Extreme asthenozoospermia and chronic respiratory disease: a new variant of the immotile cilia syndrome

Summary Two patients suspected of suffering from ciliary dyskinesis were investigated. They consulted for primary infertility and chronic respiratory disease. Functional lung studies showed obstructive changes in one patient. Both had immotile sperm with short, thick and rigid tails. Ultrastructural studies of nasal biopsies showed abnormal cilia with almost complete lack of inner dynein arms (mean number of inner arms per axoneme 0.67 ± 1.21 in patient 1 and 1.49 ± 1.17 in patient 2, compared with normal values of 5.3 ± 0.13). Other abnormalities included lack of parallel orientation of cilia and central translocation of microtubular doublets. Electron microscopy of sperm revealed hyperplasia of the fibrous sheath and axonemal disruption. This is the first report of an association of different anomalies in cilia and flagella leading to clinical manifestation of the immotile cilia syndrome. These findings emphasize the need for ultrastructural examination of respiratory cilia in men suffering from fibrous sheath alterations of sperm which so far have not been described in patients with the classical form of immotile cilia syndrome.