Schwartz-Jampel syndrome (SJS) is a rare disorder characterized by micrognathia, kyphoscoliosis, and myotonia. The greatest challenge in the anesthetic management of patients with SJS is performing tracheal intubation. The MultiViewScope (MVS) is a video laryngoscope system in which the video monitor handle can be attached to a stylet scope, laryngoscope blade, or fiberscope. We report a 21-mon...

Despite continuing controversy, it is widely assumed that children with known or suspected myopathies are at increased risk for malignant hyperthermia (MH) (1). Previous reports have suggested a relationship between MH risk and a variety of neuromuscular disorders including Duchenne type muscular dystrophy (DMD), osteogenesis imperfecta, myotonia congenita, the Schwartz-Jampel syndrome, and oth...

In 1950 Bassen and Kornzweig described a new syndrome when they reported the presence of irregular erythrocytes in an 18-year-old Jewish girl who had retinitis pigmentosa and a neurological disorder similar to Friedreich's ataxia. Several years later they described the same triad (abnormal erythrocytes, retinitis pigmentosa, and neuropathy) in the patient's brother (Kornzweig and Bassen, 1957)....

Skeletal muscle fibers from a patient with Schwartz-Jampel syndrome were studied in vitro. The fibers had normal resting membrane potentials, but their resting [Ca2+]i was elevated. The resting potentials were unstable and spontaneous depolarizations caused twitching in all fibers. Stimulated contractions were characterized by markedly slowed relaxation which was due to electrical after-activit...

The selection process of team leaders is fundamental if the effectiveness of teams is to be guaranteed. Human values have proven to be an important factor in the behaviour of individuals and leaders. The aim of this study is twofold. The first is to validate Schwartz's survey of human values. The second is to determine whether there are any relationships between the values held by individuals a...