キアリ・アーノルド奇形
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- 1. キアリ奇形chiari malformations [show details]
…tonsils that are displaced below the level of the foramen magnum Chiari II malformation (CM-II), also known as Arnold-Chiari malformation, is characterized by downward displacement of the cerebellar vermis …
- 2. 小児における先天性中枢性低換気症候群および睡眠関連低換気症のその他の原因congenital central hypoventilation syndrome and other causes of sleep related hypoventilation in children [show details]
… Most of the patients with CSA had neurologic disorders, including myelomeningocele with Chiari II malformation, brainstem compression, encephalitis, tumors, and genetic syndromes. Brainstem and/or cranial …
- 3. 脊髄髄膜瘤（二分脊椎）の病態生理および臨床症状pathophysiology and clinical manifestations of myelomeningocele spina bifida [show details]
…to detect and prevent further deterioration of function. The Chiari II malformation (sometimes termed the Arnold-Chiari malformation, a misnomer) is characterized by downward displacement of the cerebellar …
- 4. 脊髄髄膜瘤（二分脊椎）マネージメントの概要overview of the management of myelomeningocele spina bifida [show details]
…however, symptomatic retethering may occur in some patients . The Chiari II malformation (also known as Arnold-Chiari malformation type II, a misnomer) is characterized by herniation of the developing …
- 5. 神経管欠損症の超音波診断ultrasound diagnosis of neural tube defects [show details]
English Journal
- Tectocerebellar dysraphia with posterior encephalocele (Friede): report of the youngest case. Reappraisal of the condition uniting Cleland-Chiari (Arnold-Chiari) and Dandy-Walker syndromes.
- , H H, A A, A A, .
- Clinical neuropathology. ;13(4)216-20.
- This is a neuropathological report of the youngest case ever recorded of tectocerebellar dysraphia with posterior encephalocele. All previously described stigmata were ascertained as well as a few additional findings. Some morphological features of this syndrome similar to and some different from th
- PMID 7955668
- Cerebellocele and associated central nervous system anomalies in the Meckel syndrome.
- Aleksic S, Budzilovich G, Greco MA, Reuben R, Feigin I, Pearson J, Epstein F.
- Child's brain. 1984 ;11(2)99-111.
- Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the
- PMID 6723431
Japanese Journal
- 知覚障害を欠きMRIにて巨大なsyrinxが描出された脊髄空洞症の1例
- 外松 明美,田中 真,岡本 幸市,平井 俊策
- 北関東医学 40(3), 343-347, 1990
- … Chiari-Arnold type 1 malformation was also demonstrated. …
- NAID 130003690347
- 門脇 弘孝,今永 浩寿,喜多村 孝一
- 脳と発達 7(4), 256-262, 1975
- … 4, Chiari Arnold malformation ; …
- NAID 130004184180
Related Links
- WebMD explains the symptoms and treatment of Chiari malformation, a type of birth defect that can cause problems with balance and coordination. ... Chiari malformations are structural defects in the cerebellum. That's ...
- What are Chiari malformations?What causes these malformations?What are the symptoms of a Chiari malformation?How are CMs classified?What other conditions are associated with Chiari malformations?How common ...
- Doctors categorize Chiari malformation into three types, depending on the anatomy of the brain tissue that is displaced into the spinal canal, and whether developmental abnormalities of the brain or spine are present. Chiari ...
★リンクテーブル★
[★]
- 英
- Chiari malformation
- 同
- Chiari奇形、アーノルド-キアリ症候群 Arnold-Chiari syndrome、アーノルド・キアリ奇形 Arnold-Chiari奇形 Arnold-Chiari malformation、キアリ-アーノルド奇形 Chiari-Arnold malformation
概念
- 小脳や脳幹が大後頭孔を通じて脊椎管内に下垂した先天性奇形の総称である。
定義
- 先天性水頭症を伴う小脳奇形の4型 → アーノルド・キアリ奇形(1891年Chiariが記載) → 後脳奇形全体をまとめてアーノルド・キアリ症候群とよぶ
Chiari奇形I型とChiari奇形II型。その他
- SCN.282改変
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I型
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II型
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III型
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IV型
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| 脊柱管への陥入
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小脳扁桃
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延髄、
第四脳室、
小脳虫部
|
(頚部の二分脊椎部に
小脳が陥入(脳瘤)
。
延髄下垂。水頭症)
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(小脳の形成不全)
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| 発症年齢
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小児、成人
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乳幼児
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|
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| 臨床症状
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非特異的
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喉頭喘鳴、
無呼吸発作、
嚥下困難
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|
|
| 水頭症
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＋
|
＋＋＋
|
|
|
| 脊髄髄膜瘤
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－
|
＋＋＋
|
|
|
| 脊髄空洞症
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＋＋
|
＋
|
|
|
病型 SOR.437
I型
- (MRI像、頭蓋底陥入症、脊髄空洞症合併が見られる。矢頭は小脳扁桃)
- 脊髄空洞症を伴いやすい
II型
異常部位
| 大脳
|
脳梁形成不全、大脳鎌形成不全、小多脳回
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| 小脳
|
下垂
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| 中脳
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中脳水道の閉塞
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| 橋
|
|
| 延髄
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下垂
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| 脳室
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水頭症(ほぼ全例)(側脳室・第3脳室の拡大、側脳室後角の拡大)
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| 脊椎
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脊髄髄膜瘤(ほぼ全例)
|
III型
- 頚部の二分脊椎部に小脳が陥入(脳瘤)。延髄下垂。水頭症
IV型
症状
- I型：咳嗽、くしゃみ、怒責により頭蓋内圧が亢進して頭痛を来す。
- II型：脳幹症状(吸気時喘鳴、無呼吸発作、嚥下障害、水頭症に伴う脳室拡大。)
検査
治療
- 頭蓋内圧が高い場合には脳室シャント術を行う
- 後頭下減圧術(大孔減圧術)である
[★]
- 英
- Chiari-Arnold malformation
[★]
-malformation
L. 138
- 関
- abnormality、deformity、dysgenesis、dysplasia、dysplastic、malformed、teratologic