Food insecurity in children with heart disease.

PURPOSE OF REVIEW: This review discusses the epidemiology of food insecurity (FI) and its consequences in children with congenital heart disease. We aimed to highlight current interventions to screen and address food insecurity in the context of pediatric cardiology and to offer strategies for providers to engage in this meaningful work. RECENT FINDINGS: Food insecurity is consistently associated with poor health outcomes in children. In the United States, 17.3% of households with children experience FI. Nonwhite and single-parent families are disproportionately affected. Interestingly, because of a low-quality diet, FI is associated with childhood obesity, putting affected children at increased risk for cardiovascular morbidity and mortality over time. Children with congenital heart disease are susceptible to poor outcomes due to unique altered metabolic demands, increased risk for growth impairment, frequent need for specialized feeding regimens, and additional morbidity associated with heart surgery in underweight children. SUMMARY: Today, the burden of screening for FI is most commonly placed on general pediatricians. Considering the importance of nutrition to cardiovascular health and general wellbeing, and the ease with which screening can be performed, pediatric cardiologists and other subspecialists should take a more active role in FI screening.

Tilt testing.

Tilt testing can help to diagnose unexplained syncope, by precipitating an episode during cardiac monitoring. The Italian protocol, now most widely used, involves giving sublingual nitroglycerine after 15 min, while monitoring beat-to-beat blood pressure (BP) and recording on video. Tilt testing is time-consuming but it is clinically useful and can guide therapy. Complications are rare. Syncope types include vasovagal syncope where BP falls after >3 min of tilt-up and later the heart rate falls; classic orthostatic hypotension where there is an immediate, progressive BP fall with minimal heart rate change; delayed orthostatic hypotension with a late BP fall after a stable phase but little or no heart rate rise; psychogenic pseudosyncope with apparent loss of consciousness, but no BP fall and a moderate heart rate rise; and postural orthostatic tachycardia syndrome where there is a significant heart rate rise but no BP fall.

Neurosarcoidosis associated with intracerebral haemorrhage: a challenge in diagnosis and management.

Sarcoidosis is an idiopathic multisystem granulomatous disorder of unknown cause. Nervous system involvement (central and/or peripheral) is uncommon, developing in 5%-10%. The presenting symptoms are variable, reflecting the level of involvement, and frequently fluctuate and progress. Diagnosing neurosarcoidosis in people with previously confirmed systemic disease may be relatively straightforward, but diagnosing primary neurosarcoidosis is challenging. Managing neurosarcoidosis is primarily consensus based; corticosteroid is its mainstay, alongside corticosteroid-sparing agents and emerging novel therapies. We describe a 39-year-old woman who presented with cranial neuropathy. Serial imaging, cerebrospinal fluid sampling and tissue biopsy gave a diagnosis of probable neurosarcoidosis. Her clinical course was complicated by intracerebral haemorrhage following intravenous corticosteroids for neurological relapse. This is a very rare complication of neurosarcoidosis; we discuss its possible causes and suggest ways to reduce its risk.

Sudden unexpected death in epilepsy genetics: Molecular diagnostics and prevention.

Epidemiologic studies clearly document the public health burden of sudden unexpected death in epilepsy (SUDEP). Clinical and experimental studies have uncovered dynamic cardiorespiratory dysfunction, both interictally and at the time of sudden death due to epilepsy. Genetic analyses in humans and in model systems have facilitated our current molecular understanding of SUDEP. Many discoveries have been informed by progress in the field of sudden cardiac death and sudden infant death syndrome. It is becoming apparent that SUDEP genomic complexity parallels that of sudden cardiac death, and that there is a pauci1ty of analytically useful postmortem material. Because many challenges remain, future progress in SUDEP research, molecular diagnostics, and prevention rests in international, collaborative, and transdisciplinary dialogue in human and experimental translational research of sudden death.

Prolonged implantable electrocardiographic monitoring indicates a high rate of misdiagnosis of epilepsy--REVISE study.

AIMS: Syncope, epilepsy, and psychogenic pseudo-syncope are the most common causes of transient loss of consciousness (T-LOC or blackout). All can present with similar features, including abnormal limb movements. It is reported that somewhere between 13 and 42% of patients with 'epilepsy' may be misdiagnosed. A UK Parliamentary working group found that at least 74 000 English patients are misdiagnosed with epilepsy, and taking antiepileptic drugs. The likely alternative diagnosis is 'convulsive' syncope, mimicking an epileptic seizure. We hypothesized that many patients misdiagnosed with epilepsy have convulsive reflex syncope, and that prolonged electrocardiographic (ECG) monitoring with an implantable ECG recorder (ILR) would show reflex cardioinhibition during T-LOC. This would respond to permanent pacing and allow antiepileptic drugs to be withdrawn. We also aimed to evaluate tilt testing and other tests done in these patients. METHODS AND RESULTS: We included patients previously diagnosed with epilepsy, but considered to have a definite or likely misdiagnosis of epilepsy after specialist neurological review. All received an ILR (Reveal Plus(®)/Reveal DX(®), Medtronic Inc.), and tilt-table testing. One hundred and three patients were included, mean age of 46 ± 17 years, with 58 of 103 (56%) female patients. A diagnosis of epilepsy was previously made by a neurologist in 69%, but definite tonic-clonic seizures were only noted in the history in 4%. In 22 patients (21%), the ILR recorded profound bradyarrhythmia or asystole with convulsive features, and they were offered pacemaker implantation. After pacing and withdrawal of antiepileptic drugs, 60% of these patients were asymptomatic. Only 14% of patients had a positive tilt-table test. In these, there was no correlation with the ECG findings of a spontaneous blackout during ILR recording. CONCLUSION: This study shows a high incidence of the cardioinhibition of reflex syncope in patients with convulsive T-LOC previously diagnosed as epilepsy and treated with antiepileptic drugs. We believe that reflex syncope with convulsive features mimics generalized epilepsy, leading to a misdiagnosis. This may be a widespread problem accounting for many wrong diagnoses of epilepsy. There was also poor correlation in ECG findings between tilt testing and ILR recording.

Ketogenic diet and other dietary treatments for epilepsy.

BACKGROUND: The ketogenic diet, being high in fat and low in carbohydrates, has been suggested to reduce seizure frequency. It is currently used mainly for children who continue to have seizures despite treatment with antiepileptic drugs. Recently there has been interest in less restrictive ketogenic diets including the Atkins diet and the use of these diets has extended into adult practice. OBJECTIVES: To review the evidence from randomised controlled trials regarding the effects of ketogenic and similar diets. SEARCH METHODS: We searched the Cochrane Epilepsy Group's Specialised Register (June 2011), the Cochrane Central Register of Controlled Trials (CENTRAL 2011, Issue 2 of 4), MEDLINE (1948 to May week 4, 2011) and EMBASE (1980 to March 2003). No language restrictions were imposed. We checked the reference lists of retrieved studies for additional reports of relevant studies. SELECTION CRITERIA: Studies of ketogenic diets and similar diets for people with epilepsy. DATA COLLECTION AND ANALYSIS: Three review authors independently applied pre-defined criteria to extract data and also assessed study quality. MAIN RESULTS: We identified four randomised controlled trials which generated five publications.These included Kossoff 2007, Bergqvist 2005, Seo 2007, Neal 2008 and Neal 2009. All trials applied the intention-to-treat analysis with varied randomisation method. The four studies recruited a total of 289 children and adolescents and no adults. Meta-analysis could not be conducted due to heterogeneity of the studies. Seven prospective studies and four retrospective studies were also identified. AUTHORS' CONCLUSIONS: Our review update included data from four new randomised studies of the ketogenic diet. Although none were blinded, some were of good quality. These studies suggest that in children, the ketogenic diet results in short to medium term benefits in seizure control, the effects of which are comparable to modern antiepileptic drugs. However, one study of long term outcome reports a high attrition rate for the diet. This would suggest that many children find the diet difficult to tolerate. The main reasons for drop-outs in the included studies included gastrointestinal side effects and dislike for the diet.We found just three studies on the use of the diet in adults and none of these were randomised. There has been less research involving other diets. We found one randomised study of reasonable quality of the Atkins diet. This study showed similar benefits in seizure control with a less restrictive diet.For those with medically intractable epilepsy or those in whom surgery is unsuitable, a ketogenic diet could improve seizure control, but tolerability is poor. One observational study suggested that the Atkins diet may have a similar effect on seizure control, but this requires more investigation.

Synopsis of the National Institute for Health and Clinical Excellence Guideline for management of transient loss of consciousness.

DESCRIPTION: Transient loss of consciousness (TLoC) is common and often leads to incorrect diagnosis, unnecessary investigation, or inappropriate choice of specialist referral. In August 2010, the National Institute for Health and Clinical Excellence published a guideline that addressed the initial assessment of and most appropriate specialist referral for persons who have experienced TLoC. The guideline focused on correct diagnosis and relevant specialist referral and did not make treatment recommendations. This synopsis describes the principal recommendations concerning assessment and referral of a patient with TLoC. METHODS: The National Clinical Guideline Centre developed the guidelines by using the standard methodology of the National Institute for Health and Clinical Excellence. A multidisciplinary guideline panel generated review questions, discussed evidence, and formulated recommendations. The panel included a technical team from the National Clinical Guideline Centre, who reviewed and graded all relevant evidence identified from literature searches published in English up to November 2009 and performed health-economic modeling. Both guideline development and final modifications were informed by comments from stakeholders and the public. RECOMMENDATIONS: The panel made clear recommendations regarding the assessment of a person after TLoC, which emphasized the importance of clinical reasoning in diagnosis. Persons with uncomplicated faint, situational syncope, or orthostatic hypotension should receive electrocardiography but do not otherwise require immediate further investigation or specialist referral. Persons with features that suggest epilepsy should be referred for specialist neurologic assessment; brief seizure-like activity was recognized as a common occurrence during syncope that should not be regarded as indicating epilepsy. Persons with a suspected cardiac cause for TLoC or in whom TLoC is unexplained after initial assessment should receive specialist cardiovascular assessment. Guidance was provided on the appropriate choices of cardiovascular investigation, according to the presenting clinical circumstances.