Bilateral bone marrow edema syndrome of the humeral head mimicking avascular necrosis: a case report

Abstract Background Bone marrow edema syndrome (BMES), previously referred to as transient osteoporosis, is an uncommon and underdiagnosed self-limiting condition typically affecting the weight-bearing joints of the hip and lower limb. Its occurrence in upper limb or non-weight bearing joints is particularly rare. Case presentation We report the case of an otherwise healthy 42-year-old man who gradually developed severe and disabling left shoulder pain over the course of 6 months. Nine months after onset, he presented clinically with bilateral involvement and radiologically (magnetic resonance imaging, MRI) with diffuse BME associated with a subchondral insufficiency fracture, suggestive of proximal humeral avascular necrosis. Clinical evidence of subacromial shoulder impingement and MRI findings of bilateral subchondral bursitis and tenosynovitis with effusion of the biceps tendons likely resulted from primary BME, as musculoskeletal sonography confirmed the absence of rotator cuff tears. Repeated MRI findings at 2, 12, and 18 months documented near complete resolution of both edema and fracture, consistent with BMES of the proximal humerus. During this time, the patient reported a gradual improvement in both pain symptoms and range of motion. The clinical picture of insidious shoulder pain, exacerbated by activity and improved by load relief, in the absence of predisposing factors for osteonecrosis or antecedent trauma in patients of middle age, should indicate the possibility of the diagnosis of BMES. Conclusions This unprecedented report documents a rare case of bilateral BMES of the humeral head mimicking avascular necrosis. The correct diagnosis of BMES within an atypical anatomical location avoids invasive measures in the affected bone. The misdiagnosis of secondary BME and idiopathic osteonecrosis can be avoided by recognizing the characteristics of BME and subchondral fractures of the humeral head in the absence of rotator cuff tears, as well as their evolution on serial MRI

Citation classics in epilepsy

BACKGROUND: The impact of a scientific article is proportional to the citations it has received. In this study, we set out to identify the most cited works in epileptology in order to evaluate research trends in this field. METHODS: According to the Web of Science database, articles with more than 400 citations qualify as "citation classics". We conducted a literature search on the ISI Web of Science bibliometric database for scientific articles relevant to epilepsy. RESULTS: We retrieved 67 highly cited articles (400 or more citations), which were published in 31 journals: 17 clinical studies, 42 laboratory studies, 5 reviews and 3 classification articles. Clinical studies consisted of epidemiological analyses (n=3), studies on the clinical phenomenology of epilepsy (n=5) – including behavioral and prognostic aspects – and articles focusing on pharmacological (n=6) and non-pharmacological (n=3) treatment. The laboratory studies dealt with genetics (n=6), animal models (n=27), and neurobiology (n=9) – including both neurophysiology and neuropathology studies. The majority (61%) of citation classics on epilepsy were published after 1986, possibly reflecting the expansion of research interest in laboratory studies driven by the development of new methodologies, specifically in the fields of genetics and animal models. Consequently, clinical studies were highly cited both before and after the mid 80s, whilst laboratory researches became widely cited after 1990. CONCLUSIONS: Our study indicates that the main drivers of scientific impact in the field of epileptology have increasingly become genetic and neurobiological studies, along with research on animal models of epilepsy. These articles are able to gain the highest numbers of citations in the time span of a few years and suggest potential directions for future research

Impulse Control Disorders Following Deep Brain Stimulation of the Subthalamic Nucleus in Parkinson's Disease: Clinical Aspects

Parkinson's disease (PD) has been associated with the development of impulse control disorders (ICDs), possibly due to overstimulation of the mesolimbic system by dopaminergic medication. Preliminary reports have suggested that deep brain stimulation (DBS), a neurosurgical procedure offered to patients with treatment-resistant PD, affects ICD in a twofold way. Firstly, DBS allows a decrease in dopaminergic medication and hence causes an improvement in ICDs. Secondly, some studies have proposed that specific ICDs may develop after DBS. This paper addresses the effects of DBS on ICDs in patients with PD. A literature search identified four original studies examining a total of 182 patients for ICDs and nine case reports of 39 patients that underwent DBS and developed ICDs at some point. Data analysis from the original studies did not identify a significant difference in ICDs between patients receiving dopaminergic medication and patients on DBS, whilst the case reports showed that 56% of patients undergoing DBS had poor outcome with regards to ICDs. We discuss these ambivalent findings in the light of proposed pathogenetic mechanisms. Longitudinal, prospective studies with larger number of patients are required in order to fully understand the role of DBS on ICDs in patients with PD

Premonitory urges and sensorimotor processing in Tourette syndrome

Most patients with Tourette syndrome report characteristic sensory experiences (premonitory urges) associated with the expression of tic symptoms. Despite the central role of these experiences to the clinical phenomenology of Tourette syndrome, little is known about their underlying brain processes. In the present article we present the results of a systematic literature review of the published studies addressing the pathophysiological mechanisms of premonitory urges. We identified some preliminary evidence for specific alterations in sensorimotor processing at both cortical and subcortical levels. A better insight into the brain correlates of premonitory urges could lead to the identification of new targets to treat the sensory initiators of tics in patients with Tourette syndrome

Functional neuroanatomy and behavioural correlates of the basal ganglia:evidence from lesion studies

Introduction: The basal ganglia are interconnected with cortical areas involved in behavioural, cognitive and emotional processes, in addition to movement regulation. Little is known about which of these functions are associated with individual basal ganglia substructures. Methods: Pubmed was searched for literature related to behavioural, cognitive and emotional symptoms associated with focal lesions to basal ganglia structures in humans. Results: Six case-control studies and two case reports were identified as relevant. Lesion sites included the caudate nucleus, putamen and globus pallidus. These were associated with a spectrum of behavioural and cognitive symptoms, including abulia, poor working memory and deficits in emotional recognition. Discussion: It is often difficult to precisely map associations between cognitive, emotional or behavioural functions and particular basal ganglia substructures, due to the non-specific nature of the lesions. However, evidence from lesion studies shows that most symptoms correspond with established non-motor frontal-subcortical circuits

Behavioural Treatments for Tourette Syndrome: An Evidence-Based Review

Tourette syndrome (TS) is a disorder characterised by multiple motor and vocal tics and is frequently associated with behavioural problems. Tics are known to be affected by internal factors such as inner tension and external factors such as the surrounding environment. A number of behavioural treatments have been suggested to treat the symptoms of TS, in addition to pharmacotherapy and surgery for the most severe cases. This review compiled all the studies investigating behavioural therapies for TS, briefly describing each technique and assessing the evidence in order to determine which of these appear to be effective. Different behavioural therapies that were used included habit reversal training (HRT), massed negative practice, supportive psychotherapy, exposure with response prevention, self-monitoring, cognitive-behavioural therapy, relaxation therapy, assertiveness training, contingency management, a tension-reduction technique and biofeedback training. Overall, HRT is the best-studied and most widely-used technique and there is sufficient experimental evidence to suggest that it is an effective treatment. Most of the other treatments, however, require further investigation to evaluate their efficacy. Specifically, evidence suggests that exposure with response prevention and self-monitoring are effective, and more research is needed to determine the therapeutic value of the other treatments. As most of the studies investigating behavioural treatments for TS are small-sample or single-case studies, larger randomised controlled trials are advocated

The Neurobiology and Treatment of Restless Legs Syndrome

Background: Restless legs syndrome (RLS) is a relatively common neurological disorder affecting sleep and health-related quality of life. Neuroimaging studies, autopsy investigations and experimental studies using animal models have been conducted to investigate the potential causes of RLS, resulting in the generation of multiple pathophysiological hypotheses. Methods: This paper reviews the neurobiology and pharmacotherapy of RLS, with a critical analysis of the heterogeneity and methodological limitations of the existing scientific literature. Results: Although several neurotransmitter systems dysfunction and neuroanatomical abnormalities have been implicated in RLS pathogenesis, dopamine dysfunction within basal ganglia pathways, iron deficiency and opioid system abnormalities have consistently been found to be involved. Their involvement is further strengthened by the therapeutic effectiveness of dopaminergic agents, iron supplementation and opioid medications. Discussion: Converging evidence from neuroimaging, autoptic and animal studies points towards dopamine dysregulation and iron metabolism alterations as the main contributors to RLS pathophysiology. The possible interactions between different neurotransmitter systems should guide further neuropharmacological research in order to improve therapeutic efficacy for this disabling condition

Headache in patients with Tourette syndrome: A systematic literature review

Aim: To systematically review the available literature on the prevalence and clinical characteristics of headache in patients with Tourette syndrome (TS), a neurodevelopmental condition characterized by the chronic presence of motor and vocal tics. Design: We conducted a systematic literature review of original studies using three clinical databases (Medline, EMBASE and PsycInfo), according to the standards outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Outcome measures included both tic severity and tic frequency. Results: Our systematic literature review identified six studies on the prevalence and characteristics of headache in patients with TS (data on paediatric patients were available from five studies and data on adult patients from four studies). The proportion of patients with TS who had a diagnosis of headache ranged from 29% to 62%. Migraine was the most commonly investigated headache disorder in this patient population. The findings of the reviewed studies showed that the prevalence of migraine in patients with TS ranges between 13% and 43% and is significantly higher compared to the two control groups (p < 0.05). Discussion: The prevalence of headache in patients with TS could be higher than in the general population, with the most reliable data being available for migraine. Overall, the literature on the prevalence and characteristics of headache in TS is sparse and highly heterogeneous. Further research on the prevalence and clinical correlates of migraine, tension-type headache and other types of headache in patients with TS across the lifespan is needed to inform clinical practice aimed at improving health-related quality of life in patients with tics