Long-term results of cricopharyngeal myotomy in oculopharyngeal muscular dystrophy.

Objective: To analyze long-term results of extramucosal cricopharyngeal myotomy in oculopharyngeal muscular dystrophy.

Study Design: The preoperative and postoperative evaluations including symptoms, type of feeding, weight, and functional examinations were retrospectively evaluated in 39 patients. Results were defined postoperatively as successful, partial, or failed.

Defective satellite cells in congenital myotonic dystrophy.

In this study we have developed an in vitro cell culture system which displays the majority of the defects previously described for congenital myotonic dystrophy (CDM) muscle in vivo. Human satellite cells were isolated from the quadriceps muscles of three CDM fetuses with different clinical severity. By Southern blot analysis all three cultures were found to have approximately 2300 CTG repeats.

Swallowing disorders in paralysis of the lower cranial nerves: a functional analysis.

Deficits of the lower cranial nerves (nerves IX, X, XI, and XII) occurring after treatment of skull base tumors may cause disabling swallowing disorders. To assess the mechanisms of swallowing disorders involved in such cases, we performed functional examinations: a videoendoscopic swallowing study and simultaneous manometry and videofluoroscopy in 7 patients. This study shows that the main mechanism of the swallowing disorders was a disturbance of the pharyngeal stage, including a decrease of pharyngeal propulsion, reduced laryngeal closure, and cricopharyngeal dysfunction, which led to aspiration.