Ten‐Year Reflections on the Neurophysiological Abnormalities of Focal Dystonias in Humans

The physiological landscape of dystonia has changed considerably over the past 10 years. Initial ideas that dystonic motor symptoms could be explained by a combination of loss of inhibition and increased plasticity, together with subtle deficits in sensory processing, have been questioned, whereas the possible role of the cerebellum has risen in importance. In addition, it has been recognized that symptoms affect more than just the motor and sensory systems and encompass independent cognitive and psychological changes. Finally, it has become clear that, despite similarities in symptoms, there may be pathophysiological differences between idiopathic, inherited, and acquired forms of dystonia. In other words, progress in the pathophysiology of dystonia has followed the usual pattern from an initial phase in which core deficits are readily explained by highly simplified models to a realization that within a highly interconnected network, effects are more nuanced with widespread changes that might either compensate or contribute to the clinical symptoms to different degrees in different individuals. © 2019 International Parkinson and Movement Disorder Society     

Iron deficiency anaemia revisited

Iron deficiency anaemia is a global health concern affecting children, women and the elderly, whilst also being a common comorbidity in multiple medical conditions. The aetiology is variable and attributed to several risk factors decreasing iron intake and absorption or increasing demand and loss, with multiple aetiologies often coexisting in an individual patient. Although presenting symptoms may be nonspecific, there is emerging evidence on the detrimental effects of iron deficiency anaemia on clinical outcomes across several medical conditions. Increased awareness about the consequences and prevalence of iron deficiency anaemia can aid early detection and management. Diagnosis can be easily made by measurement of haemoglobin and serum ferritin levels, whilst in chronic inflammatory conditions, diagnosis may be more challenging and necessitates consideration of higher serum ferritin thresholds and evaluation of transferrin saturation. Oral and intravenous formulations of iron supplementation are available, and several patient and disease‐related factors need to be considered before management decisions are made. This review provides recent updates and guidance on the diagnosis and management of iron deficiency anaemia in multiple clinical settings.     

Symptom‐related changes in current perception threshold of restless legs syndrome

Few studies have investigated the mechanisms responsible for the symptoms of restless legs syndrome (RLS). However, these studies were mainly performed during the asymptomatic period and therefore their findings might not apply to changes in sensory processing that occur during the symptomatic period. The objective of this study was to investigate the function of sensory nerve fibres in RLS patients using the current perception threshold (CPT) test during the daytime and in the presence of symptoms. Ninety‐three patients with RLS and 34 healthy controls were included in the study. RLS patients were further divided into two subgroups, those who were experiencing RLS symptoms during the CPT test (symptom+) and those without symptoms (symptom?). Demographic data, RLS rating scale score and visual analogue scale were collected. Of the 127 enrolled subjects, CPT values were significantly lower in RLS patients than in controls for all three frequencies. Among the control and RLS subgroups (53 symptom+, 40 symptom?), symptom+ patients showed lower CPT values than controls. This finding indicates a relative hyperaesthetic state in the sensory afferents of peripheral nerves in symptom+ patients. There were no significant differences between the symptom? group and controls. The significantly lower CPT values for all three frequencies in symptom+ patients suggest that central sensory processing disturbance of sensory nerve fibres’ input may be involved in the development of symptoms in RLS patients.     

Optimisation of the organ donor and effects on transplanted organs: a narrative review on current practice and future directions

Mortality remains high for patients on the waiting list for organ transplantation. A marked imbalance between the number of available organs and recipients that need to be transplanted persists. Organs from deceased donors are often declined due to perceived and actual suboptimal quality. Adequate donor management offers an opportunity to reduce organ injury and maximise the number of organs than can be offered in order to respect the donor's altruistic gift. The cornerstones of management include: correction of hypovolaemia; maintenance of organ perfusion; prompt treatment of diabetes insipidus; corticosteroid therapy; and lung protective ventilation. The interventions used to deliver these goals are largely based on pathophysiological rationale or extrapolations from general critical care patients. There is currently insufficient high-quality evidence that has assessed whether any interventions in the donor after brain death may actually improve immediate post-transplant function and long-term graft survival or recipient survival after transplantation. Improvements in our understanding of the underlying mechanisms following brain death, in particular the role of immunological and metabolic changes in donors, offer promising future therapeutic opportunities to increase organ utilisation. Establishing a UK donor management research programme involves consideration of ethical, logistical and legal issues that will benefit transplanted patients while respecting the wishes of donors and their families.