Orbicularis oculi muscle activation during swallowing in humans.

Intraoral trigeminal afferents elicit EMG activity from the lower facial muscle, orbicularis oris (OR) during swallowing. The upper facial muscles and especially orbicularis oculi (OC) were not previously known to be associated with deglutitional events. Nevertheless, given the large area of intraoral mucosa and teeth innervated by the trigeminal nerve afferents, a connection between OC motoneurons and deglutition may theoretically be expected, which we sought to evaluate in this study. Healthy controls were investigated for the possible synchronization of orbicularis OC and OR muscles during deglutition by the following methods: EMG activities were recorded during voluntary dry, 3-, 10-, 20-ml discrete wet swallowing, and sequential swallowing from a cup, concurrent with respiratory recording. A polygraphic recording was obtained from these muscles to determine whether they were synchronously activated during spontaneous swallowing. The polygraphic recording during spontaneous swallowing demonstrated that the OC and OR muscles were synchronously activated in all subjects. This synchronous activation was less prominent in voluntary discrete swallowing. It is proposed that this might be based on trigemino-solitarii-facial pathways with weaker connection to OC muscles. The synchronization of OC muscle activity with deglutition may be an evolutionary process that should be rudimentary in higher mammals including humans. The swallowing-induced cranial muscle activities could potentially explain some movement disorders, such as craniofacial dystonias.

A regional panorama of dysferlinopathies.

OBJECTIVE: We describe the characteristic features of 11 patients (6 men and 5 women) with dysferlinopathies confirmed by muscle biopsies. In addition, we aimed to provide a realistic comprehensive picture of the severe muscle diseases in the Aegean Region of Turkey. MATERIAL AND METHOD: We retrospectively reviewed 90 patients who underwent muscle biopsy examinations between 2008 and 2011 in the pathology laboratory of Izmir Dr.Behcet Uz Children's Hospital. Biopsy specimens of all patients clinically diagnosed as muscular dystrophy referred from 4 different centers of neurological disorders were collected. RESULTS: Dystrophinopathy was the most (n=45) and gammasarcoglycanopathy was the second common (n=13) muscular dystrophy in this series. The mean age of all 90 patients was 8.8 years (3 months- 64 years). Only 14 cases (15.5%) were older than 14, and 23 cases were younger than two years. Dysferlinopathy was the most common dystrophy in the older age group. There were statistical significant differences between the types of dystrophy and inflammation (0.021), creatine kinase levels (p= 0.001), age (p=0.001), and gender (p < 0.001) of the patients. CONCLUSION: The present study revealed that dysferlinopathies is not an uncommon form of muscular dystrophies in western Turkey. We have concluded that if avoidance from unnecessary therapeutic interventions is desired, we must be aware of the relative frequencies of dysferlinopathies.