小脑半球发育不全
Hypoplasia or agenesis of cerebellar hemispheres
- 上级条目
- LA06 小脑结构发育异常
- 层级
- 5 级
定义
小脑发育不全相当于小脑结构发育不全，可涉及小脑蚓部和/或小脑半球，从部分到全部发育不全。它被描述为与各种临床实体有关：染色体异常、子宫内暴露于毒素和感染性病原体、代谢紊乱（糖基化紊乱和CoQ10缺陷）以及各种罕见的遗传性神经疾病。它可局限于小脑，或影响其他中枢神经系统结构：中脑（磨牙综合征）、桥脑和延髓（桥脑-小脑发育不良）、大脑皮层（无脑回小脑发育不全综合征）。
Cerebellar hypoplasia corresponds to underdevelopment of cerebellar structures that can involve the vermis and/or the cerebellar hemispheres from partial to total agenesis. It has been described in the context of various clinical entities: chromosomal anomalies, in utero exposure to toxins and infectious agents, metabolic disorders (disorders of glycosylation and CoQ10 deficiencies), and a wide variety of rare genetic neurological diseases. It can be confined to the cerebellum, or affect other CNS structures: the midbrain (molar tooth syndromes), pons and medulla (ponto-cerebellar hypoplasia), cerebral cortex (lissencephaly cerebellar hypoplasia syndromes).
除外词 明确排除、需编到其他条目的情况
英文词条（1）
索引词 可用于检索该条目的同义词与别名
- 小脑半球发育不全
- 单侧孤立性半球小脑发育不全
- 双侧孤立性半球小脑发育不全
英文词条（3）
- Hypoplasia or agenesis of cerebellar hemispheres
- Isolated unilateral hemispheric cerebellar hypoplasia
- Isolated bilateral hemispheric cerebellar hypoplasia
后组配轴 Postcoordination
该条目未定义后组配轴，直接使用上面的编码即可。后组配是在主干编码之后追加侧别、具体解剖部位、严重程度、病因等细节的可选组合方式，不是必填项。