Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease type IIIa are phenotype-specific: An investigator-initiated, randomized, crossover study.

Glycogen storage disease type IIIa (GSDIIIa) is an inborn error of carbohydrate metabolism caused by a debranching enzyme deficiency. A subgroup of GSDIIIa patients develops severe myopathy. The purpose of this study was to investigate whether acute nutritional ketosis (ANK) in response to ketone-ester (KE) ingestion is effective to deliver oxidative substrate to exercising muscle in GSDIIIa patients.

Compensatory Movement Patterns Are Based on Abnormal Activity of the Biceps Brachii and Posterior Deltoid Muscles in Patients with Symptomatic Rotator Cuff Tears.

Background: Abnormal movement patterns due to compensatory mechanisms have been reported in patients with rotator cuff tears. The long head of the biceps tendon may especially be overactive and a source of pain and could induce abnormal muscle activation in these patients. It is still unknown why some patients with a rotator cuff tear develop complaints and others do not.

Development of muscle ultrasound density in healthy fetuses and infants.

Muscle ultrasound density (MUD) is a non-invasive parameter to indicate neuromuscular integrity in both children and adults. In healthy fetuses and infants, physiologic MUD values during development are still lacking. We therefore aimed to determine the physiologic, age-related MUD trend of biceps, quadriceps, tibialis anterior, hamstrings, gluteal and calf muscles, from pre- to the first year of postnatal life.

Long-term obstructive sleep apnea therapy: a 10-year follow-up of mandibular advancement device and continuous positive airway pressure.

Study Objectives: Obstructive sleep apnea (OSA) is a sleep-related breathing disorder, commonly managed by either continuous positive airway pressure (CPAP) or a mandibular advancement device (MAD). Long-term follow-up and comparison regarding efficacy of these therapies is scarce. In this study the results of treatment, patient adherence, and satisfaction over a 10-year follow-up of these therapies are reported.

Dental side effects of long-term obstructive sleep apnea therapy: a 10-year follow-up study.

Objectives: Patients with obstructive sleep apnea (OSA) are usually treated with either mandibular advancement device (MAD) or continuous positive airway pressure (CPAP) therapy. The objective of this study is to evaluate changes in dental occlusion associated with long-term MAD and CPAP therapy.

Materials And Methods: Data from 14 OSA patients using MAD and 17 OSA patients using CPAP therapy were evaluated at baseline, 2-year and 10-year follow-up.

The importance of the intensive care unit environment in sleep-A study with healthy participants.

Sleep disruption is common among intensive care unit patients, with potentially detrimental consequences. Environmental factors are thought to play a central role in ICU sleep disruption, and so it is unclear why environmental interventions have shown limited improvements in objectively assessed sleep. In critically ill patients, it is difficult to isolate the influence of environmental factors from the varying contributions of non-environmental factors.

Clinical- and Cost-Effectiveness of a Mandibular Advancement Device Versus Continuous Positive Airway Pressure in Moderate Obstructive Sleep Apnea.

Study Objectives: Limited evidence exists on the cost-effectiveness of mandibular advancement device (MAD) compared to continuous positive airway pressure (CPAP) therapy in moderate obstructive sleep apnea (OSA). Therefore, this study compares the clinical and cost-effectiveness of MAD therapy with CPAP therapy in moderate OSA.

Methods: In a multicentre randomized controlled trial, patients with an apnea-hypopnea index (AHI) of 15 to 30 events/h were randomized to either MAD or CPAP.

Leg muscle strength is reduced and is associated with physical quality of life in Antineutrophil cytoplasmic antibody-associated vasculitis.

Objective: Physical quality of life is reduced in ANCA-associated vasculitis (AAV). This study aims to investigate whether this may be explained by reduced muscle strength and physical activity resulting from disease damage and steroid myopathy.

Methods: Forty-eight AAV patients were sequentially included from the outpatient clinic.

Lack of analgesic effects of transcranial pulsed electromagnetic field stimulation in neuropathic pain patients: A randomized double-blind crossover trial.

Background: Neuromodulation is nowadays investigated as a promising method for pain relief. Research indicates that a single 30-minute stimulation with transcranial pulsed electromagnetic fields (tPEMF) can induce analgesic effects. However, it is unknown whether tPEMF can induce analgesia in neuropathic pain patients.

Construct Validity and Reliability of the SARA Gait and Posture Sub-scale in Early Onset Ataxia.

In children, gait and posture assessment provides a crucial marker for the early characterization, surveillance and treatment evaluation of early onset ataxia (EOA). For reliable data entry of studies targeting at gait and posture improvement, uniform quantitative biomarkers are necessary. Until now, the pediatric test construct of gait and posture scores of the Scale for Assessment and Rating of Ataxia sub-scale (SARA) is still unclear.

Cognitive deficits after aneurysmal and angiographically negative subarachnoid hemorrhage: Memory, attention, executive functioning, and emotion recognition.

Objective: The authors' aim was to investigate cognitive outcome in patients with aneurysmal and angiographically negative subarachnoid hemorrhage (aSAH and anSAH), by comparing them to healthy controls and to each other. Besides investigating cognitive functions as memory and attention, they focused on higher-order prefrontal functions, namely executive functioning (EF) and emotion recognition.

Method: Patients and healthy controls were assessed with tests measuring memory (15 Words Test, Digit Span), attention and processing speed (Trail Making Test A and B), EF (Zoo Map, Letter Fluency, Dysexecutive Questionnaire), and emotion recognition (Facial Expressions of Emotion Stimuli and Tests).

Tremor Detection Using Parametric and Non-Parametric Spectral Estimation Methods: A Comparison with Clinical Assessment.

In the clinic, tremor is diagnosed during a time-limited process in which patients are observed and the characteristics of tremor are visually assessed. For some tremor disorders, a more detailed analysis of these characteristics is needed. Accelerometry and electromyography can be used to obtain a better insight into tremor.

[How to deal with blast injuries. 15 tips for healthcare providers].

The recent terror attacks in Paris and Brussels have made the subject of injuries caused by explosives, also known as 'blast injuries', a very current one. The Netherlands has limited experience with terrorist attacks. This means that Dutch medical care providers possibly do not have sufficient knowledge about dealing with blast injuries.

Muscle Ultrasound in Patients with Glycogen Storage Disease Types I and III.

In glycogen storage diseases (GSDs), improved longevity has resulted in the need for neuromuscular surveillance. In 12 children and 14 adults with the "hepatic" (GSD-I) and "myopathic" (GSD-III) phenotypes, we cross-sectionally assessed muscle ultrasound density (MUD) and muscle force. Children with both "hepatic" and "myopathic" GSD phenotypes had elevated MUD values (MUD Z-scores: GSD-I > 2.

Validity and Predictive Value of a Portable Two-Channel Sleep-Screening Tool in the Identification of Sleep Apnea in Patients With Heart Failure.

Background: Sleep apnea is an important comorbidity in heart failure (HF) and is associated with an adverse outcome. Diagnosing sleep apnea is difficult, and polysomnography, considered to be the criterion standard, is not widely available. We assessed the validity of a portable 2-channel sleep-screening tool for the identification of sleep apnea in patients with HF.

Usage of positional therapy in adults with obstructive sleep apnea.

Study Objectives: Many positional therapy (PT) strategies are available for treating positional obstructive sleep apnea (OSA). PT is primarily supplied to selected patients as a secondary treatment option when other therapies have failed. To our knowledge this is the largest study to date to assess effectiveness and long-term compliance of PT (both commercial waistband and self-made constructions, mimicking the tennis ball technique) as primary treatment in patients with different positional OSA severities.

Visual screening of muscle ultrasound images in children.

In children, non-invasive muscle ultrasound (MU) imaging has become increasingly important for the detection of neuromuscular pathology, by either quantitative or visual assessment. MU quantification requires time, expertise and equipment. If application of visual MU screening provides reliable results, ubiquitous application could be advocated.

Intensive care unit depth of sleep: proof of concept of a simple electroencephalography index in the non-sedated.

Introduction: Intensive care unit (ICU) patients are known to experience severely disturbed sleep, with possible detrimental effects on short- and long- term outcomes. Investigation into the exact causes and effects of disturbed sleep has been hampered by cumbersome and time consuming methods of measuring and staging sleep. We introduce a novel method for ICU depth of sleep analysis, the ICU depth of sleep index (IDOS index), using single channel electroencephalography (EEG) and apply it to outpatient recordings.

Muscle ultrasound quantifies segmental neuromuscular outcome in pediatric myelomeningocele.

In pediatric spina bifida aperta (SBA), non-invasive assessment of neuromuscular integrity by muscle ultrasound density (MUD) could provide important information about the clinical condition. We therefore aimed to determine the association between pediatric SBA MUD and segmental neurologic function. We included 23 children (age range: 1-18 y) with SBA with L4-5 lesions, and we associated SBA MUD with control values and segmental neuromuscular function.

Oral appliance versus continuous positive airway pressure in obstructive sleep apnea syndrome: a 2-year follow-up.

Study Objectives: Oral appliance therapy has emerged as an important alternative to continuous positive airway pressure (CPAP) in treating patients with obstructive sleep apnea syndrome (OSAS). In this study we report about the subjective and objective treatment outcome of oral appliance therapy and CPAP in patients with OSAS.

Design: Cohort study of a previously conducted randomized clinical trial.

A novel magnetic stimulator increases experimental pain tolerance in healthy volunteers - a double-blind sham-controlled crossover study.

Unlabelled: The 'complex neural pulse'(TM) (CNP) is a neuromodulation protocol employing weak pulsed electromagnetic fields (PEMF). A pioneering paper reported an analgesic effect in healthy humans after 30 minutes of CNP-stimulation using three nested whole head coils. We aimed to devise and validate a stimulator with a novel design entailing a multitude of small coils at known anatomical positions on a head cap, to improve applicability.