EDITOR: Propofol is used when deep sedation, immobilization and quick emergence are required and is often used in children during magnetic resonance imaging (MRI) scans. Because the MRI scan is prolonged, a propofol infusion or repetitive small boluses are usually administered. A 13-yr-old patient (weight 44 kg), underwent MRI scan of the head for evaluation of recurrent headaches. The patient ...

It has been increasingly apparent in recent years that in addition to cases which fall into recognizable categories of muscle disease, a number of less common disorders occur from time to time which do not correspond to the accepted descriptions. Some of these appear to be metabolic in origin and can be elucidated, at least in part, by modern methods of investigation (McArdle, 1951) while other...

inflammatory myopathy is a paraneoplastic syndrome. inflammatory myopathy may be the first manifestation of underlying malignancy. it was reported in patients with colon cancer, breast cancer, ovarian cancer, lung cancer and non-hodgkin lymphoma. there are few reports regarding inflammatory myopathy in patients with gastric cancer. we want to present inflammatory myopathy as early manifestation...

Background: Statins frequently cause myopathy especially in combination with fibrates, and physical activity is considered a trigger for the muscle disorder. Elevated plasma levels of creatine kinase (CK), lactate dehydrogenase (LDH) and aldolase, are the main indicators of the severity of myopathy. Carvedilol is commonly used with lipid-lowering drugs in the management of heart failure, hypert...

Congenital muscle fiber-type disproportion is a condition that can be defined only in the muscle biopsy by 2 obligatory criteria of “disproportion”: (1) a massive type I myofiber predominance of 80% or more, and (2) myofibers of type I are uniformly smaller than normal for age by 2 standard deviations or more, but are not necessarily angular or rounded as in myofiber atrophy. Internal sarcolemm...

Inherited metabolic disorders are single-gene genetic diseases associated with multiorgan damage. Some of these conditions increase the risk of stroke through a variety of mechanisms, and there is evidence that early recognition and initiation of appropriate treatment may improve prognosis. In this 2-part review we provide an update of the genetics, stroke pathophysiology, clinical manifestatio...

Introduction In January 2012, a program was set up to rapidly screen patients with undiagnosed muscle disorders who were referred to a neuromuscular specialist in an adult hospital. A nurse was trained to do the DBS procedure as part of a series of required laboratory tests. All individuals referred to a neuromuscular specialist, either within the context of a neuromuscular clinic or an EMG lab...

Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients might have cerebellar ataxia, a high content of protein in the cerebrospinal fluid, proximal myopath...