Brain dysfunction in primary ciliary dyskinesia?
To identify possible altered flow of cerebrospinal fluid due to defective ependymal ciliary motion, 8 subjects, including 6 with Kartagener's syndrome (KS), 1 with primary ciliary dyskinesia (PCD) and 1 with situs inversus were studied by computerized tomography (CT) examination of the brain and paranasal sinuses and had their nasal cilia studied by in vitro ciliary beat frequency (CBF) analysis. Five of the 6 patients with KS and the one with PCD had abnormal CBF (slowed or absent), while the CBF of 1 KS patient and the subject with situs inversus was normal. CT evaluation of the brains of all subjects was normal. Sinus examinations of the 7 patients with KS and PCD revealed mucosal thickening. We conclude that slowed or absent ciliary activity as measured in vitro is not associated with brain abnormalities as demonstrated by CT examination.
Roth
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Y
Department of Polymer Research, Weizmann Institute of Science, Rehovot, Israel.
Baum
G L
GL
Tadmor
R
R
D000293:Adolescent; D000328:Adult; D002552:Cerebral Ventricles / Q000503:physiopathology*; D002648:Child; D002923:Cilia / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000000981:diagnostic imaging / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed