We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Ependymal ciliary motion and their role in congenital hydrocephalus.
- Authors
Sakamoto, Koichiro; Nakajima, Madoka; Kawamura, Kaito; Nakamura, Eri; Tada, Norihiro; Kondo, Akihide; Arai, Hajime; Miyajima, Masakazu
- Abstract
Purpose: Since a case of hydrocephalus in humans considered to be caused by ciliary dysfunction was first reported by Greenstone et al. in 1984, numerous papers on the correlation between ciliary function and hydrocephalus have been published. Methods: We reviewed the published literature on primary ciliary dyskinesia in humans causing hydrocephalus, focusing on articles specifically examining the relation between ciliary function and hydrocephalus and its treatment. In addition, the authors' experience is briefly discussed. Results: Full texts of 16 articles reporting cases of human hydrocephalus (including ventriculomegaly) due to defects in ependymal ciliary function or primary ciliary dyskinesia observed in clinical practice were extracted. In recent years, studies on animal models, especially employing knockout mice, have revealed genetic mutations that cause hydrocephalus via ciliary dysfunction. However, a few reports on the onset of hydrocephalus in human patients with primary ciliary dyskinesia have confirmed that the incidence of this condition was extremely low compared to that in animal models. Conclusion: In humans, it is rare for hydrocephalus to develop solely because of abnormalities in the cilia, and it is highly likely that other factors are also involved along with ciliary dysfunction.
- Subjects
CILIA &; ciliary motion; DYSKINESIAS; CILIARY motility disorders; HYDROCEPHALUS; GENETIC mutation; KNOCKOUT mice
- Publication
Child's Nervous System, 2021, Vol 37, Issue 11, p3355
- ISSN
0256-7040
- Publication type
Article
- DOI
10.1007/s00381-021-05194-9