adj.

間代性の(痙攣)、クローヌス性の

関: clonicity, clonus

WordNet

of or relating to abnormal neuromuscular activity characterized by rapidly alternating muscle contraction and relaxation; "clonic spasm"
convulsion characterized by alternating contractions and relaxations
of or relating to or characteristic of the atmosphere around a low pressure center; "cyclonic cloud pattern" (同)cyclonal, cyclonical
of or relating to or characteristic of a violent tropical storm; "cyclonic destruction" (同)cyclonal, cyclonical
of or relating to or characteristic of the atmosphere around a high pressure center

PrepTutorEJDIC

熱帯(温帯)性低気圧の;大たつまきの

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/05/13 12:42:26」(JST)

wiki en

[Wiki en表示]

For the film, see Parts: The Clonus Horror.

Look up clonus in Wiktionary, the free dictionary.

Clonus (from the Greek for "violent, confused motion") is a series of involuntary, rhythmic, muscular contractions and relaxations. Clonus is a sign of certain neurological conditions, particularly associated with upper motor neuron lesions involving descending motor pathways, and in many cases is, accompanied by spasticity (another form of hyperexcitability).^[1] Unlike small, spontaneous twitches known as fasciculations (usually caused by lower motor neuron pathology), clonus causes large motions that are usually initiated by a reflex. Studies have shown clonus beat frequency to range from 3–8 Hz on average, and may last a few seconds to several minutes depending on the patient’s condition.^[1]

Susceptibility [edit]

Clonus is typically seen in patients with stroke, multiple sclerosis, spinal cord damage and hepatic encephalopathy.^[2] Clonus has also appeared after ingesting potent serotonergic drugs, where ingestion strongly predicts imminent serotonin toxicity (serotonin syndrome).

Mechanism of clonus [edit]

Two proposed mechanisms [edit]

1) Self re-excitation of hyperactive stretch reflexes:^[1]

The self re-excitation of hyperactive stretch reflexes theory involves a repetitive contract- relax cycle in the affected muscle, which creates oscillatory movements in the affected limb.^[1] In order for self re-excitation to exist, both an increase in motor neuron excitability and nerve signal delay are required.^[1] Increased motor neuron excitability is likely accomplished by alterations to the net inhibition of neurons occurring as a result of injury to the CNS (stroke/ spinal cord injury).^[1] This lack of inhibition biases neurons to a net excitatory state, therefore increasing total signal conduction.^[1] Signaling delay is present due to an increased nerve conduction time.^[1] Long delays are primarily due to long reflex pathways, which are common in distal joints and muscles.^[1] This may therefore explain why clonus is typically found in distal structures like the ankle.^[1] Frequency of clonus beats have been found to be directly proportional to the length of the reflex pathway it is found in.^[1]

2) Presence of a central oscillator:^[1]

Clonus, with respect to the presence of a central oscillator, functions on the theory that when the central oscillator is turned on by a peripheral event, it will continue to rhythmically excite motor neurons; therefore creating clonus.^[1]

Although the two proposed mechanisms are very different in [theory] and are still debated, some studies now propose the potential of both mechanisms co-existing to create clonus.^[1] It is thought that the stretch reflex pathway may be stimulated first, and through its events, cause a decreased synaptic current threshold.^[1] This decreased synaptic current threshold would enhance motor neuron excitability as nerve impulses would be more readily conducted, and thus turn on this central oscillator.^[1] This theory is still being investigated.^[1]

Clonus and Spasticity [edit]

Clonus tends to co-exist with spasticity in many cases of stroke and spinal cord injury likely due to their common physiological origins. ^[1] Some consider clonus as simply an extended outcome of spasticity.^[1] Although closely linked, clonus is not seen in all patients with spasticity.^[1] Clonus tends to not be present with spasticity in patients with significantly increased muscle tone, as the muscles are constantly active and therefore not engaging in the characteristic on/off cycle of clonus.^[1] Clonus results due to an increased motor neuron excitation (decreased action potential threshold) and is common in muscles with long conduction delays, such as the long reflex tracts found in distal muscle groups.^[1] Clonus is commonly seen in the ankle but may exist in other distal structures as well.^[2]

Clonus locations [edit]

Ankle (medial gastrocnemius)^[2]
Patella (knee cap)
Triceps surae^[2] ^[3]
Biceps brachii^[3]

Clonus is most commonly found at the ankle, specifically with a dorsiflexion/plantarflexion movement (up and down).^[2] Some case studies have also reported clonus in the finger, toe, and laterally in the ankle (as opposed to the typical up and down motion).^[4] ^[3]

Eliciting Clonus (Testing) [edit]

Clonus at the ankle is tested by rapidly flexing the foot into dorsiflexion (upward), inducing a stretch to the gastrocnemius muscle.^[1] Subsequent beating of the foot will result, however only a sustained clonus (5 beats or more) is considered abnormal. Clonus can also be tested in the knees by rapidly pushing the patella (knee cap), towards the toes.

Video Demonstrations [edit]

[1] Clonus at the ankle

[2] Clonus at the knee (patella)

External links [edit]

Dr. Gilman on Serotonin Toxicity

References [edit]

^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j ^k ^l ^m ⁿ ^o ^p ^q ^r ^s ^t ^u ^v ^w Hilder, Joseph M.; Zev W. Rymer (September). "A Stimulation Study of Reflex Instability in Spasticity: Origins of Clonus". IEEE Transactions on Rehabilitation Engineering 7 (3): 327–340. Retrieved May 6, 2012.
^ ^a ^b ^c ^d ^e ">Douglas, Wallace M.; Bruce H Ross, Christine K. Thomas (Aug 25). "Motor unit behaviour during clonus". Journal of Applied Physiology 99 (6): 2166–2172. Retrieved May 6, 2012.
^ ^a ^b ^c Weisenburg, Theodore H (November). "Triceps, Biceps and Finger Clonus". Journal of Nervous and Mental Diseases 30 (11): 681–683. Retrieved May 6, 2012.
^ Mitchell, John K. (May). "Two unusual forms of clonus: toe clonus and lateral ankle clonus". Journal of Nervous and Mental Disease 29 (5): 260–261. Retrieved May 6, 2012.

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. ミオクローヌスの分類および評価 classification and evaluation of myoclonus
2. 成人における初回痙攣発作の評価 evaluation of the first seizure in adults
3. 成人におけるてんかん重積状態 status epilepticus in adults
4. 小児痙攣およびてんかんの分類、病因、および臨床的特徴の概要 overview of the classification etiology and clinical features of pediatric seizures and epilepsy
5. 統合失調症に対するクロザピン投与のガイドライン guidelines for prescribing clozapine in schizophrenia

English Journal

Increased cerebral oxygenation precedes generalized tonic clonic seizures.

Moseley BD1, Britton JW2, So E3.
Epilepsy research.Epilepsy Res.2014 Nov;108(9):1671-4. doi: 10.1016/j.eplepsyres.2014.09.017. Epub 2014 Sep 22.
Based on previous fMRI and SPECT studies, it has been suggested seizures may be preceded by increased cerebral blood flow. Recently, we demonstrated transcutaneous regional cerebral oxygen saturation (rSO2) sensors are feasible for use in patients undergoing video EEG monitoring. We reanalyzed our d
PMID 25277885

Medication side effects among people with epilepsy taking phenobarbital in Zambia.

Elafros MA1, Bui E2, Birbeck GL3.
Epilepsy research.Epilepsy Res.2014 Nov;108(9):1680-4. doi: 10.1016/j.eplepsyres.2014.08.005. Epub 2014 Aug 30.
Phenobarbital remains one of the most widely used antiepileptic drugs worldwide, yet there are limited data regarding side effects associated with its use in routine clinical care settings in low-income countries. Available data suggests that phenobarbital is as effective as other first-line drugs f
PMID 25219354

Epilepsy in Menkes disease: An electroclinical long-term study of 28 patients.

Verrotti A1, Cusmai R2, Darra F3, Martelli P4, Accorsi P4, Bergamo S5, Bevivino E2, Coppola G6, Freri E7, Grosso S8, Matricardi S7, Parisi P9, Sartori S5, Spalice A10, Specchio N2, Carelli A11, Zini D3, Bernardina BD3, Giordano L4.
Epilepsy research.Epilepsy Res.2014 Nov;108(9):1597-603. doi: 10.1016/j.eplepsyres.2014.08.006. Epub 2014 Aug 30.
BACKGROUND: Epilepsy is a frequent and severe feature of Menkes disease (MD) but only few studies described the long-term evolution of these children. We report a series of 28 epileptic MD patients, with clinical characteristics, EEG abnormalities, brain malformations and long-term outcome.METHODS:
PMID 25218893

Japanese Journal

小児欠神てんかんと初期診断した25例の臨床経過と脳波所見

中村紀子,安元佐和,藤田貴子 [他]
福岡大学医学紀要 = Medical bulletin of Fukuoka University 40(3・4), 105-110, 2013-12
NAID 40019946792

小児欠神てんかんと初期診断した25例の臨床経過と脳波所見

中村紀子,安元佐和,藤田貴子,友納優子,井原由紀子,二之宮信也,井手口博,井上貴仁,廣瀬伸一
福岡大学医学紀要 40(3/4), 105-110, 2013-12
NAID 110009658436

ケトン食療法長期継続中のDravet症候群の検討

坂内優子,小国弘量,小国美也子 [他],伊藤康,大澤眞木子,SAKAUCHI Masako,OGUNI Hirokazu,OGUNI Miyako,ITO Yasushi,OSAWA Makiko
東京女子医科大学雑誌 83(E1), E58-E64, 2013-01-31
【目的】ケトン食療法を長期維持できている症例を検討し、効果や問題点などを明らかにする。,【方法】当科通院加療中のDravet症候群のうち、1年以上ケトン食療法を継続中の患児についてケトン食治療の実際、治療効果、副作用などについて調査した。,【対象】男児2例、女児2例、調査時年齢5歳0ヶ月〜11歳10ヶ月であった。てんかん発症月齢0歳3ヶ月〜0歳7ヶ月。臨床病型は典型的Dravet症候群 3例、ミオ …
NAID 110009559426

Related Pictures

★リンクテーブル★

リンク元	「100Cases 33」「100Cases 34」「clonus」「間代性」
拡張検索	「myoclonic epilepsy and ragged red fibers」「progressive myoclonic epilepsy」「myoclonic epilepsy」「myoclonic cerebellar dyssynergia」「opsoclonic meningoencephalitis」

「100Cases 33」

　　[★]

☆case33　頭痛と混乱

■glossary

accompany

vt.

(人)と同行する、(人)に随行する。(もの)に付随する。～と同時に起こる。～に加える(添える、同封する)(with)

slurred n. 不明瞭

強直間代痙攣 tonic-clonic convulsion

　意識消失とともに全身の随意筋に強直痙攣が生じ(強直痙攣期tonic convulsion)、次いで全身の筋の強直と弛緩とが律動的に繰り返される時期(間代痙攣期clonic convulsion)を経て、発作後もうろう状態を呈する一連の発作。

■症例

28歳、女性　黒人　南アフリカ　手術室看護師　ロンドン住在

主訴：頭痛と混乱

現病歴：過去3週間で頭痛が続いており、ひどくなってきた。現在も頭痛が持続しており、頭全体が痛い。友人曰く「過去六ヶ月で体重が10kg減っていて、最近、混乱してきたようだ」。発話は不明瞭。救急室にいる間に強直間代痙攣を起こした。

・診察 examination

やせている。55kg。38.5℃。口腔カンジダ症(oral candidiasis)。リンパ節腫脹無し。心血管、呼吸器系、消化器系正常。痙攣前における神経検査では時間、場所、人の見当識無し。神経局所症状無し(no focal neurological sign)。眼底両側に乳頭浮腫有り。

・検査 investigation

血算：白血球増多

血液生化学：ナトリウム低下

CT：供覧

■キーワード＆着目するポイント

・口腔カンジダ症(oral candidiasis)

・頭痛、精神症状、強直間代痙攣

・眼底両側に乳頭浮腫

・CT 所見

・低ナトリウム血症は二次的なもの

■アプローチ

・口腔カンジダ症(oral candidiasis)　→　細胞免疫低下状態(DM、免疫抑制、AIDSなど) or 常在細菌叢の攪乱(長期の抗菌薬の使用)

　・The occurrence of thrush in a young, otherwise healthy-appearing person should prompt an investigation for underlying HIV infection.(HIM.1254)

　・More commonly, thrush is seen as a nonspecific manifestation of severe debilitating illness.(HIM.1254)

・精神症状、強直間代痙攣　→　一次的、あるいは二次的な脳の疾患がありそう

・頭痛　→　漠然としていて絞れないが、他の症状からして機能性頭痛ではなく症候性頭痛っぽい。

・眼底両側に乳頭浮腫　→　脳圧亢進の徴候　→　原因は・・・脳腫瘍、ことにテント下腫瘍と側頭葉の腫瘍、クモ膜下出血、脳水腫など、そのほか、眼窩内病変、低眼圧などの局所的要因、悪性高血圧、血液疾患、大量出血、肺気腫などの全身的要因 (vindicate本のp342も参考になる)

　・頭痛と脳圧亢進　→　頭蓋内圧占拠性病変、脳炎(IMD.274)

・CT 所見　→　ringformの病変、脳浮腫、脳圧亢進

・低ナトリウム血症　→　脳ヘルニアに続発して起こることがあるらしい。実際には下垂体にトキソプラズマによる病変が形成されることにより起こりうる。

・そのほか出身地、体重減少もHIVを疑わせる点

パターン認識でHIV + 精神症状 + てんかん発作(強直間代痙攣) + 脳圧亢進 + CT 所見 = 一番ありそうなのはToxoplasma gondiiによるトキソプラズマ脳症 cerebral toxoplasosis (トキソプラズマ脳炎 toxoplasmic encephalitis)

■Toxoplasma gondii

　原虫　胞子原虫類

(感染予防学 080521のプリント、CASES p,92、HIM p.1305-)

・疫学：西洋では30-80%の成人がトキソプラズマの感染の既往がある・・・うぇ(CASES)。日本では10%前後(Wikipedia)。

・生活環

　・終宿主：ネコ：ネコの小腸上皮細胞で有性・無性生殖　糞便にオーシストの排泄

　・中間宿主：ヒト.ブタを含むほ乳類と鳥類：無性生殖で増殖、シストの形成

　　　急性期の増殖盛んな急増虫体 tachyzoiteとシスト内の緩増虫体 bradyzoite

・病原、病因 phathogenesis

　・緩増虫体(bradyzoite)、接合子嚢(oocyst)

・感染経路

　1. オーシストの経口摂取

　2. 中間宿主の生肉中のシストの経口摂取

　3. 初感染妊婦からの経胎盤感染。既感染なら胎盤感染しないらしい(HIM.1306)

　(4)移植臓器、輸血。確率は低い(at low rate)(HIM.1306)

・病態

　1. 先天性トキソプラズマ症　congenital toxoplasmosis

　　　①網脈絡膜炎、 ②水頭症、 ③脳内石灰化、 ④精神・運動障害

　2. 後天性トキソプラズマ症　acquired toxoplasmosis

　　(1) 健常者

　　　・多くは不顕性感染。発熱、リンパ節腫脹、皮疹(rash)

　　　・(少数例)筋肉痛、暈疼痛、腹痛、斑状丘疹状皮疹(maculopapular rash)、脳脊髄炎、混乱(HIM.1308)

　　　・(まれ)肺炎、心筋炎、脳症、心膜炎、多発筋炎

　　　・網膜、脈絡叢に瘢痕や、脳に小さい炎症性の病変を残すことあり(CASES)。

　　　・急性感染の症状は数週間で消失　筋肉や中枢神経系に緩増虫体が残存

　　(2)HIV 感染者、臓器移植例、がん化学療法例

　　　シスト内緩増虫体→急増虫体→播種性の多臓器感染

　　　AIDSでは、トキソプラズマ性脳炎が指標疾患 AIDS-defineing illness(CASES)

・治療

　(日本)アセチルスピラマイシン、ファンシダール(感染予防学 080521)

■トキソプラズマ脳炎 toxoplasmic encephalitis、トキソプラズマ脳症 cerebral toxoplasosis

・症状

　発熱、頭痛、混乱ｍ、痙攣、認知の障害、局所神経徴候(不全片麻痺、歯垢、脳神経損傷、視野欠損、感覚喪失)(CASES)

・画像検査

　(CT,MRI)多発性、両側性、ring-enhancing lesion、特に灰白質-白質境界、大脳基底核、脳幹、小脳が冒されやすい(CASES)

・鑑別診断(臨床症状・画像診断の所見で)

　リンパ腫、結核、転移性脳腫瘍(CASES)

・病歴と画像所見からの鑑別診断

　リンパ腫、結核、転移性腫瘍

？

このCTがcerebral toxoplasmosisに特徴的かは不明

□最後に残る疑問

　AIDSでWBC(leukocyte)の数はどうなるんだろう？？？AIDSの初診患者ではWBCが低い人が多いらしいし()、HIVはCD4+ T cellとmacrophageに感染して殺すから、これによってB cellは減るだろうし、CD8+ T cellも若干減少するだろうからWBCは減るんじゃないか？！好中球はAIDSとは関係ない？好中球は他の感染症に反応性に増加している？ちなみに、好酸球は寄生虫(原虫)の感染のために増える傾向にあるらしい(HIMのどこか)。

葉酸拮抗剤である。

サルファ剤と併用され、抗トキソプラズマ薬、抗ニューモシチス・カリニ薬として相乗的に働く。

ST 合剤

SMX-TMP

スルファメトキサゾール・トリメトプリム合剤 sulfamethoxazole and trimethoprim mixture

□AIDSの定義(http://en.wikipedia.org/wiki/CDC_Classification_System_for_HIV_Infection_in_Adults_and_Adolescents)

According to the US CDC definition, one has AIDS if he/she is infected with HIV and present with one of the following:

A CD4+ T-cell count below 200 cells/μl (or a CD4+ T-cell percentage of total lymphocytes of less than 14%).

or he/she has one of the following defining illnesses:

01. Candidiasis of bronchi, trachea, or lungs
02. Candidiasis esophageal
03. Cervical cancer (invasive)
04. Coccidioidomycosis, disseminated or extrapulmonary
05. Cryptococcosis, extrapulmonary
06. Cryptosporidiosis, chronic intestinal for longer than 1 month
07. Cytomegalovirus disease (other than liver, spleen or lymph nodes)
08. Encephalopathy (HIV-related)
09. Herpes simplex: chronic ulcer(s) (for more than 1 month); or bronchitis, pneumonitis, or esophagitis
10. Histoplasmosis, disseminated or extrapulmonary
11. Isosporiasis, chronic intestinal (for more than 1 month)
12. Kaposi's sarcoma
13. Lymphoma Burkitt's, immunoblastic or primary brain
14. Mycobacterium avium complex
15. Mycobacterium, other species, disseminated or extrapulmonary
16. Pneumocystis carinii pneumonia
17. Pneumonia (recurrent)
18. Progressive multifocal leukoencephalopathy
19. Salmonella septicemia (recurrent)
20. Toxoplasmosis of the brain
21. Tuberculosis
22. Wasting syndrome due to HIV

People who are not infected with HIV may also develop these conditions; this does not mean they have AIDS. However, when an individual presents laboratory evidence against HIV infection, a diagnosis of AIDS is ruled out unless the patient has not:

undergone high-dose corticoid therapy or other immunosuppressive/cytotoxic therapy in the three months before the onset of the indicator disease
OR been diagnosed with Hodgkin's disease, non-Hodgkin's lymphoma, lymphocytic leukemia, multiple myeloma, or any cancer of lymphoreticular or histiocytic tissue, or angioimmunoblastic lymphoadenopathy
OR a genetic immunodeficiency syndrome atypical of HIV infection, such as one involving hypogamma globulinemia

AND

the individual has had Pneumocystis carinii pneumonia
OR one of the above defining illnesses AND a CD4+ T-cell count below 200 cells/μl (or a CD4+ T-cell percentage of total lymphocytes of less than 14%).

□AIDSのステージング

Revised World Health Organization (WHO) Clinical Staging of HIV/AIDS For Adults and Adolescents (2005)

http://en.wikipedia.org/wiki/WHO_Disease_Staging_System_for_HIV_Infection_and_Disease_in_Adults_and_Adolescents

■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 34」

　　[★]

☆case34　てんかん発作

■症例

23歳　アフリカ-カリブ系　女性　銀行の店員

主訴：てんかん発作

現病歴：強直間代発作を2回起こしたところを母親が目撃していた。娘の行動は次第におかしくなり、また娘は自分のことを話している声が聞こえていた(幻聴)。最近、頭痛を訴えていた。体重が減少し、脱毛があった。盗汗と主に足と手の小関節に及ぶflitting 関節痛を訴えていた。服用薬なし。タバコ1日5-10本、アルコール10 unit/week(缶ビール(350ml)6本弱)

既往歴：特記なし(medical or psychiatric history)

家族歴：

生活歴：

・身体診断

意識：眠そうだが痛みに反応。頚部硬直：なし。頭髪：薄く斑状。体温38.5℃。リンパ節：たくさんの部位で触知。

心拍：104/分、整。血圧：164/102 mmHg。心血管系、呼吸器系、腹部：異常なし。

神経所見：focal anormalityなし(局所性異常)。乳頭浮腫：認めず。

・検査

(血液生化学)

上昇：erythrocyte sedimentation rate、urea、creatinine

低下：hemoglobin、white cell count、platelet

正常：mean corpuscular volume、sodium、potassium、glucose

(腰椎穿刺)

上昇：leukocytes、CSF protein

正常：CSF glucose

(尿検査)

蛋白：＋＋＋。血尿：＋＋＋。赤血球：＋＋。赤血球円柱：あり。

(その他)

胸部X線：異常なし。ECG：洞性頻脈。頭部 CT：正常。CSF グラム染色：陰性(光顕レベルで細菌なし)

■glossary

fall out　(毛髪などが)抜ける

night sweat 盗汗、寝汗

tonic-clonic generalized seizure 全般性強直間代発作

flit　vi (鳥・蝶など)すいすい/ひらひら飛ぶ、飛び回る、飛翔する。(人が)軽やかに通る、行き交う。(時が)(飛ぶように)すぎる。(幻想など)去来する、よぎる、(表情が)かすめる

drowsy　adj. 眠い。眠そうな。眠気を誘う。ものうい。眠ったような

stiffness　n. 堅いこと、堅ぐるしさ、がんこさ

delirium tremens 振戦譫妄

hypertensive encephalopathy 高血圧性脳症

alopecia　脱毛症

African-Caribbean アフリカ-カリブ系

fit n.　(病気の)発作。引きつけ、痙攣。(感情の)激発、一時的興奮、気まぐれ

chorea n. 舞踏病

recreational drug 耽溺性があり乱用される脱法薬物

urgent adj. (物・事が)急を要する、緊急の、背拍質(pressing)。(～を)緊急に必要とする(in)。(S is ～ (with O1) for [in] O2)(人が)(O1(人)に)O2(物・事)をしつこく求める。(人に/～するように)催促する(for/to do)。(要求などが)執拗な。

□てんかん発作

・新規発生したてんかん発作の原因(ICU.805-806)

・薬物中毒(テオフィリンなど)

・薬物からの離脱(アルコールなど)

・感染症(髄膜炎、膿瘍など)

・頭部外傷

・虚血障害(虚血あるいはびまん性)

・占拠性病変(腫瘍あるいは血腫)

・代謝性障害(肝性脳症、尿毒症性脳症、敗血症、低血糖、低ナトリウム血症、低カルシウム血症など)

・合併症(ICU.805-806)