Cerebrospinal fluid hypersecretion in pediatric hydrocephalus

被引:52
作者
Karimy, Jason K. [1 ]
Duran, Daniel [1 ]
Hu, Jamie K. [1 ]
Gavankar, Charuta [1 ]
Gaillard, Jonathan R. [1 ]
Bayri, Yasar [4 ]
Rice, Hunter [1 ]
DiLuna, Michael L. [1 ]
Gerzanich, Volodymyr [5 ]
Simard, J. Marc [5 ,6 ]
Kahle, Kristopher T. [1 ,2 ,3 ]
机构
[1] Yale Sch Med, Dept Neurosurg, 300 Cedar St,TAC S311, New Haven, CT 06519 USA
[2] Yale Sch Med, Dept Pediat Cellular & Mol Physiol, New Haven, CT USA
[3] Yale Sch Med, Ctr Mendelian Genom, New Haven, CT USA
[4] Marmara Univ, Sch Med, Dept Neurosurg, Istanbul, Turkey
[5] Univ Maryland, Sch Med, Dept Neurosurg, Baltimore, MD 21201 USA
[6] Univ Maryland, Sch Med, Dept Pathol & Physiol, Baltimore, MD 21201 USA
基金
美国国家卫生研究院;
关键词
pediatric hydrocephalus; choroid plexus; cerebrospinal fluid; ion transport; epithelia; NKCC1; ENDOSCOPIC 3RD VENTRICULOSTOMY; CHOROID-PLEXUS CAUTERIZATION; DIFFUSE VILLOUS HYPERPLASIA; ALANINE-RICH KINASE; CSF PRODUCTION; MOLECULAR PHYSIOLOGY; SURGICAL-MANAGEMENT; INTERSTITIAL FLUID; LATERAL VENTRICLE; CLINICAL ARTICLE;
D O I
10.3171/2016.8.FOCUS16278
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Hydrocephalus, despite its heterogeneous causes, is ultimately a disease of disordered CSF homeostasis that results in pathological expansion of the cerebral ventricles. Our current understanding of the pathophysiology of hydrocephalus is inadequate but evolving. Over this past century, the majority of hydrocephalus cases has been explained by functional or anatomical obstructions to bulk CSF flow. More recently, hydrodynamic models of hydrocephalus have emphasized the role of abnormal intracranial pulsations in disease pathogenesis. Here, the authors review the molecular mechanisms of CSF secretion by the choroid plexus epithelium, the most efficient and actively secreting epithelium in the human body, and provide experimental and clinical evidence for the role of increased CSF production in hydrocephalus. Although the choroid plexus epithelium might have only an indirect influence on the pathogenesis of many types of pediatric hydrocephalus, the ability to modify CSF secretion with drugs newer than acetazolamide or furosemide would be an invaluable component of future therapies to alleviate permanent shunt dependence. Investigation into the human genetics of developmental hydrocephalus and choroid plexus hyperplasia, and the molecular physiology of the ion channels and transporters responsible for CSF secretion, might yield novel targets that could be exploited for pharmacotherapeutic intervention.
引用
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页数:11
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