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In patients with mitochondrial disease, fatigue and muscle problems are the most common complaints. They also experience these complaints during mastication. To measure endurance of continuous mastication in patients with mitochondrial diseases, the 6-min mastication test (6MMT) was developed. This study included the collection of normal data for the 6MMT in a healthy population (children and adults). During 6 min of continuous mastication on a chew tube chewing cycles per minute, total amount of chewing cycles and the difference between minute 1 (M 1 ) and minute 6 (M 2 ) were collected in 271 healthy participants (5-80 years old). These results were compared with those of nine paediatric and 25 adult patients with a mitochondrial disease. Visual analogue scale (VAS) scores were collected directly after the test and after 5 min. A qualitative rating was made on masticatory movements. The reproducibility of the 6MMT in the healthy population with an interval of approximately 2 weeks was good. The inter-rater reliability for the observations was excellent. The patient group demonstrated lower total amount of chewing cycles or had greater differences between M 1 and M 6 . The 6MMT is a reliable and objective test to assess endurance of continuous chewing. It demonstrates the ability of healthy children and adults to chew during 6 min with a highly stable frequency of mastication movements. The test may give an explanation for the masticatory problems in patient groups, who are complaining of pain and fatigue during mastication., (© 2017 John Wiley & Sons Ltd.)

In this review we provide a current overview of the clinical features, pathophysiology, epidemiology, and diagnostic and therapeutic strategies in neuralgic amyotrophy (NA). The disorder has several phenotypic variations, with a classic form in 70% of the patients. It is not rare, with an incidence of 1 per 1,000 individuals, but it is still often missed. Recurrences are common, yet the proposed multifactorial etiology, which includes genetic, biomechanical, and immunologic factors, limits our capacity to predict or prevent them. NA is a clinical diagnosis, and ancillary studies serve to exclude infectious or malignant causes or to assess a differential diagnosis. If patients are seen early and are still in pain, a short trial of high-dose oral corticosteroids is advised, and adequate analgesia may require opioids and non-steroidal anti-inflammatory drugs. Persistent complaints are common, and a multidisciplinary rehabilitation approach focusing on scapular coordination, energy distribution strategies, and self-management is indicated., (© 2015 Wiley Periodicals, Inc.)

Objective: Neuralgic amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic amyotrophy in a primary care setting., Methods: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic amyotrophy. Neuralgic amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation., Results: Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic amyotrophy of 1 per 1000., Conclusions: Our findings suggest that neuralgic amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

Background: Chronic fatigue is present in more than 60% of the patients with a neuromuscular disease and can be their most disabling symptom. In combination with other impairments, fatigue often results in low levels of physical activity and decreased social participation, leading to high societal costs. 'Energetic' is a self-management group program aimed at improving social participation, physical endurance and alleviating fatigue in these patients. The primary aim of this study is to evaluate the effectiveness and cost-effectiveness of the Energetic program., Methods/design: A multicentered, assessor-blinded, two-armed randomized controlled trial is conducted with evaluations at inclusion and four, seven and fifteen months later. The study includes patients with a neuromuscular disease and chronic fatigue and, when present, their caregivers. The participants are randomized (ratio 1:1) to either an intervention group, receiving the Energetic program, or a control group, receiving usual care (i.e., no specific intervention). The Energetic program covers four months and includes four modules: 1) individually tailored aerobic exercise training; 2) education about aerobic exercise; 3) self-management training in applying energy conservation strategies; and 4) implementation and relapse prevention in daily life. Two months after cessation of the program a booster session is provided. The primary outcome is the perceived performance score of the Canadian Occupational Performance Measure (COPM). Secondary outcomes include the COPM-satisfaction score, and measures of fatigue, physical endurance, activity engagement, mood, and self-efficacy. Caregiver burden is also evaluated as a secondary outcome. Health-related quality of life and medical and societal costs are assessed to estimate cost-effectiveness of the program., Discussion: The Energetic study is the first randomized controlled trial to evaluate the effectiveness and cost-effectiveness of a combined physical and self-management group training program for improving social participation, physical endurance and alleviating fatigue in patients with neuromuscular diseases. It will generate new insights in (cost-)effective rehabilitation strategies for these incurable conditions., Trial Registration: Clinicaltrials.gov NCT02208687 .

Objective: To assess the incidence of classic neuralgic amyotrophy (NA) in a primary care setting., Design: Prospective cohort study., Method: During the year 2012 we registered all new cases of neck, shoulder or arm symptoms from two large primary care centres serving a population of 14,118. Prior to commencing the study, general practitioners attended a short training session on how to diagnose classic NA. After inclusion, patients in whom general practitioners suspected NA were offered a neurologic assessment for diagnostic confirmation., Results: Of the 492 patients identified with new onset of neck, shoulder or arm symptoms, 34 were suspected of having NA. Neurologic evaluation confirmed the diagnosis in 14 patients. For the Netherlands, this translates to an incidence of 1 in 1,000., Conclusion: Our findings suggest that NA is 30-50 times more common than previously thought; in the Netherlands, this would mean 17,000 instead of 500 new cases each year. Lack of awareness of the disorder and its clinical presentation seems the most likely explanation for this difference. Since NA may lead to sustained symptoms and functional limitations in the chronic phase, increased attention is urgently required to improve diagnostics and treatment.

Background: Individuals with a spinal cord injury (SCI) demonstrate altered circadian variation in thermoregulatory control. Recently, we reported that tetraplegia is associated with a blunted release of melatonin in the evening. In order to examine whether this finding relates to circadian thermoregulation, we compared the correlations between evening changes in melatonin, core and skin temperature between thoracic and cervical SCI and able-bodied participants., Methods: In 10 able-bodied, 9 paraplegic and 8 tetraplegic participants, we measured, between 1900 and 2300 h, core temperature, proximal skin temperature (above and below the level of the lesion) and physical activity. Salivary melatonin was also sampled during this period and analyzed using enzyme linked immunosorbant assay., Results: Between 1900 and 2300 h, core and upper limb skin temperature gradually decreased in all groups (p = 0.01). A significant group × time interaction was evident in lower body skin temperature (p = 0.03). Lower body skin temperature was significantly higher in able-bodied controls compared with tetraplegics between 1900 and 2000 h (p < 0.05). In able-bodied and paraplegic participants, the changes in melatonin and core temperature were inversely correlated (r = -0.44 and -0.54, respectively, both p = 0.01). Melatonin and mean skin temperature changes were also inversely correlated (able-bodied controls: r = -0.24; p = 0.05 and paraplegics: r = -0.30; p= 0.02)., Conclusion: The inverse correlation between evening changes in melatonin and thermoregulation is of a similar magnitude in paraplegic and able-bodied controls. In contrast, changes in skin temperature, below the level of the lesion, are unrelated to changes in melatonin in tetraplegics.

Background: Neuralgic Amyotrophy (NA) is characterized by neuropathic pain, subsequent patchy paresis and possible sensory loss in the upper extremity. Many patients experience difficulties in performing activities of daily life and are unable to resume work. We developed a combined physical- and occupational therapy program for patients recovering from NA., Objective: Evaluation of the effectiveness of a multidisciplinary intervention program for patients with subacute NA., Methods: We performed a within subject proof-of-principle pilot study in eight patients with subacute NA. Patients followed 8 hours of physical and 8 hours of occupational therapy spread over a 16-week period., Primary Outcome Measures: The Canadian Occupational Performance Measure (COPM) and Shoulder Rating Questionnaire (SRQ)., Secondary Outcome Measure: Disability of Arm Shoulder and Hand (DASH)., Results: Improvements (mean (95% CI)) were found in the performance and satisfaction scores of the COPM +2.3 (0.9-3.7) and +1.4 (0.4-2.4) points, respectively and the SRQ +14.8 (7.4-22.0) points. The majority of patients (6 out of 8) also demonstrated improvements in the DASH., Conclusion: The proposed physical and occupational therapy program, may be effective for patients with subacute NA, as demonstrated by improvements in activity, performance and participation.

Individuals with a spinal cord injury (SCI), especially with tetraplegia, experience poor sleep quality, and this may be related to impaired control of circadian rhythmicity. Here, we examined the evening onset of melatonin secretion, an important hormone for the initiation of sleep, in people with a complete cervical (tetraplegia) and thoracic (paraplegia) SCI, and age- and sex-matched able-bodied control participants. Multiple samples of salivary melatonin were obtained during the evening hours and analyzed by ELISA methods in 10 control partcipants, 9 individuals with paraplegia, and 6 individuals with tetraplegia. Sleep quality was assessed using questionnaires. Interactive effects of group and time were found for melatonin levels (P=0.022). In the control and paraplegia groups, the mean melatonin level increased significantly from 2.59 ± 1.04 and 4.28 ± 3.28 pg/ml at 7 PM to 10.62 ± 4.59 and 13.10 ± 7.39 pg/ml at 11 PM, respectively (P<0.001). In the tetraplegia group, melatonin level was 5.25 ± 3.72 at 7 PM but only 2.41 ± 1.25 pg/ml at 11 PM (P>0.05). Decreased sleep quality was more prevalent in individuals with tetraplegia (83%) and paraplegia (75%) compared with controls (20%; P=0.02). Unlike in the control and paraplegia groups, the evening increase in melatonin concentration was completely absent in the tetraplegia group. This provides biological insight into sleep regulation in humans and provides better understanding of the poor sleep quality in people with tetraplegia.

Background: Patients with Parkinson's disease (PD) are physically less active than controls, and autonomic dysfunction may contribute to this sedentary lifestyle. Specifically, an altered cardiovascular response to physical effort may restrict physical activities., Objective: To assess the cardiovascular responses to a submaximal exercise test in PD patients and controls, 546 sedentary PD patients and 29 sedentary healthy controls performed the Åstrand-Rhyming submaximal cycle exercise test., Methods: Average heart rate was used to estimate maximal oxygen consumption (VO2max). Variables that may affect submaximal activity in PD patients, including disease severity, fatigue, and level of physical activity in daily life, were recorded., Results: Fewer PD patients (46%) completed the submaximal exercise test successfully than the controls (86%). The estimated VO2max of patients with a successful test was 34% lower than the controls (p < 0.001). Multivariate regression analyses revealed that higher body weight, lower systolic blood pressure, lower resting heart rate, and lower maximal workload were associated with an increased risk of an inadequate heart rate increase during submaximal exercise (R2 = 27%). PD patients with a successful submaximal exercise test had lower estimated VO2max values than controls., Conclusions: Importantly, half of the PD patients had an inadequate heart rate increase during submaximal exercise, which was likely caused by cardiac sympathetic denervation leading to autonomic dysfunction. PD patients should therefore be screened to identify their limitations in exercise performance. Caution should be applied when prescribing beta blockers, as they might limit physical activities further.

The pathophysiology of orthostatic hypotension in Parkinson's disease (PD) is incompletely understood. The primary focus has thus far been on failure of the baroreflex, a central mediated vasoconstrictor mechanism. Here, we test the role of two other possible factors: 1) a reduced peripheral vasoconstriction (which may contribute because PD includes a generalized sympathetic denervation); and 2) an inadequate plasma volume (which may explain why plasma volume expansion can manage orthostatic hypotension in PD). We included 11 PD patients with orthostatic hypotension (PD + OH), 14 PD patients without orthostatic hypotension (PD - OH), and 15 age-matched healthy controls. Leg blood flow was examined using duplex ultrasound during 60° head-up tilt. Leg vascular resistance was calculated as the arterial-venous pressure gradient divided by blood flow. In a subset of 9 PD + OH, 9 PD - OH, and 8 controls, plasma volume was determined by indicator dilution method with radiolabeled albumin ((125)I-HSA). The basal leg vascular resistance was significantly lower in PD + OH (0.7 ± 0.3 mmHg·ml(-1)·min) compared with PD - OH (1.3 ± 0.6 mmHg·ml(-1)·min, P < 0.01) and controls (1.3 ± 0.5 mmHg·ml(-1)·min, P < 0.01). Leg vascular resistance increased significantly during 60° head-up tilt with no significant difference between the groups. Plasma volume was significantly larger in PD + OH (3,869 ± 265 ml) compared with PD - OH (3,123 ± 377 ml, P < 0.01) and controls (3,204 ± 537 ml, P < 0.01). These results indicate that PD + OH have a lower basal leg vascular resistance in combination with a larger plasma volume compared with PD - OH and controls. Despite the increase in leg vascular resistance during 60° head-up tilt, PD + OH are unable to maintain their blood pressure.

Maintaining blood pressure during orthostatic challenges is primarily achieved by baroreceptor-mediated activation of the sympathetic nervous system, which can be divided into preganglionic and postganglionic parts. Despite their preganglionic autonomic failure, spinal cord-injured individuals demonstrate a preserved peripheral vasoconstriction during orthostatic challenges. Whether this also applies to patients with postganglionic autonomic failure is unknown. Therefore, we assessed leg vasoconstriction during 60° head-up tilt in five patients with pure autonomic failure (PAF) and two patients with autonomic failure due to dopamine-β-hydroxylase (DBH) deficiency. Ten healthy subjects served as controls. Leg blood flow was measured using duplex ultrasound in the right superficial femoral artery. Leg vascular resistance was calculated as the arterial-venous pressure gradient divided by blood flow. DBH-deficient patients were tested off and on the norepinephrine pro-drug l-threo-dihydroxyphenylserine (l-DOPS). During 60° head-up tilt, leg vascular resistance increased significantly in PAF patients [0.40 ± 0.38 (+30%) mmHg·ml(-1)·min(-1)]. The increase in leg vascular resistance was not significantly different from controls [0.88 ± 1.04 (+72%) mmHg·ml(-1)·min(-1)]. In DBH-deficient patients, leg vascular resistance increased by 0.49 ± 0.01 (+153%) and 1.52 ± 1.47 (+234%) mmHg·ml(-1)·min(-1) off and on l-DOPS, respectively. Despite the increase in leg vascular resistance, orthostatic hypotension was present in PAF and DBH-deficient patients. Our results demonstrate that leg vasoconstriction during orthostatic challenges in patients with PAF or DBH deficiency is not abolished. This indicates that the sympathetic nervous system is not the sole or pivotal mechanism inducing leg vasoconstriction during orthostatic challenges. Additional vasoconstrictor mechanisms may compensate for the loss in sympathetic nervous system control.

Autonomic dysreflexia is a hypertensive episode in spinal cord-injured individuals induced by exaggerated sympathetic activity and thought to be alpha-adrenergic mediated. alpha-Adrenoceptor antagonists have been a rational first choice; nevertheless, calcium channel blockers are primarily used in autonomic dysreflexia management. However, alpha-adrenoceptor blockade may leave a residual vasoconstrictor response to sympathetic nonadrenergic transmission unaffected. The aim was to assess the alpha-adrenergic contribution and, in addition, the role of supraspinal control to leg vasoconstriction during exaggerated sympathetic activity provoked by autonomic dysreflexia in spinal cord-injured individuals and by a cold pressure test in control individuals. Upper leg blood flow was measured using venous occlusion plethysmography during supine rest and during exaggerated sympathetic activity in 6 spinal cord-injured individuals and 7 able-bodied control individuals, without and with phentolamine (alpha-adrenoceptor antagonist) and nicardipine (calcium channel blocker) infusion into the right femoral artery. Leg vascular resistance was calculated. In spinal cord-injured individuals, phentolamine significantly reduced the leg vascular resistance increase during autonomic dysreflexia (8+/-5 versus 24+/-13 arbitrary units; P=0.04) in contrast to nicardipine (15+/-10 versus 24+/-13 arbitrary units; P=0.12). In controls, phentolamine completely abolished the leg vascular resistance increase during a cold pressure test (1+/-2 versus 18+/-14 arbitrary units; P=0.02). The norepinephrine increase during phentolamine infusion was larger (P=0.04) in control than in spinal cord-injured individuals. These results indicate that the leg vascular resistance increase during autonomic dysreflexia in spinal cord-injured individuals is not entirely alpha-adrenergic mediated and is partly explained by nonadrenergic transmission, which may, in healthy subjects, be suppressed by supraspinal control.

Background: orthostatic hypotension is common in older men and associated with morbidity and mortality. During orthostatic challenges, older men maintain their blood pressure by an augmented increase in total peripheral resistance. Changes in the leg vascular bed contribute importantly to blood pressure regulation during orthostatic challenges, partly because of blood pooling in the legs. Little is known about the contribution of the leg vascular bed to the augmented increase in total peripheral resistance., Objective: to examine tilt-induced peripheral vasoconstriction in the leg vascular bed of young and older men., Methods: we measured forearm and calf blood flow in 12 young and 12 older men, using venous occlusion plethysmography during 30 degrees head-up tilt (HUT). Forearm and calf vascular resistance were calculated as mean arterial blood pressure divided by blood flow., Results: during HUT, calf and forearm vascular resistance increased in older and young men. The increase in forearm vascular resistance was similar between older (40 +/- 6%) and young men (51 +/- 12%). However, the increase in calf vascular resistance was lower in older (96 +/- 15%) than in young men (175 +/- 30%)., Conclusion: advancing age leads to an attenuated tilt-induced increase in calf vascular resistance, which may contribute to age-related orthostatic hypotension.

Leg vascular resistance is calculated as the arterial-venous pressure gradient divided by blood flow. During orthostatic challenges it is assumed that the hydrostatic pressure contributes equally to leg arterial, as well as to leg venous pressure. Because of venous valves, one may question whether, during orthostatic challenges, a continuous hydrostatic column is formed and if leg venous pressure is equal to the hydrostatic pressure. The purpose of this study was, therefore, to measure intravenous pressure in the great saphenous vein of 12 healthy individuals during 30 degrees and 70 degrees head-up tilt and compare this with the calculated hydrostatic pressure. The height difference between the heart and the right medial malleolus level represented the hydrostatic column. The results demonstrate that there were no differences between the measured intravenous pressure and the calculated hydrostatic pressure during 30 degrees (47.2 +/- 1.0 and 46.9 +/- 1.5 mmHg, respectively) and 70 degrees head-up tilt (83.9 +/- 0.9 and 85.1 +/- 1.2 mmHg, respectively). Steady-state levels of intravenous pressure were reached after 95 +/- 12 s during 30 degrees and 161 +/- 15 s during 70 degrees head-up tilt. In conclusion, the measured leg venous pressure is similar to the calculated hydrostatic pressure during orthostatic challenges. Therefore, the assumption that hydrostatic pressure contributes equally to leg arterial as well as to leg venous pressure during orthostatic challenges can be made.

Objective: To report a rare cause of spinal cord injury., Study Design: Case report., Case Report: A 36-year-old woman presented with acute onset of paresis of the upper and lower extremity (level C5, ASIA B) the day after delivering a healthy daughter (39 weeks' gestation). Prior to giving birth, she was admitted with gestational hypertension. Directly postpartum, blood pressure increased markedly, and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome was diagnosed. The next day, tetraplegia developed. Magnetic resonance imaging showed multiple epidural and subdural hematomas in the cervical and thoracic spinal canal extending from C2 to T8, with displacement and compression of the cord. Three weeks after treatment with intravenous labetalol, she made a significant recovery (level T1, ASIA D)., Conclusions: This rare cause of spinal cord injury shows the importance of closely monitoring pregnant patients with hypertension complicated with HELLP syndrome both pre- and postpartum.

In patients with orthostatic intolerance, the mechanisms to maintain BP (blood pressure) fail. A physical counter-manoeuvre to postpone or even prevent orthostatic intolerance in these patients is leg crossing combined with muscle tensing. Although the central haemodynamic effects of physical counter-manoeuvres are well documented, not much is known about the peripheral haemodynamic events. Therefore the purpose of the present study was to examine the peripheral haemodynamic effects of leg crossing combined with muscle tensing during 70 degrees head-up tilt. Healthy subjects (n=13) were monitored for 10 min in the supine position followed by 10 min in 70 degrees head-up tilt and, finally, for 2 min of leg crossing with muscle tensing in 70 degrees head-up tilt. MAP (mean arterial BP), heart rate, stroke volume, cardiac output and total peripheral resistance were measured continuously by Portapres. Leg blood flow was measured using Doppler ultrasound. Leg vascular conductance was calculated as leg blood flow/MAP. A significant increase in MAP (13 mmHg), stroke volume (27%) and cardiac output (18%), a significant decrease in heart rate (-5 beats/min) and no change in total peripheral resistance during the physical counter-manoeuvre were observed when compared with baseline 70 degrees head-up tilt. A significant increase in leg blood flow (325 ml/min) and leg vascular conductance (2.9 arbitrary units) were seen during the physical counter-manoeuvre when compared with baseline 70 degrees head-up tilt. In conclusion, the present study indicates that the physical counter-manoeuvre of leg crossing combined with muscle tensing clearly enhances leg blood flow and, at the same time, elevates MAP.

Despite loss of centrally mediated sympathetic vasoconstriction to the legs, spinal cord-injured individuals cope surprisingly well with an orthostatic challenge. This study assessed changes in leg vascular resistance following head-up tilt in healthy (C) and in paraplegic (P) individuals. After 10 min of supine rest, subjects were tilted 30 degrees head-up. Mean arterial pressure (MAP) and total peripheral resistance (TPR) increased in C (MAP from 76.7 +/ -6.6 mmHg to 80.6 +/- 8.2 mmHg; TPR from 1.12 +/- 0.26 AU to 1.19 +/ -0.31 AU) while both remained unchanged in P. Echo Doppler ultrasound determined red blood cell velocity in the femoral artery, which decreased (P from 18.9+/-6.2 cm/s to 12.5 +/- 4.5 cm/s, P = 0.001; C from 16.3 +/- 6.2 cm/s to 10.8 +/- 5.0 cm/s, P = 0.001) and leg vascular resistance, which increased (P from 402 +/- 137 AU to 643 +/- 274 AU, P = 0.001; C from 238 +/- 68 AU to 400 +/- 122 AU, P = 0.003) from supine to upright. The present study shows that independent of supraspinal sympathetic control, humans are able to increase leg vascular resistance and maintain blood pressure during head-up tilt.

Despite the loss of centrally mediated sympathetic vasoconstriction, spinal cord-injured (SCI) individuals cope surprisingly well with orthostatic challenges. In the pathophysiology of this intriguing observation spinal sympathetic-, veno-arteriolar-(VAR), and myogenic reflexes seem to play a role. The purpose of this study was to assess whether central (stroke volume, heart rate, blood pressure and total peripheral resistance) and peripheral (leg blood flow, leg vascular resistance and femoral arterial diameter) hemodynamic responses to head-up tilt are different in two groups of SCI patients, i. e., SCI individuals with upper motor neuron lesions (who have spinal reflexes, VAR and myogenic reflexes) (U; n=6) and those with lower motor neuron lesion (who have no spinal reflexes, perhaps no VAR due to nerve degeneration, but intact myogenic reflexes) (L; n=5). Ten healthy male individuals served as controls (C) (normal supraspinal sympathetic control and presence of all reflexes). After 10 min supine rest all individuals were tilted to 30 degrees head-up tilt. Red blood cell velocity (measured by echo Doppler ultrasound) in the femoral artery decreased and vascular resistance increased significantly in all three groups in the upright position compared with supine. Mean arterial pressure (MAP) remained unchanged in U and L and increased significantly in C in the upright versus supine position. The present study shows that all SCI individuals were able to maintain MAP by increasing leg vascular resistance during head-up tilt, despite nerve degeneration in L and lack of centrally mediated sympathetic control in all SCI individuals. Results of the present study suggest that not spinal reflexes but local (myogenic) reflex activity plays a pivotal role in peripheral vascular responses upon head-up tilt when central control mechanisms fail.

Objective: To examine the effect of different levels of fractional inspired oxygen (FiO(2)) (15%, 21%, 50%) on peak oxygen consumption (VO(2)peak) during arm exercise in persons with spinal cord injury and in able-bodied controls., Design: Case-control study., Setting: University medical center in the Netherlands., Participants: Ten able-bodied controls, 6 persons with paraplegia, and 6 persons with tetraplegia., Interventions: Inspiration of 15%, 21%, and 50% oxygen during a 15-minute period before and during arm exercise., Main Outcome Measures: Oxygen uptake (VO(2)peak, VO(2)peak/kg), power output, ventilation, and base excess., Results: In the able-bodied controls, significant FiO(2) dependency was seen in power output, VO(2)peak, and VO(2)peak/kg. Persons with paraplegia showed significant FiO(2) dependency in VO(2) and VO(2)/kg. In persons with tetraplegia, no FiO(2) dependency was observed; however, VO(2) and VO(2)/kg were significantly higher at 50% than at 15% FiO(2). Ventilation and base excess did not change in able-bodied controls or in persons with paraplegia with different levels of FiO(2). In persons with tetraplegia, ventilation was significantly higher at 15% than at 50% FiO(2), and base excess did not change. No significant interactions between groups and FiO(2) were observed., Conclusions: Oxygen consumption during peak arm-cranking exercise is enhanced with an increased inspiratory oxygen fraction in able-bodied controls as well as in persons with paraplegia and to a lesser extent in persons with tetraplegia, indicating that peak oxygen consumption during arm exercise is limited by oxygen supply rather than by the small muscle mass and related biochemical limitations.

It has been shown that peak oxygen uptake (O(2)peak) during leg exercise is enhanced by an increased inspiratory oxygen fraction ( FiO(2)), indicating that oxygen supply is the limiting factor. Whether oxygen supply is a limiting factor in arm exercise performance is unknown. The purpose of this study, therefore, was to examine the effect of different levels of FiO(2 )on O(2)peak during arm exercise in healthy individuals. Nine men successfully performed three incremental arm-cranking exercise tests with FiO(2)15%, FiO(2)21% and FiO(2)50% applied in counterbalanced order. A significant FiO(2 )dependency was observed for O(2)peak ( p=0.02) and power output ( p=0.03) and post hoc tests revealed a significant difference in O(2)peak between 15 and 50% FiO(2 )( p=0.02), but not between 15 and 21% FiO(2), and 21 and 50% FiO(2). The results of this study show that O(2)peak is enhanced with increasing FiO(2), and suggest that O(2)peak during arm exercise is limited by oxygen supply rather than by the metabolic machinery within the muscle itself.

Venous occlusion strain gauge plethysmography (VOP) is based on the assumption that the veins are occluded and arterial inflow is undisturbed by the venous cuff pressure. Literature is not clear concerning the pressure that should be used. The purpose of this study was to determine the optimal venous occlusion pressure at which the highest arterial inflow is achieved in the forearm, calf, and leg by using VOP. We hypothesized that, for each limb segment, an optimal (range of) venous cuff pressure can be determined. Arterial inflow in each limb segment was measured in nine healthy individuals by VOP by using pressures ranging from 10 mmHg up to diastolic blood pressure. Arterial inflows were similar at cuff pressures between 30 and 60 mmHg for the forearm, leg, and calf. Arterial inflow in the forearm was significantly lower at 10 mmHg compared with the other cuff pressures. In addition, arterial inflows at 20 mmHg tended to be lower in each limb segment than flow at higher cuff pressures. In conclusion, no single optimum venous cuff pressure, at which a highest arterial inflow is achieved, exists, but rather a range of optimum cuff pressures leading to a similar arterial inflow. Venous cuff pressures ranging from 30 mmHg up to diastolic blood pressure are recommended to measure arterial inflow by VOP.

The purpose of the present study was to determine the effect of a spinal cord injury (SCI) on resting vascular resistance in paralyzed legs in humans. To accomplish this goal, we measured blood pressure and resting flow above and below the lesion (by using venous occlusion plethysmography) in 11 patients with SCI and in 10 healthy controls (C). Relative vascular resistance was calculated as mean arterial pressure in millimeters of mercury divided by the arterial blood flow in milliliters per minute per 100 milliliters of tissue. Arterial blood flow in the sympathetically deprived and paralyzed legs of SCI was significantly lower than leg blood flow in C. Because mean arterial pressure showed no differences between both groups, leg vascular resistance in SCI was significantly higher than in C. Within the SCI group, arterial blood flow was significantly higher and vascular resistance significantly lower in the arms than in the legs. To distinguish between the effect of loss of central neural control vs. deconditioning, a group of nine SCI patients was trained for 6 wk and showed a 30% increase in leg blood flow with unchanged blood pressure levels, indicating a marked reduction in vascular resistance. In conclusion, vascular resistance is increased in the paralyzed legs of individuals with SCI and is reversible by training.

The purpose of this study was to assess the relationship between inactivity and shear stress, the frictional force of blood against the endothelium, in spinal cord injury (SCI) subjects. SCI group offers a unique "model of nature" to study the effects of inactivity. Nine SCI subjects with upper (SCI-U) and 5 with a lower (SCI-L) motoneuron lesion and 10 able-bodied controls (C) were included. A venous blood sample was withdrawn to determine blood viscosity. Red blood cell velocities and arterial diameters of the common carotid artery (CCA) and common femoral artery (CFA) were measured by using echo-Doppler ultrasound in a supine position. No differences were observed in wall shear stress in the CCA between groups. In the CFA, peak and mean wall shear stress were significantly increased in SCI (14.1 and 1.2 Pa, respectively) compared with C (10.2 and 0.9 Pa, respectively). Because SCI-U and SCI-L showed no differences in shear stress levels, inactivity and not nerve degeneration seems to cause the elevated shear stress levels in the CFA in SCI. However, the lack of central neural control as a causal factor cannot be ruled out.