Transitioning outcome measures: relationship between the CMTPedS and CMTNSv2 in children,adolescents, and young adults with Charcot‐Marie‐Tooth disease

Long‐term studies of Charcot‐Marie‐Tooth (CMT) disease across the entire lifespan require stable endpoints that measure the same underlying construct (e.g., disability). The aim of this study was to assess the relationship between the CMT Pediatric Scale (CMTPedS) and the adult CMT Neuropathy Score (CMTNSv2) in 203 children, adolescents, and young adults with CMT. There was a moderate curvilinear correlation between the CMTPedS and the CMTNSv2 (Spearman's rho ρ = 0.716, p < 0.0001), although there appears to be a floor effect of the CMTNSv2 in patients with a milder CMT phenotype. Univariate analyses indicate that the relationship between the CMTPedS and CMTNSv2 scores improves with worsening disease severity and advancing age. Although one universal scale throughout life would be ideal, our data supports the transition from the CMTPedS in childhood to the CMTNSv2 in adulthood as a continuum of measuring lifelong disability in patients with CMT.     

Changes in oro-facial function and hand-grip strength during a 2-year observation period after stroke

Objectives

Recovery from stroke-related oro-facial impairment has rarely been investigated. In this longitudinal study chewing efficiency, maximum lip and bite force as well as masseter muscle thickness were evaluated and compared with hand-grip force.

Materials and methods

Thirty-one hospitalized stroke patients and 24 controls were recruited for this cohort study with 2-year follow-up. Chewing efficiency was evaluated with a color mixing ability test, lip forces with a traction dynamometer, bite force with a digital gauge, masseter muscle thickness using ultrasound measurements and grip strength with a hand dynamometer.

Results

During the 2-year observation period, patients were evaluated four times. A total of 21 patients dropped out of the study. Stroke patients showed significantly impaired chewing efficiency and lower lip forces than controls with no significant improvement over time. Bite forces were not different between ipsi- and contralesional sides, in contrast to contralesional hand-grip strength which was significantly impaired and did not improve during the observation period. On the first examination with a median of 40 days after stroke, masseter thickness was reduced contralesional, but did not continue to show significant side-differences during follow-up.

Conclusions

Stroke affects the upper limb and the masseter muscles differently on a functional and morphological level. Further research is needed to evaluate the predictive value of oro-facial parameters on functional outcome after stroke.

Clinical relevance

Impaired chewing efficiency and reduced lip force are quantifiable symptoms in stroke patients which seem not to improve in absence of oro-facial rehabilitation procedures.     

Assessing Decreased Sensation and Increased Sensory Phenomena in Diabetic Polyneuropathies

Loss of sensation and increased sensory phenomena are major expressions of varieties of diabetic polyneuropathies needing improved assessments for clinical and research purposes. We provide a neurobiological explanation for the apparent paradox between decreased sensation and increased sensory phenomena. Strongly endorsed is the use of the 10-g monofilaments for screening of feet to detect sensation loss, with the goal of improving diabetic management and prevention of foot ulcers and neurogenic arthropathy. We describe improved methods to assess for the kind, severity, and distribution of both large- and small-fiber sensory loss and which approaches and techniques may be useful for conducting therapeutic trials. The abnormality of attributes of nerve conduction may be used to validate the dysfunction of large sensory fibers. The abnormality of epidermal nerve fibers/1 mm may be used as a surrogate measure of small-fiber sensory loss but appear not to correlate closely with severity of pain. Increased sensory phenomena are recognized by the characteristic words patients use to describe them and by the severity and persistence of these symptoms. Tests of tactile and thermal hyperalgesia are additional markers of neural hyperactivity that are useful for diagnosis and disease management.Altered sensation (loss or increased sensory phenomena) may be early and prominent manifestations of varieties of polyneuropathy associated with diabetes. These neuropathies may be classified into four major varieties: distal symmetric sensorimotor polyneuropathies (typical and atypical diabetic sensorimotor polyneuropathy [DSPN]); compression and entrapment varieties (median neuropathy at the wrist [carpal tunnel syndrome]); radiculoplexus neuropathies (lumbosacral [Bruns Garland syndrome], thoracic, and cervical); and cranial neuropathies (1–3). Although none of these varieties are uniquely associated with DM, all varieties are more prevalent in diabetes. Underlying mechanisms are different among these varieties (1–3).Decreased sensation and increased sensory phenomena are not being adequately evaluated in clinical medicine. Possible reasons include the following: 1) methodologies of such assessments are not sufficiently emphasized in training of health care professionals; 2) insufficient time is taken in their evaluation (i.e., to assess kind, severity, and distribution of sensation loss, let alone to assess increased sensory phenomena); 3) reference values are often not available or used; 4) standard techniques of assessment are typically not used, for example, to assess clinical sensation with cotton wool, disposable stick pins, tuning forks, or other; 5) validated quantitative sensation tests (QSTs) are generally not used; and 6) compensation for such testing is unavailable.Here we review the neurobiology underlying decreased and increased sensory phenomena occurring in diabetic polyneuropathies (DPNs) and methodologies of their assessment. Especially emphasized in this review are improved methods to screen for sensation loss of feet, with the goal of preventing ulcers and neurogenic arthropathy; use of composite scores of neuropathic signs; computer-assisted (smart) QSTs; nerve conduction (NC) measurements; and counts of intraepidermal nerve fibers as neuropathy end points for therapeutic trials of DPN severity. Also described are measures of increased sensory phenomena.     

Guía clínica de la Asociación Europea de Urología sobre tecnologías láser

ContextThe European Association of Urology (EAU) Guidelines Office has set up a guideline working panel to analyse the scientific evidence published in the world literature on lasers in urologic practice.ObjectiveReview the physical background and physiologic and technical aspects of the use of lasers in urology, as well as current clinical results from these new and evolving technologies, together with recommendations for the application of lasers in urology. The primary objective of this structured presentation of the current evidence base in this area is to assist clinicians in making informed choices regarding the use of lasers in their practice.Evidence acquisitionStructured literature searches using an expert consultant were designed for each section of this document. Searches were carried out in the Cochrane Database of Systematic Reviews, the Cochrane Central Register of Controlled Trials, and Medline and Embase on the Dialog/DataStar platform. The controlled terminology of the respective databases was used, and both Medical Subject Headings and EMTREE were analysed for relevant entry terms. One Cochrane review was identified.Evidence synthesisDepending on the date of publication, the evidence for different laser treatments is heterogeneous. The available evidence allows treatments to be classified as safe alternatives for the treatment of bladder outlet obstruction in different clinical scenarios, such as refractory urinary retention, anticoagulation, and antiplatelet medication. Laser treatment for bladder cancer should only be used in a clinical trial setting or for patients who are not suitable for conventional treatment due to comorbidities or other complications. For the treatment of urinary stones and retrograde endoureterotomy, lasers provide a standard tool to augment the endourologic procedure.ConclusionsIn benign prostatic obstruction (BPO), laser vaporisation, resection, or enucleation are alternative treatment options. The standard treatment for BPO remains transurethral resection of the prostate for small to moderate size prostates and open prostatectomy for large prostates. Laser energy is an optimal treatment method for disintegrating urinary stones. The use of lasers to treat bladder tumours and in laparoscopy remains investigational.     

The society for cardiovascular angiography and interventions structural heart disease early career task force survey results: Endorsed by the society for cardiovascular angiography and interventions

Over the last decade, structural heart disease interventions have emerged as a new field in interventional cardiology. Currently, the Accreditation Council for Graduate Medical Education accredited interventional cardiology fellowship programs in the United States provide high‐quality and well established training curriculum in coronary and peripheral interventions, but training in structural interventions remains in its infancy. The current survey seeks to collect relevant information and assess the opinion of interventional cardiology program directors in ACGME‐accredited institutions that are actively involved in structural interventional training. Our study describes the actual number of structural procedures performed by interventional cardiology fellows in ACGME‐accredited programs, the form of the structural training today and the suggestions from program directors who are actively trying to integrate structural training in the interventional cardiology fellowship programs. © 2012 Wiley Periodicals, Inc.