A case of idiopathic hypertrophic cranial pachymeningitis manifested only by positive rheumatoid factor and abnormal findings of the anterior falx

In this report, we present a rare case of a 52-year-old man with a unique form of hypertrophic pachymeningitis involving the anterior part of the falx and who was positive for rheumatoid factor. The clinical symptom was only headache, without any cranial nerve palsies or ataxia. Diagnosis was made by gallium scintigraphy and magnet resonance imaging but was not confirmed by dural biopsy. Treatment with corticosteroid alone was extremely effective for him, while in most cases hypertrophic pachymeningitis recurs or progresses despite the treatment.     

MPO-ANCA-positive Wegener’s granulomatosis presenting with hypertrophic cranial pachymeningitis: case report and review of the literature

We describe a case of hypertrophic cranial pachymeningitis (HCP) associated with Wegeners granulomatosis (WG) in a 60-year-old man presenting with chronic headache and multiple cranial nerve neuropathies. A test for antibodies to the neutrophil cytoplasmic protein myeloperoxidase (MPO-ANCA) was positive in this case. We review the literature on perinuclear (p)-ANCA-related HCP, including our case. This case indicates the link between MPO-ANCA-positive WG and HCP.     

R-wave amplitude response to myocardial ischemia in hypertrophic cardiomyopathy

Background and Purpose

R-wave amplitude change during exercise has been reported to enhance diagnostic value for myocardial ischemia in coronary heart disease.

Methods

We summed up R-wave amplitude in all the 12 leads during exercise testing and correlated the results with regional myocardial ischemia or diffuse subendocardial ischemia as detected by scintigraphy in 49 patients with hypertrophic cardiomyopathy (HCM) and 16 controls.

Results

The sum of R-wave amplitude decreased during exercise in patients with HCM (mean, 12.4 mV to 11.7 mV, P < .01) as well as in controls (8.0 mV to 7.7 mV, P < .05). Percent changes in the sum of R-wave amplitude did not differ between 4 subgroups of patients with HCM: one having both regional and subendocardial ischemia, one only the former, one only the latter, and one neither of them (mean, 6.5%, 7.7%, 4.6%, and 5.1%; P = .79).

Conclusions

R-wave amplitude response to exercise failed to demonstrate myocardial ischemia in our patients with HCM.     

Strategies for assessing the prevalence of cardiovascular sudden deaths in young competitive athletes

Background/Objectives

Prevalence/incidence of sudden death due to cardiovascular disease in young competitive athletes has become an important part of the debate over the most effective and practical preparticipation screening strategies for this population. Since event reporting is not mandatory, identification of cases has been achieved largely through publicly available data and internet searches. The accuracy of this methodology has not been studied and deserves scrutiny.

Methods

We assessed recognition of sudden cardiovascular deaths in college (NCAA) athletes with the U.S. National Registry of Sudden Death in Athletes that uses largely public domain sources, and also the NCAA Memorial Resolutions List.

Results

For 2002–2011, 64 total sudden death cases were identified by both sources. The Registry identified 56 cases (88%), including 14 not found in the NCAA List. The NCAA List identified 50 cases (78%), including 8 unrecognized by the Registry (p = 0.16). Failure to initially recognize these 8 deaths using established Registry search mechanisms was due to the absence of key search terms in media reports. Cases not identified by the 2 methodologies did not differ significantly regarding demographics, cause of death, or sport.

Conclusions

Internet-based, public domain methodology is useful and identified more cases of sudden cardiovascular death in college athletes than did the internal list provided by the NCAA. Nevertheless, these findings support the principle that multiple sources are additive and beneficial in identifying the maximum number of sudden death events.     

影像呈结节状占位样的特发性肥厚性硬脑膜炎临床分析

 目的 探讨影像呈结节状占位样的特发性肥厚性硬脑膜炎(IHCP)的临床、影像及病理特点。方法 对4例影像呈结节状的IHCP患者的临床资料进行回顾性分析。结果 4例均为男性,平均发病年龄40.25(33～50)岁,病程长(4个月～12年),病情反复发作。主要表现为慢性头痛、多组脑神经麻痹及癫痫发作。头颅影像示局部硬脑膜增厚,呈团块或结节状,与脑膜瘤等肿瘤相似。2例组织病理学检查示硬脑膜结缔组织增生、散在中性粒细胞及浆细胞浸润。甲泼尼龙或联合免疫抑制剂治疗有效。结论 结节状占位样IHCP多以慢性头痛起病,糖皮质激素或联合免疫抑制剂治疗有效,病情易复发。头颅影像可见硬脑膜肥厚,呈团块或结节状占位,易与脑肿瘤、炎性肉芽肿相混淆。
        

Expressão fenotípica da miocardiopatia hipertrófica e realce tardio na ressonância magnética cardíaca

Introduction and AimThe prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients.MethodsFrom a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow‐up were analyzed.ResultsPatients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/É ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter‐defibrillator (ICD) (p=0.015). At follow‐up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality.ConclusionsThe presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events.     

Predicting severity of pathological scarring due to burn injuries: a clinical decision making tool using Bayesian networks

It is important for clinicians to understand which are the clinical signs, the patient characteristics and the procedures that are related with the occurrence of hypertrophic burn scars in order to carry out a possible prognostic assessment. Providing clinicians with an easy‐to‐ use tool for predicting the risk of pathological scars. A total of 703 patients with 2440 anatomical burn sites who were admitted to the Department of Plastic and Reconstructive Surgery, Burn Center of the Traumatological Hospital in Torino between January 1994 and May 2006 were included in the analysis. A Bayesian network (BN) model was implemented. The probability of developing a hypertrophic scar was evaluated on a number of scenarios. The error rate of the BN model was assessed internally and it was equal to 24·83%. While classical statistical method as logistic models can infer only which variables are related to the final outcome, the BN approach displays a set of relationships between the final outcome (scar type) and the explanatory covariates (patient's age and gender, burn surface area, full‐thickness burn surface area, burn anatomical area and wound‐healing time; burn treatment options such as advanced dressings, type of surgical approach, number of surgical procedures, type of skin graft, excision and coverage timing). A web‐based interface to handle the BN model was developed on the website www.pubchild.org (burns header). Clinicians who registered at the website could submit their data in order to get from the BN model the predicted probability of observing a pathological scar type.