Myocardial deformation assessment in patients with precapillary pulmonary hypertension: A cardiac magnetic resonance study

PurposeThe purpose of this study was to investigate right atrial and ventricular strain parameters on cardiac magnetic resonance (CMR) in patients with precapillary pulmonary hypertension (PPH) and whether they can aid in the assessment of PPH prognosis.Materials and methodsAdult patients with groups 1 and 4 PPH were invited to participate in the study. Age- and sex-matched healthy volunteers were also recruited as controls. At baseline, patients underwent clinical examination, N-terminal pro-B-type natriuretic peptide measurement and CMR with feature tracking post-processing (CMR-FT). Healthy controls underwent only CMR-FT. The study's primary endpoint was clinical failure, defined as death, hospitalization or demonstrable clinical deterioration during follow-up. Patients who were unable to perform 6-minute walking test due to musculoskeletal disorders were excluded from the study.ResultsThirty-six patients (8 men, 28 women; mean age, 50.6 ± 13.8 [SD] years [range: 18.6–78.5 years]) and 12 healthy control subjects (5 mean, 7 women; mean age, 40.6 ± 13.5 [SD] years [range: 23.1–64.4 years]) were recruited. Right ventricular global longitudinal strain (GLS) was significantly impaired in PPH patients (?20.2 ± 5.3 [SD] % [range: ?28.8 to ?9.1%] vs. ?28.4 ± 3.1% [?33.7 to ?22.7%] respectively, P < 0.001). The right atrial GLS was significantly impaired in PPH compared to healthy controls (?19.9 ± 4.5% [range: ?28.6 to ?3.6%] vs. ?26.5 ± 4.2% [range: ?32.8 to ?15.8%] respectively) (P < 0.001). Clinical failure occurred in 19 (19/36, 53%) of patients. Right ventricular GLS predicted clinical failure most reliably among CMR parameters (?22.6 ± 3.8 [SD] % [range: ?27.6 to ?12.7%] for patients without clinical failure vs. ?18 ± 5.6 [SD] % [range: ?28.8 to ?9.1%] for patients with clinical failure; hazard ratio [HR] = 1.85; P = 0.007; area under the AUC curve = 0.75). Lower absolute right atrial GLS was significantly associated with clinical failure (?22.7 ± 3.0 [SD] % [range: ?28.6 to ?17.7%] for patients without clinical failure vs. ?16.9 ± 5.8 [SD] % [range: ?24.2 to ?3.6%] for patients with clinical failure) (HR = 1.53; P = 0.035).ConclusionCMR feature tracking-derived myocardial strain parameters of both the right atrium and ventricle can assist clinicians in the prognosis of PPH.     

Hip Displacement in Cerebral Palsy: The Role of Surveillance

IntroductionHip displacement is common in cerebral palsy (CP) and is related to the severity of neurological and functional impairment. It is a silent, but progressive disease, and can result in significant morbidity and decreased quality of life, if left untreated. The pathophysiology of hip displacement in CP is a combination of hip flexor-adductor muscle spasticity, abductor muscle weakness, and delayed weight-bearing, resulting in proximal femoral deformities and progressive acetabular dysplasia. Due to a lack of symptoms in the early stages of hip displacement, the diagnosis is easily missed. Awareness of this condition and regular surveillance by clinical examination and serial radiographs of the hips are the key to early diagnosis and treatment.Hip surveillance programmesSeveral population-based studies from around the world have demonstrated that universal hip surveillance in children with CP allows early detection of hip displacement and appropriate early intervention, with a resultant decrease in painful dislocations. Global hip surveillance models are based upon the patients’ age, functional level determined by the Gross Motor Function Classification system (GMFCS), gait classification, standardized clinical exam, and radiographic indices such as the migration percentage (MP), as critical indicators of progressive hip displacement.ConclusionDespite 25 years of evidence showing the efficacy of established hip surveillance programmes, there is poor awareness among healthcare professionals in India about the importance of regular hip surveillance in children with CP. There is a need for professional organizations to develop evidence-based guidelines for hip surveillance which are relevant to the Indian context.     

Efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) created using covered stents of different diameters: A systematic review and meta-analysis

PurposeThe purpose of this study was to make a systematic review and meta-analysis to determine the stent diameter (8 mm vs. 10 mm) that conveys better safety and clinical efficacy for transjugular intrahepatic portosystemic shunt (TIPS).Materials and methodsFour databases were used to identify clinical trials published from inception until March 2020. Data were extracted to estimate and compare one-year and three-year overall survivals, hepatic encephalopathy, variceal rebleeding, and shunt dysfunction rates between patients with 8 mm covered stents and those with 10 mm covered stents.ResultsFive eligible studies were selected, which included 489 patients (316 men, 173 women). The 8 mm covered stent group had higher efficacy regarding one-year or three-year overall survival (odds ratio [OR], 2.88; P = 0.003) and (OR, 1.81; P = 0.04) and lower hepatic encephalopathy (OR, 0.69; P = 0.04) compared with 10 mm covered stent group. There were no significant differences in variceal rebleeding rate (OR 0.80; P = 0.67). However, shunt dysfunction was lower in 10 mm covered stent group (OR, 2.26; P = 0.003).ConclusionsOur results suggest that the use of 8 mm covered stents should be preferred to that of 10 mm covered stents for TIPS placement when portal pressure is frequently monitored.     

Compensatory postural responses to backward loss of balance in patients with cerebellar disease

BackgroundImpaired control of balance and coordinated reactions are a primary deficit of cerebellar dysfunction. As compared to other neurological patients with balance impairments, there has been little research assessing the characteristics of compensatory responses associated with falls in patients with cerebellar disease (CD).Research questionThe aim of this study was to examine the effects of cerebellar disease on compensatory balance control in response to postural perturbation. Do CD patients increase the number of steps when responding to instability because of inappropriate initial step reactions or poor control of trunk motion or both?MethodsIn this explorative study, 10 patients suffering from degenerative cerebellar ataxia and 10 age-matched healthy controls were examined. The balance recovery reactions were assessed using a lean-and-release postural perturbation method. Spatiotemporal characteristics of stepping movement and COM variables associated with torso motion were analyzed using 3D motion capture system.ResultsCD patients took multiple steps whereas matched controls generally took single steps to recover balance following perturbation. The characteristics of the initial step at the time of the fall revealed that foot reaction time, foot response time, and step distance of the initial step were similar between CD patients and matched controls. However, CD patients exhibited a shorter foot-to−COM distance, higher COM velocity, and less trunk flexion with which to attenuate their body momentum after the landing of the first step than did matched controls.SignificanceAlthough initial step responses were probably adequate, poor control of torso motion appears to be a particular problem that causes multiple-step reactions in CD patients. This observation would help to guide the development of tailored fall intervention strategies in CD patients aimed at promoting their recovery capacity in response to a pronounced balance challenge.     

Motor dysfunction in mild cognitive impairment as tested by kinematic analysis and transcranial magnetic stimulation

ObjectivePrevious studies have demonstrated voluntary movement alterations as well as motor cortex excitability and plasticity changes in patients with mild cognitive impairment (MCI). To investigate the pathophysiology of movement abnormalities in MCI, we tested possible relationships between movement abnormalities and primary motor cortex alterations in patients.MethodsFourteen amnestic MCI (aMCI) patients and 16 healthy controls were studied. Cognitive assessment was performed using clinical scales. Finger tapping was recorded by a motion analysis system. Transcranial magnetic stimulation was used to test the input/output curve of motor evoked potentials, intracortical inhibition, and short-latency afferent inhibition. Primary motor cortex plasticity was probed by theta burst stimulation. We investigated correlations between movement abnormalities, clinical scores, and cortical neurophysiological parameters.ResultsMCI patients showed less rhythmic movement but no other movement abnormalities. Cortical excitability measures were normal in patients, whereas plasticity was reduced. Movement rhythm abnormalities correlated with frontal dysfunction scores.ConclusionOur study in MCI patients demonstrated abnormal voluntary movement and plasticity changes, with no correlation between the two. Altered rhythm correlated with frontal dysfunction.SignificanceOur results contribute to the understanding of pathophysiological mechanisms of motor impairment in MCI.     

DRPLA: An unusual disease or an underestimated cause of ataxia in Brazil?

BackgroundDentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant spinocerebellar ataxia caused by pathological expansion of CAG trinucleotide repeats in the ATN1 gene. Most cases were described in patients from Japanese ancestry who presented with adult-onset progressive cerebellar ataxia associated with cognitive impairment, choreoathetosis and other movement disorders. DRPLA has been rarely described in Brazilian patients.MethodsWe performed a retrospective observational multicentric study including six different Neurology Centers in Brazil. All patients with genetically confirmed diagnosis of DRPLA had their medical records evaluated and clinical, genetic and neuroimaging features were analyzed.ResultsWe describe of eight Brazilian patients (5 male, 3 female) from four nuclear families with genetically confirmed DRPLA. The most common neurological features included cerebellar ataxia (n = 7), dementia (n = 3), chorea (n = 2), psychiatric disturbances (n = 2), progressive myoclonic epilepsy (n = 2) and severe bulbar signs (n = 1). Progressive myoclonic epilepsy was observed in two juvenile-onset cases before 20-year. A large CAG trinucleotide length was observed in the two juvenile-onset cases and genetic anticipation was observed in all cases. Neuroimaging studies disclosed cerebellar atrophy (n = 6), as well as brainstem and cerebellar atrophy (n = 2) and leukoencephalopathy (n = 1).ConclusionThe patients described herein reinforce that clinical features of DRPLA are highly influenced by age of onset, genetic anticipation and CAG repetition lengths. There is a large complex spectrum of neurological features associated with DRPLA, varying from pure cerebellar ataxia to dementia associated with other movement disorders (myoclonus, choreoathetosis). DRPLA is an unusual cause of cerebellar ataxia and neurodegeneration in Brazilian patients.