated by this released calcium eats away at muscle fibers.^[22]

Substrates within the muscle generally serve to power muscular contractions. They include molecules such as adenosine triphosphate (ATP), glycogen and creatine phosphate. ATP binds to the myosin head and causes the ‘ratchetting’ that results in contraction according to the sliding filament model. Creatine phosphate stores energy so ATP can be rapidly regenerated within the muscle cells from adenosine diphosphate (ADP) and inorganic phosphate ions, allowing for sustained powerful contractions that last between 5–7 seconds. Glycogen is the intramuscular storage form of glucose, used to generate energy quickly once intramuscular creatine stores are exhausted, producing lactic acid as a metabolic byproduct. Contrary to common belief, lactic acid accumulation doesn't actually cause the burning sensation we feel when we exhaust our oxygen and oxidative metabolism, but in actuality, lactic acid in presence of oxygen recycles to produce pyruvate in the liver, which is known as the Cori cycle.

Substrates produce metabolic fatigue by being depleted during exercise, resulting in a lack of intracellular energy sources to fuel contractions. In essence, the muscle stops contracting because it lacks the energy to do so.

References

^ Marx, John (2010). Rosen's Emergency Medicine: Concepts and Clinical Practice (7th ed.). Philadelphia, PA: Mosby/Elsevier. p. Chapter 11. ISBN 978-0-323-05472-0.
^ Ropper, Allan H.; Samuels, Martin A. (2009). Adams and Victor's Principles of Neurology, Ninth Edition. McGraw-Hill. ISBN 978-0071499927.
^ Paul L, Wood L, Behan WM, Maclaren WM (January 1999). "Demonstration of delayed recovery from fatiguing exercise in chronic fatigue syndrome". Eur. J. Neurol. 6 (1): 63–9. doi:10.1046/j.1468-1331.1999.610063.x. PMID 10209352.
^ McCully KK, Natelson BH (November 1999). "Impaired oxygen delivery to muscle in chronic fatigue syndrome". Clin. Sci. 97 (5): 603–8; discussion 611–3. doi:10.1042/CS19980372. PMID 10545311.
^ De Becker P, Roeykens J, Reynders M, McGregor N, De Meirleir K (November 2000). "Exercise capacity in chronic fatigue syndrome". Arch. Intern. Med. 160 (21): 3270–7. doi:10.1001/archinte.160.21.3270. PMID 11088089.
^ De Becker P, McGregor N, De Meirleir K (September 2001). "A definition-based analysis of symptoms in a large cohort of patients with chronic fatigue syndrome". J. Intern. Med. 250 (3): 234–40. doi:10.1046/j.1365-2796.2001.00890.x. PMID 11555128.
^ Carruthers, Bruce M.; Jain, Anil Kumar; De Meirleir, Kenny L.; Peterson, Daniel L.; Klimas, Nancy G.; et al. (2003). "Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Clinical Working Case Definition, Diagnostic and Treatment Protocols". Journal of Chronic Fatigue Syndrome. 11 (1): 7–115. doi:10.1300/J092v11n01_02. ISBN 0-7890-2207-9. ISSN 1057-3321.
^ Jammes Y, Steinberg JG, Mambrini O, Brégeon F, Delliaux S (March 2005). "Chronic fatigue syndrome: assessment of increased oxidative stress and altered muscle excitability in response to incremental exercise". J. Intern. Med. 257 (3): 299–310. doi:10.1111/j.1365-2796.2005.01452.x. PMID 15715687.
^ Loris McVittie (June 12, 2008). "Information from CDC and FDA on the Safety of Gardasil Vaccine". Office of Vaccines Research and Review at the US FDA. Archived from the original on October 4, 2008. Retrieved 2008-07-21. We have approved your supplement to your biologics license application (BLA) for Human Papillomavirus Quadrivalent (Types 6, 11, 16 and 18) Vaccine, Recombinant (GARDASIL), to include arthralgia, myalgia, asthenia, fatigue, and malaise in the Adverse Reactions section of the package insert to reflect reports received during post-marketing surveillance, to include corresponding changes to the patient package insert, and to include additional minor editorial changes to the package insert.
^ Gandevia SC, Enoka RM, McComas AJ, Stuart DG, Thomas CK (1995). "Neurobiology of muscle fatigue. Advances and issues". Adv. Exp. Med. Biol. 384: 515–25. doi:10.1007/978-1-4899-1016-5_39. PMID 8585476.
^ Kent-Braun JA (1999). "Central and peripheral contributions to muscle fatigue in humans during sustained maximal effort". European journal of applied physiology and occupational physiology. 80 (1): 57–63. doi:10.1007/s004210050558. PMID 10367724.
^ Gandevia SC (2001). "Spinal and supraspinal factors in human muscle fatigue". Physiol. Rev. 81 (4): 1725–89. PMID 11581501.
^ Kay D, Marino FE, Cannon J, St Clair Gibson A, Lambert MI, Noakes TD (2001). "Evidence for neuromuscular fatigue during high-intensity cycling in warm, humid conditions". Eur. J. Appl. Physiol. 84 (1–2): 115–21. doi:10.1007/s004210000340. PMID 11394239.
^ Vandewalle H, Maton B, Le Bozec S, Guerenbourg G (1991). "An electromyographic study of an all-out exercise on a cycle ergometer". Archives internationales de physiologie, de biochimie et de biophysique. 99 (1): 89–93. doi:10.3109/13813459109145909. PMID 1713492.
^ Bigland-Ritchie B, Woods JJ (1984). "Changes in muscle contractile properties and neural control during human muscular fatigue". Muscle Nerve. 7 (9): 691–9. doi:10.1002/mus.880070902. PMID 6100456.
^ Noakes TD (2000). "Physiological models to understand exercise fatigue and the adaptations that predict or enhance athletic performance". Scandinavian journal of medicine & science in sports. 10 (3): 123–45. doi:10.1034/j.1600-0838.2000.010003123.x. PMID 10843507.
^ Davis JM (1995). "Carbohydrates, branched-chain amino acids, and endurance: the central fatigue hypothesis". Int J Sport Nutr. 5 (Suppl): S29–38. PMID 7550256.
^ Newsholme, E. A., Acworth, I. N., & Blomstrand, E. 1987, 'Amino acids, brain neurotransmitters and a functional link between muscle and brain that is important in sustained exercise', in G Benzi (ed.), Advances in Myochemistry, Libbey Eurotext, London, pp. 127-133.
^ Newsholme EA, Blomstrand E (1995). "Tryptophan, 5-hydroxytryptamine and a possible explanation for central fatigue". Adv. Exp. Med. Biol. 384: 315–20. doi:10.1007/978-1-4899-1016-5_25. PMID 8585461.
^ R. Robergs; F. Ghiasvand; D. Parker (2004). "Biochemistry of exercise-induced metabolic acidosis". Am J Physiol Regul Integr Comp Physiol. 287 (3): R502–16. doi:10.1152/ajpregu.00114.2004. PMID 15308499.
^ Sahlin K (1986). "Muscle fatigue and lactic acid accumulation". Acta Physiol Scand Suppl. 556: 83–91. PMID 3471061.
^ Kolata, Gina (February 12, 2008). "Finding May Solve Riddle of Fatigue in Muscles". The New York Times.

External links

Jun Mapili's Muscle Weakness Coding Checklist
AllRefer
Unexplained Muscle Weakness - Information About McArdle's Disease

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. 筋力低下患者へのアプローチ approach to the patient with muscle weakness
2. 救急外来における急性の筋力低下を訴える成人の評価 evaluation of the adult with acute weakness in the emergency department
3. 脱力がみられる小児の病因および評価 etiology and evaluation of the child with weakness
4. 筋力低下の評価における筋力検査 muscle examination in the evaluation of weakness
5. 筋緊張低下や脱力感のある乳児へのアプローチ approach to the infant with hypotonia and weakness

English Journal

Biomechanical behaviour of cancellous bone on patellofemoral arthroplasty with Journey prosthesis: a finite element study.

Castro AP1, Completo A, Simões JA, Flores P.
Computer methods in biomechanics and biomedical engineering.Comput Methods Biomech Biomed Engin.2015 Aug;18(10):1090-1098. Epub 2014 Jan 27.
Isolated patellofemoral (PF) arthritis of the knee is a common cause of anterior knee pain and disability. Patellofemoral arthroplasty (PFA) is a bone conserving solution for patients with PF degeneration. Failure mechanisms of PFA include growing tibiofemoral arthritis and loosening of components.
PMID 24460149

Quadriceps muscle weakness and atrophy are associated with a differential epigenetic profile in advanced COPD.

Puig-Vilanova E1, Martínez-Llorens J1, Ausin P1, Roca J2, Gea J1, Barreiro E1.
Clinical science (London, England : 1979).Clin Sci (Lond).2015 Jun 1;128(12):905-21. doi: 10.1042/CS20140428.
Epigenetic mechanisms regulate muscle mass and function in models of muscle dysfunction and atrophy. We assessed whether quadriceps muscle weakness and atrophy are associated with a differential expression profile of epigenetic events in patients with advanced COPD (chronic obstructive pulmonary dis
PMID 25628226

Immune-mediated necrotizing myopathy, associated with antibodies to signal recognition particle, together with lupus nephritis: case presentation and management.

O'Grady J1, Harty L1, Mayer N2, Critcher V2, Ryan J2.
Journal of clinical medicine research.J Clin Med Res.2015 Jun;7(6):490-4. doi: 10.14740/jocmr2133w. Epub 2015 Apr 8.
A male patient with limb weakness, myalgia and edema was subsequently found to have an immune-mediated necrotizing myopathy (IMNM) on biopsy. Targeted myopathic antibody analysis revealed antibodies to signal recognition particle (SRP). Anti-SRP-associated necrotizing myopathy was diagnosed. This ca
PMID 25883715

Clinical course and treatment of anti-HMGCR antibody-associated necrotizing autoimmune myopathy.

Ramanathan S1, Langguth D1, Hardy TA1, Garg N1, Bundell C1, Rojana-Udomsart A1, Dale RC1, Robertson T1, Mammen AL1, Reddel SW1.
Neurology® neuroimmunology & neuroinflammation.Neurol Neuroimmunol Neuroinflamm.2015 Apr 2;2(3):e96. doi: 10.1212/NXI.0000000000000096. eCollection 2015.
OBJECTIVE: We examined a cohort of Australian patients with statin exposure who developed a necrotizing autoimmune myopathy (NAM) associated with a novel autoantibody against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) and describe the clinical and therapeutic challenges of managing thes
PMID 25866831

Japanese Journal

人工呼吸管理中の呼吸理学療法 (特集 ARDS)

及川真人,花田匡利,神津玲 [他]
呼吸器内科 = Respiratory medicine 28(2), 118-125, 2015-08
NAID 40020566306

筋力低下に対する軌道上ヒト対象研究 (特集宇宙医学とリハビリテーション)

志波直人
総合リハビリテーション 43(7), 621-626, 2015-07
NAID 40020513934

医療バクテリア社会の弱点を突く

ジンマー C.
日経サイエンス 45(6), 64-69, 2015-06
NAID 40020457048

UPKIパス:FCFキャンパスカードと証明書ストアサーバとの連携によるクライアント証明書活用システムの改良

中村素典,西村健,山地一禎
情報処理学会研究報告. IOT, [インターネットと運用技術] 2015-IOT-29(30), 1-8, 2015-05-14
認証処理におけるパスワードの脆弱性が強く認識されるようになり,より強力な認証手段が求められている.そのような認証手段の 1 つとして PKI に基づくクライアント証明書が有効であることが広く知られているが,クライアント証明書の利用者側の取り扱いが煩雑であることから十分な普及には至っていない.一方,大学では,FeliCa カードをベースとした FCF キャンパスカードが学生証や教職員証として広く普及 …
NAID 110009900815

Related Pictures

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★リンクテーブル★

リンク元	「マクログロブリン血症」「100Cases 14」「100Cases 52」「defect」「欠点」
拡張検索	「muscle weakness」

「マクログロブリン血症」

Weakness
Classification and external resources
Specialty	Neurology
ICD-10	R53
ICD-9-CM	728.87 (728.9 before 10/01/03); alternatively, 780.79
DiseasesDB	22832
MedlinePlus	003174
MeSH	D018908
	[edit on Wikidata]

　　[★]

英: macroglobulinemia
同: ワルデンシュトレーム・マクログロブリン血症 Waldenström macroglobulinemia Waldenstrom macroglobulinemia WM、原発性マクログロブリン血症 primary macroglobulinemia、マクログロブリネミア
関: 単クローン性免疫グロブリン血症。高γグロブリン血症

first aid step1 2006 p.304

Bence Jones protein：multiple myeloma(κ/λ Ig light chains in urine), Waldenström's macroglobulinemia (IgM)
monoclonal antibody spike：multiple myeloma (M protein = IgG/IgA), MGUS (monoclonal gammopathy of undetermined significance), Waldenström's macroglobulinemia (M protein = IgM)

	ALL	ALL(バーキット型)	悪性リンパ腫、CLL	マクログロブリン血症	骨髄腫
分化段階	前駆B細胞	未熟B細胞	成熟B細胞		形質細胞
細胞表面免疫グロブリン	－	＋	＋	±	－
免疫グロブリン分泌	－	－	－	＋	＋

WCH.2669

pathologic feature	MCL	B-cell CLL	FL	MZL	WM	MM
paraprotein	None	Small IgG or IgM	Usually none	Small IgM	IgM (large)	IgA, IgG
morphology	Centrocyte-like; small-to-medium-sized lymphocytes	Small lymphocyte with clumped chromatin	Follicle center cells (follicular pattern)	Monocytoid B cells, heterogeneous	Plasmacytoid lymphocytes and plasma cells	Plasma cells
surface Ig	＋	＋	＋	＋	＋	＋
cytoplasmic Ig	－	－	－	－	＋＋	＋＋＋
CD19	＋	＋	＋	＋	＋	＋
CD20	＋＋	＋	＋＋	＋	＋	15% CD20+
CD23	－	＋	±	－	－	－
CD22	＋	－	－	＋	＋	－
CD38	－	±	－	－	＋	＋＋
CD138	－	－	－	－	＋	＋＋
CD5	＋	＋	－	－	Usually CD5-	－
CDlO	±	－	＋	－	－	－
cytogenetic aberrations	t(11;14)(q13;q32), cyclin Dl+	13q-, 6q-, +12, 11q23-	t(14;18)(q32; q21), bcl-2+	t(11;18)(q21; q21), +3	6q-	t(4;14)(p16.3;q32),t(11;14)(q13;q32),t(14;16)(q32;q23), other +14q32,13q-, aneuploidy
somatic hypermutation	？	＋, 50%; -, 50%	＋＋	＋＋	＋＋＋	＋＋＋
bone marrow involvement (%)	25	～100	85	50	＞90	100
bone lytic lesions	No	No	No	No	5%	70%

概念

病因

疫学

60-70歳

症状

also see WCH.2671

血管粘稠度上昇　＋　病的蛋白質による血管内皮損傷：[眼症状] ソーセージ様の網膜血管のうっ滞・拡張、網膜出血、綿花様白斑、網膜静脈閉塞症、滲出性網膜剥離
リンパ球の浸潤による症状：weakness, 体重減少、骨痛、肝脾腫、リンパ節腫

診断

鑑別診断

慢性リンパ性白血球、骨髄腫(IgM myeloma: bone lesion, CD138+ plamac cell infiltration)、kymphocytic lymphoma

myelomaとの共通点と異差

HIM.706

共通点：

症状：weakness、疲労、反復感染

WMに特徴的：

lymphadenopathy、hepatosplenomegalyがあること。bone lesionや高カルシウム血症は起こさない。(大きさの違いから？)IgMは殆ど人から排泄されないし、-20%の人しかBJPを排泄しないから、腎病変は一般的でない。

WMでより一般的：

鼻出血、視力障害(visual disturbance)、神経症状(めまい、頭痛、一時的な麻痺)

QB.G-269 WCH.2671

	多発性骨髄腫	マクログロブリン血症
	多発性骨髄腫	ワルデンシュトレーム・マクログロブリン血症
	MM	WM
病変のfocus	主に骨髄	主にリンパ組織	集積することで出現する症状が異なる
腫瘍細胞	形質細胞	やや幼弱なB細胞
赤血球連戦形成	＋	＋＋＋	IgMのせい
出血傾向	少	多	IgMのせい
肝脾腫、リンパ節腫脹	少	多
骨破壊	70％	5％
過粘稠度症候群	少	多	IgMのせい
M蛋白	IgG,IgA,IgD,IgE,BJP	IgM
腎障害	多	少	IgMは糸球体濾過されにくい
眼底変化	少	多	IgMのせい
年齢	40-60歳代	40-70歳代

検査

血算

赤沈：高度促進
貧血
白血球：増多(リンパ球様細胞の増多)
血小板：減少　　←　　MMより頻度は低い、はず。一般的でなく、病気の末期まで出現しない(WCH.2671)。患者のほとんどが正常範囲内で、10万/ul以下であるのはほんの9%の患者であり、本疾患における血小板減少症の有病率は22%と報告されている(WCH.2671)。

血液生化学

尿検査

尿タンパク：陽性(10%でBJPが見られる。)

治療

アルキル化剤(メルファラン、シクロホスファミド)　＋　プレドニゾロン

予後

USMLE

Q book p.246 41

「100Cases 14」

　　[★]

☆case14　複視

■glossary

diplopia n. 複視

筋力低下、筋無力、筋脱力 muscle weakness, muscular weakness

sunken

vt. sinkのpp.

adj.

沈没した、沈んだ、水中の

沈下した、一段低いところにある

落ち込んだ、くぼんだ

3,4-ジアミノピリジン 3,4-diaminopyridine 3,4-DAP K+チャネル阻害薬;ランバート・イートン筋無力症治療

■症例

43-year-old woman

cheif complaint: diplopia

present history: diplopia and holding her head up ; more marked in the evenings, for the last 3 months. difficulty of chewing. voice has become quieter. weight loss (3kg / 6months). non-smoker. drinks about 15 units/week. no regular medication.

past history: no significant previous medical illnesses.

family history: lives with her husband and three children.

・診察 examination

looks well.

organ systems: normal; cardiovascular, respiratory, and abdominal systems.

muscle power; grossly normal. decrease after testing a movement repetitively.

motor function: normal; tone, coordination, reflexes and sensation.

bilateral ptosis. exacerbated by prolonged upward gaze

eye: normal; pupillary reflexes, eye movements, and funduscopy

■答え

diagnosis: myathenia gravis

differential diagnoses:

CASES

上位and/or下位運動ニューロン motor neurone disease　運動ニューロン疾患：線維束性攣縮。進行例では筋力低下

筋 muscular dystrophy　筋ジストロフィー：ある種の筋肉が選択的に筋力低下する。家族歴がある。

筋 dystrophia myotonica　強直性筋ジストロフィー：咬筋、側頭筋、胸鎖乳突筋の筋萎縮、四肢遠位端の筋萎縮。顔貌が特徴的(前頭部脱毛、無表情、窪んだ頬)。家族歴ある。筋電図が診断に有用(急降下爆撃音)。

筋 polymyositis　多発筋炎：普通は皮疹と関節痛が出現。CKが上昇。筋生検が診断に有用

筋 myopathy　ミオパチー：甲状腺中毒性ミオパチー、甲状腺機能低下症によるミオパチー、クッシング症候群によるミオパチー、アルコール性のミオパチー

神経筋接合部？ non-metastatic associations of malignancy (paraneoplastic syndrome(傍腫瘍性症候群 = 腫瘍随伴症候群)のこと)：胸腺腫の症例の10%に重症筋無力症がみられる。ランバート・イートン筋無力症症候群は小細胞癌と関連がある。

HIM.2674

神経筋接合部 congenital myasthenia syndrome　先天性筋無力症症候群
神経筋接合部 drug-induced myasthenia　薬剤性筋無力症

Treatment with penicillamine (used for scleroderma or rheumatoid arthritis) may result in true autoimmune MG, but the weakness is usually mild, and recovery occurs within weeks or months after discontinuing its use.

　重症筋無力症の誘発：ペニシラミン(強皮症や関節リウマチの治療に用いられる)。

　　mildだし、薬剤の中断で改善する。

Aminoglycoside antibiotics or procainamide can cause exacerbation of weakness in myasthenic patients; very large doses can cause neuromuscular weakness in normal individuals.

　重症筋無力症の悪化：アミドグリコシド系抗菌薬、プロカインアミド

　　MGの患者の筋脱力が悪化する。

神経筋接合部 Lambert-Eaton myasthenic syndrome　ランバート・イートン筋無力症症候群：全身の筋肉が冒されるが、特に下肢の近位筋が冒される。MGと同じように～70%の患者で脳神経所見(眼瞼下垂、複視など)が認められる。MGと違うのは(1)反射が消失・減弱すること、(2)自律神経系の変化(口渇、勃起不全)を生じる、(3)神経刺激検査で漸増(waxing)が見られることである。病因は神経筋接合部のP/Q type calcium channelsに対する抗体の出現であり、85%の患者で見いだされる。治療はMGのように血漿交換や免疫抑制薬が使われる。3,4-DAPやpyridostigmineは症状に対する治療のために用いる。前者は運動神経の終末部でカルシウムチャネルをブロックし活動電位を延長させる。後者はアセチルコリンエステラーゼを阻害してシナプスにおける神経伝達物質の濃度を上げる。
精神疾患 neurasthenia　神経衰弱症：歴史的な用語。器質的な障害を伴わない筋無力症のような脱力を伴う症候群。患者は筋脱力や疲労を訴えてやってくる。筋肉の検査では器質的変化は認めないが"jerky release"あるいは"give-away weakness"が認められる。患者の主訴は反復動作による筋力低下よりむしろ疲労や感情鈍麻である。
内分泌疾患 hyperthyroidism　甲状腺機能亢進症：MGが疑われる患者にはthyroid function testをルーチンにやる。甲状腺機能異常は筋無力症の筋力低下を大きくすることがある。
神経筋接合部 botulism　ボツリヌス症：ボツリヌス毒素はシナプス前膜からの神経伝達物質の開口分泌を妨げる。症状はbulbar weakness (複視、構音障害、嚥下困難)。感覚障害はない。深部腱反射は初期には保たれている。進行すれば反射は見られなくなる。筋脱力は全身性。呼吸困難に陥ることがある。精神状態は正常。自律神経症状(麻痺性イレウス、便秘、urinary retention、瞳孔の散大、瞳孔の反応性低下、口渇)。確定診断は血清中の毒素の検出だけど、見つかることはまれ。神経伝導検査(nerve conduction studies)：compound muscle action potentials (CMAPs)の低下。高頻度の刺激で振幅が増加。治療：intubation for airway protection、呼吸補助、aggressive inpatient supportive care(e.g., nutrition, DVT prophylaxis)。馬の抗毒素を検査結果が帰ってくる前に投与する(？)。
上位運動ニューロン intracranial mass lesions　頭蓋内占拠病変：複視はintracranial mass lesionが外眼筋の神経を圧迫することにより生じる。
筋 progressive external ophthalmoplegia　進行性外眼筋麻痺：外眼筋の筋脱力を伴う。四肢の近位筋の筋力低下やそのほかの全身症状を伴うことがある。ミトコンドリアの異常を有する。

■参考文献

HIM = Harrison's Principles of Internal Medicine 17th Edition

CASES = 100 Cases in Clinical Medicine Second edition

IMD = 内科診断学第2版

「100Cases 52」

　　[★]

☆case52　全身性の筋力低下

■glossary

筋力低下、筋無力、筋脱力 muscle weakness, muscular weakness

denture 義歯

leg 脚、下肢

bruising 打撲、打撲傷

hair follicle 毛包

edentulous adj. 歯のない。歯を失った、全歯欠損の

■症例

82歳、男性

主訴：筋力低下と全身の倦怠感

現病歴：(以前から続く)筋痛。関節痛(特に肘、手首、膝)。3週間前に転倒して足を打ち、足に局所的な痛みがある。喫煙歴なし。飲酒歴なし。服用薬なし。

既往歴：12年前に心筋梗塞になった。心筋梗塞のβブロッカーを処方されていたが、過去6年間は処方薬を持ってなかった。20年前に胆嚢摘出術。

家族歴：

社会歴：労働者として働いていた。63歳に退職。2階のアパート(in a second-floor flat)に単身で住んでいる。妻は5年前に死亡。息子が一人おり、アイルランドに在住している。息子とは3年間会っていない。

・診察 examination

　肢帯周辺の筋肉に圧痛。肘、手首、肘周辺にも圧痛。口には異常がないが舌がかなり平坦化。歯はなく、義歯は無くしている。循環器系、呼吸器系、消化器系に異常なし。下肢では右脚の脛の前面に(superficial laceration)が認められる。このlacerationは血かにじみ出ており治癒していない。下肢には出血斑が認められる部分がある。腕や脚に打撲傷がみとめられる広い範囲がある。彼が言うには、この打撲傷はどんな外傷とも関係がないとのことである。

・検査 investigation

　ヘモグロビン低値、MCV 低値

■答え

(第一パラグラフ)

・dietary historyは重要なhistoryの中でも重要な部分。

・特に今回のような症例では重要。

・今回の症例では、多くの特徴が栄養的な問題を示している。

　・家族の援助が無く、5年間widowerである。

　・2階のアパートに独りで住んでいる。get outが困難である。

　・義歯を無くしており、食べるのが痕案である。

(第二パラグラフ)

・凝固異常に関係しうる点状出血の皮疹(petechial rash)が見られるが、血小板数は正常。

・毛包の周りに分布しているかどうかを見るために皮疹を注意深く診察することが重要。　←　どういうこと？

・多くの特徴が壊血病を示唆。

・体内のビタミンC ストックは2-3ヶ月。

・壊血病の特徴：皮疹、筋肉と関節の痛みと圧痛、創傷治癒の遅延、小球性貧血

・全歯欠損している患者では、古典的な壊血病の特徴である歯肉出血はないであろう(would not be present)

(第三パラグラフ)

・血清中のビタミンCレベルは正常な患者で広い範囲にあるので、測定は難しい。

・この患者ではアスコルビン酸を経口的にビタミンCレベルを正常に戻すことで(replacement)することで、2週間で症状が消えた。

・この状況で(in this situation)、他の栄養の欠乏を探したり、退院後再発しない状況を保証するための手続きをすることが重要である。

■KEY POINTS

・特に老人では栄養歴は臨床的評価に入れるべき

・ビタミン不足はあらゆる栄養吸収不良の問題がない場合、貧しい食生活を送っている患者に起こる。

「defect」

　　[★]

n.

欠損、欠損症、異常、欠陥、欠点

関: aberrant、aberrantly、aberration、abnormal、abnormality、abnormally、absence、agenesis、anomalous、anomalously、anomaly、defective、deficiency、deficient、deficit、deletion、deletional、deviant、drawback、extraordinarily、extraordinary、fault、flaw、inadequacy、miss、missing、morphological defect、shortcoming、unusual、unusually、weakness

「欠点」

　　[★]

英: drawback、defect、fault、inadequacy、weakness、shortcoming
関: 異常、過失、欠損、欠損症、弱点、脱力感、不適切、不適当、弱さ、弱いこと、欠陥、断層、脱力

「muscle weakness」

　　[★]

筋力低下
筋衰弱、筋脱力、

[Rosen10-1] Marx, John (2010). Rosen's Emergency Medicine: Concepts and Clinical Practice (7th ed.). Philadelphia, PA: Mosby/Elsevier. p. Chapter 11. ISBN 978-0-323-05472-0.

[2] Ropper, Allan H.; Samuels, Martin A. (2009). Adams and Victor's Principles of Neurology, Ninth Edition. McGraw-Hill. ISBN 978-0071499927.

[3] Paul L, Wood L, Behan WM, Maclaren WM (January 1999). "Demonstration of delayed recovery from fatiguing exercise in chronic fatigue syndrome". Eur. J. Neurol. 6 (1): 63–9. doi:10.1046/j.1468-1331.1999.610063.x. PMID 10209352.

[4] McCully KK, Natelson BH (November 1999). "Impaired oxygen delivery to muscle in chronic fatigue syndrome". Clin. Sci. 97 (5): 603–8; discussion 611–3. doi:10.1042/CS19980372. PMID 10545311.

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v t e Myopathy (M60–M63, 728.0–3,8)

Pain	Myalgia Fibromyalgia

Inflammation	Myositis Pyomyositis

Lytic	Muscle weakness Rhabdomyolysis Muscle atrophy/Amyotrophy

Other	Myositis ossificans Fibrodysplasia ossificans progressiva Compartment syndrome Anterior Diastasis of muscle Diastasis recti Muscle spasm

v t e Common cold

Viruses	Adenovirus Coronavirus Enterovirus Human metapneumovirus Human parainfluenza viruses Human respiratory syncytial virus Orthomyxoviruses Influenza A virus Influenza B virus Influenza C virus Rhinovirus

Symptoms	Cough Fatigue Fever Headache Loss of appetite Malaise Muscle aches Nasal congestion Rhinorrhea Sneezing Sore throat Weakness

Complications	Acute bronchitis Bronchiolitis Croup Otitis media Pharyngitis Pneumonia Sinusitis Strep throat

Drugs	Antiviral drugs Pleconaril (experimental)

匿名

検索

案内

weakness