出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2019/08/07 04:20:29」(JST)
Chorea | |
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Specialty | Neurology |
Chorea (or choreia, occasionally) is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. The term chorea is derived from the Ancient Greek: χορεία ("dance"; see choreia), as the quick movements of the feet or hands are comparable to dancing.
The term hemichorea refers to chorea of one side of the body, such as chorea of one arm but not both (analogous to hemiballismus).
Chorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic, but appear to flow from one muscle to the next.
These 'dance-like' movements of chorea often occur with athetosis, which adds twisting and writhing movements. Walking may become difficult, and include odd postures and leg movements.
Unlike ataxia, which affects the quality of voluntary movements, or Parkinsonism, which is a hindrance of voluntary movements, the movements of chorea and ballism occur on their own, without conscious effort. Thus, chorea is said to be a hyperkinetic movement disorder.
When chorea is serious, slight movements will become thrashing motions; this form of severe chorea is referred to as ballism or ballismus.
Huntington's disease is a neurodegenerative disease and most common inherited cause of chorea. The condition was formerly called Huntington's chorea but was renamed because of the important non-choreic features including cognitive decline and behavioural change.[1]
Other genetic causes of chorea are rare. They include the classical Huntington's disease 'mimic' or phenocopy syndromes, called Huntington's disease-like syndrome types 1, 2 and 3; inherited prion disease, the spinocerebellar ataxias type 1, 3 and 17, neuroacanthocytosis, dentatorubral-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders, Wilson's disease, benign hereditary chorea, Friedreich's ataxia, mitochondrial disease and Rett syndrome.[2]
The most common acquired causes of chorea are cerebrovascular disease and, in the developing world, HIV infection—usually through its association with cryptococcal disease.[2]
Sydenham's chorea occurs as a complication of streptococcal infection. Twenty percent (20%) of children and adolescents with rheumatic fever develop Sydenham's chorea as a complication. It is increasingly rare, which may be partially due to penicillin, improved social conditions, and/or a natural reduction in the bacteria ( Streptococcus ) it has stemmed from. Psychological symptoms may precede or accompany this acquired chorea and may be relapsing and remitting. The broader spectrum of paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection can cause chorea and are collectively referred to as PANDAS.[2]
Chorea gravidarum refers to choreic symptoms that occur during pregnancy. If left untreated, the disease resolves in 30% of patients before delivery but, in the other 70%, it persists. The symptoms then progressively disappear in the next few days following the delivery.[2]
Chorea may also be caused by drugs (commonly levodopa, anti-convulsants and anti-psychotics).[2]
Other acquired causes include systemic lupus erythematosus, antiphospholipid syndrome, thyrotoxicosis, polycythaemia rubra vera,[2] transmissible spongiform encephalopathies and coeliac disease.[3]
There is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Although there are many drugs that can control it, no cure has yet been identified.
Form | Treatment |
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Huntington's-related | A common treatment is dopaminergic antagonists, although treatment is largely supportive. Tetrabenazine is the only FDA approved drug for the treatment of Huntington's Disease related chorea. |
Sydenham's chorea | Haloperidol, carbamazepine and valproic acid. Usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence. |
Chorea gravidarum | haloperidol,[4][5][6] chlorpromazine alone or in combination with diazepam, also pimozide can also be used. |
Wilson's disease | Reducing levels of copper in the body using D-penicillinamine, trientine hydrochloride, tetrathiomolybdate, and other chelating agents |
Drug-induced chorea | Adjusting medication dosages. |
Metabolic and endocrine-related choreas | Treated according to their causes. |
Historically, choreas like Huntington disease and Sydenham's chorea were called Saint Vitus' dance, related to a series of social phenomena of the same name.[7]
Classification | D
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External resources |
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Diseases of the nervous system, primarily CNS (G04–G47, 323–349) | |||||||||||||||||||||||||
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Spinal cord/ myelopathy |
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Both/either |
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リンク元 | 「不随意運動」「舞踏様運動」「choreatic syndrome」「senile chorea」「choreatic disorder」 |
関連記事 | 「movement」「choreiform」 |
大脳基底核の障害との関連 | 特徴 | 好発部位 | 代表疾患 | ||
振戦 | tremor | 黒質 | 律動的な振動運動 | 指、手 | Parkinson病 本態性振戦 |
舞踏病様運動 | choreiform movement | 尾状核 | 不規則で、目的のない、非対称性運動 [show details] <youtube>http://www.youtube.com/watch?v=JzAPh2v-SCQ</youtube>
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顔面、四肢 | Huntington舞踏病 |
バリズム | ballism/ballismus | 視床下核 | 舞踏様病の一種。運動はより急速、粗大、持続性。四肢の抹消よりも体幹に誓い部分に強く起こり、上下肢を投げ出すよう激しい運動 [show details] <youtube>http://www.youtube.com/watch?v=Cn3WSt6omzY</youtube>
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四肢 | 視床下核 Juys体の出血・梗塞 |
アテトーゼ | athetosis | 赤核、被殻、淡蒼球 | 舞踏病よりゆっくりで、持続性のある運動。舞踏病に比べ一定の運動。虫が這うような運動。 [show details] <youtube>http://www.youtube.com/watch?v=I63SobW58J0</youtube>
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手・指 | 脳性麻痺 CO中毒 レンズ核障害 |
ミオクローヌス | myoclonus | 赤核 | 1つまたは多くの筋の短時間の不随意な収縮。関節や四肢の強い運動を伴わないのが原則 [show details] <youtube v=faiVIKmmi5k></youtube>
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全身・局所 | Creutzfeldt-Jakob病 Ramsay Hunt症候群 てんかん リピドーシス ミトコンドリア脳筋症 |
痙攣 | cramp/convulsion | 筋肉が不随意に,激しく攣縮する状態 | |||
ジストニー | dystonia | 異常姿勢。筋緊張の亢進で異常な姿勢となり、体幹の捻転、胸郭の傾斜、頚の捻転、肘の過伸展、手首の過屈曲、指の過伸展などを呈する。 [show details] <youtube v=1MQtaMENDV0></youtube>
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体幹・近位筋 | 捻転ジストニー | |
チック | tic | 顔、頚部、肩などに起こる、比較的急激で、繰り返して起こる運動 [show details] <youtube>http://www.youtube.com/watch?v=xIAbmfxIm6I</youtube>
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顔面 | てんかん 緊張 |
<youtube>http://www.youtube.com/watch?v=aV85-BOkM64</youtube> <youtube>http://www.youtube.com/watch?v=SGkCQC2QSRg</youtube> <youtube>http://www.youtube.com/watch?v=kINXIjs_V3M</youtube>
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