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1. 舞踏病の概要overview of chorea [show details]
… varies from childhood to the 80s. Chorea is a key feature of HD in adults, and the defining sign at the time of diagnosis. Onset of symptoms before 20 years of age is considered juvenile HD. The clinical …
2. シデナム舞踏病sydenham chorea [show details]
… often in school-aged children. Symptoms of SC usually begin one to eight months after the inciting infection. The onset is typically insidious but may be abrupt. Neurologic symptoms include chorea (ie, …
3. 小児における運動過多な運動障害hyperkinetic movement disorders in children [show details]
…normal infants make movements that resemble chorea, but this physiologic chorea resolves by eight months of age. Children with attention deficit disorder and hyperactivity may have distal chorea (chorea …
4. 急性リウマチ熱：臨床症状および診断acute rheumatic fever clinical manifestations and diagnosis [show details]
… Sydenham chorea (also known as chorea minor or "St. Vitus dance") is a neurologic disorder consisting of abrupt, nonrhythmic, involuntary movements, muscular weakness, and emotional disturbances . Chorea has… 100,000 school-aged children per year or all-age rheumatic heart disease [RHD]…
5. 急性リウマチ熱：疫学および病因acute rheumatic fever epidemiology and pathogenesis [show details]
… in the development of Sydenham chorea in patients with ARF. In an animal model, monoclonal antibodies that caused chorea bound to both NABG and mammalian lysoganglioside .… 100,000 school-aged children worldwide , but it is lower (≤2 cases per 100,000 school-aged children) in the United States and other developed countries .…

English Journal

Brain structure in juvenile-onset Huntington disease.

Tereshchenko A, Magnotta V, Epping E, Mathews K, Espe-Pfeifer P, Martin E, Dawson J, Duan W, Nopoulos P.
Neurology. 2019 Apr;92(17)e1939-e1947.
To assess brain morphometry in a sample of patients with juvenile-onset Huntington disease (JOHD) and several mouse models of Huntington disease (HD) that likely represent the human JOHD phenotype. Despite sharing the mutation in the Huntingtin gene, adult-onset HD characteristically presents as a h
PMID 30971481

Assessment of the Performance of a Modified Motor Scale as Applied to Juvenile Onset Huntington's Disease.

Horton MC, Nopoulos P, Nance M, Landwehrmyer GB, Barker RA, Squitieri F, , Burgunder JM, Quarrell O.
Journal of Huntington's disease. 2019 ;8(2)181-193.
Huntington's disease can present at almost any age but traditionally, those with an onset ≤20 years are described as having juvenile onset Huntington's disease (JOHD). They are more likely to have bradykinesia and dystonia earlier in the course of the disease. The Total Motor Score of the Unified
PMID 30856116

Juvenile Huntington's disease: left behind?

, S S, J J, J J, .
The Lancet. Neurology. 2018 11;17(11)932-933.
PMID 30243863

Japanese Journal

ハンチントン病--update

長谷川一子
MDSJ letters 4(1), 4-9, 2011
NAID 40018916229

若年で高血糖性 chorea-ballism を発症したMELASの1例

中垣英明,古屋淳一郎,三田洋 [他],長野祐久,荒木栄一,山田猛
臨床神経学 : CLINICAL NEUROLOGY 45(7), 502-505, 2005-07-01
NAID 10016605411

乳児期後半から発達遅滞を認めた歯状核赤核淡蒼球ルイ体萎縮症（ＤＲＰＬＡ）の１例

金山学,塚本東子,宮地泰士,濱口貴代,藤本伸治,石川達也,戸苅創
脳と発達 36(5), 407-412, 2004
早期発症の歯状核赤核淡蒼球ルイ体萎縮症 (dentatorubral-pallidoluysian atrophy: DRPLA) の症例 (女児) を経験した.症例は生後6カ月に座位を獲得するまでは正常発達.その後, 発達遅滞が見られ, 加えて小脳失調症状を伴い, 4歳6カ月以後ミオクロニー発作が起こってからは発達の退行も見られた.脳波異常があり頭部MRIでも小脳と脳幹の萎縮などの所見が見られた …
NAID 130004068290