High resolution sonography in the evaluation of the peripheral nervous system in polyneuropathy – a review of the literature

Clinical, laboratory and electrodiagnostic studies are the mainstay in the diagnosis of polyneuropathy. An accurate etiological diagnosis is of paramount importance to provide the appropriate treatment, prognosis and genetic counselling. High resolution sonography of the peripheral nervous system allows nerves to be readily visualized and to assess their morphology. Ultrasonography has brought pathophysiological insights and substantially added to diagnostic accuracy and treatment decisions amongst mononeuropathies. In this study the literature on its clinical application in polyneuropathy is reviewed. Several polyneuropathies have been studied by means of ultrasound: Charcot–Marie–Tooth, hereditary neuropathy with liability to pressure palsies, chronic inflammatory demyelinating polyneuropathy, Guillain‐Barré syndrome, multifocal motor neuropathy, paraneoplastic polyneuropathy, leprosy and diabetic neuropathy. The most prominent reported pathological changes were nerve enlargement, increased hypo‐echogenicity and increased intraneural vascularization. Sonography revealed intriguingly different patterns of nerve enlargement between inflammatory neuropathies and axonal and inherited polyneuropathies. However, many studies concerned case reports or case series and showed methodological shortcomings. Further prospective studies with standardized protocols for nerve sonography and clinical and electrodiagnostic testing are needed to determine the role of nerve sonography in inherited and acquired polyneuropathies.     

Tracheal intubation with a camera embedded in the tube tip (Vivasight™)

We studied tracheal intubation in manikins and patients with a camera embedded in the tip of the tracheal tube (Vivasight^?). Four people in two teams and two individuals attempted intubation of a manikin through an i‐gel^? 10 times each. The tracheas of 12 patients with a Mallampati grade of 1 were intubated with a Vivasight tracheal tube through a Berman airway, passed over a Frova^? introducer. All 60 manikin intubations were successful, taking a mean (SD) time of 1.4 (0.5) s. The fastest intubation was performed in 0.5 s. All 12 participants’ tracheas were successfully intubated in a median (IQR [range]) time of 90 (70–120 [50–210]) s. Seven participants complained of a sore throat, comparable with earlier findings for standard laryngoscopy and intubation: five mild; one moderate; and one severe. Tracheal intubation with the Vivasight through the i‐gel or Berman airway is an alternative to existing techniques, against which it should be compared in randomised controlled trials in human participants. It has potential as a fast airway rescue technique.     

Tools in the Assessment of Sarcopenia

This review provides a framework for the development of an operational definition of sarcopenia and of the potential end points that might be adopted in clinical trials among older adults. While the clinical relevance of sarcopenia is widely recognized, there is currently no universally accepted definition of the disorder. The development of interventions to alter the natural history of sarcopenia also requires consensus on the most appropriate end points for determining outcomes of clinical importance which might be utilized in intervention studies. We review current approaches to the definition of sarcopenia and the methods used for the assessment of various aspects of physical function in older people. The potential end points of muscle mass, muscle strength, muscle power, and muscle fatigue, as well as the relationships between them, are explored with reference to the availability and practicality of the available methods for measuring these end points in clinical trials. Based on current evidence, none of the four potential outcomes in question is sufficiently comprehensive to recommend as a uniform single outcome in randomized clinical trials. We propose that sarcopenia may be optimally defined (for the purposes of clinical trial inclusion criteria as well as epidemiological studies) using a combination of measures of muscle mass and physical performance. The choice of outcome measures for clinical trials in sarcopenia is more difficult; co-primary outcomes, tailored to the specific intervention in question, may be the best way forward in this difficult but clinically important area.     

Congenital myopathy with focal loss of cross-striations revisited

In 1977 Wijngaarden et al. reported a Dutch family with a congenital myopathy characterized by external ophthalmoplegia and a remarkable histological feature, focal loss of cross-striations. A small number of other families with similar clinical and pathological features led to the consideration of this congenital myopathy as a distinct entity. Here we present more than 30 years of follow-up from the Dutch family and report recently identified compound heterozygous mutations in the skeletal muscle ryanodine receptor (RYR1) gene, c.10627-2A>G and p.Arg3539His (c.10616G>A). Focal loss of cross-striations on muscle biopsy is another histopathological feature that should raise the possibility of RYR1 involvement.     

Validity and suitability of the Bayley-III Low Motor/Vision version: A comparative study among young children with and without motor and/or visual impairments

The aim of the present study was to examine the validity of the Bayley-III Low Motor/Vision version, and its suitability for children with motor and/or visual impairment(s). This version contains accommodated items, that is, adaptations to minimize impairment bias, without altering what the test measures. We hypothesized that the accommodations would not affect the item scores of children without impairment, and that children with impairment(s) would benefit from the accommodations. We tested 41 children without impairment and 63 children with impairment with both the standard Bayley-III and the Low Motor/Vision versions, in randomly counterbalanced order. The test administrators filled in an evaluation form. Results showed that the accommodations did not affect the test scores of children without impairment and did improve the test scores of children with impairment on the Cognition scale, while no improvement was found for the other scales. The test administrators indicated that the vast majority of the children with impairment had been able to show their abilities on the test and that the accommodations were beneficial in 29 out of these 52 cases. For some children, the accommodated instrument appeared to be unsuitable because the impairment was too severe. The conclusion is that the accommodations improve the validity of the Bayley-III when used with children with mild to moderate motor and/or visual impairment, especially with regard to the Cognition scale.