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Background: A previously healthy male patient in his fifties presented with subacute onset of severe, diffuse dysautonomia with orthostatic hypotension as the main symptom. A lengthy interdisciplinary workup revealed a rare condition., Case Presentation: Over the course of a year, the patient was twice admitted to the local department of internal medicine because of severe hypotension. Testing showed severe orthostatic hypotension with normal cardiac function tests and no apparent underlying cause. On referral to neurological examination, symptoms of a broader autonomic dysfunction were discovered, with symptoms of xerostomia, irregular bowel habits, anhidrosis and erectile dysfunction. The neurological examination was normal, except for bilateral mydriatic pupils. The patient was tested for ganglionic acetylcholine receptor (gAChR) antibodies. A strong positive result confirmed the diagnosis of autoimmune autonomic ganglionopathy. There were no signs of underlying malignancy. The patient received induction treatment with intravenous immunoglobulin and later maintenance treatment with rituximab, resulting in significant clinical improvement., Interpretation: Autoimmune autonomic ganglionopathy is a rare but likely underdiagnosed condition, which may cause limited or widespread autonomic failure. Approximately half of the patients have ganglionic acetylcholine receptor antibodies in serum. It is important to diagnose the condition as it can cause high morbidity and mortality, but responds to immunotherapy.

Background: An implantable loop recorder (ILR) is an effective tool for diagnosing unexplained syncope (US). We examined the diagnostic utility of an ILR in detecting arrhythmic causes of US and determining which clinical factors are associated with pacemaker (PM) implantation., Methods: This retrospective, multicenter, observational study was conducted from February 2006 to April 2018 at 11 hospitals in Korea. Eligible patients with recurrent US received an ILR to diagnose recurrent syncope and document arrhythmia., Results: A total of 173 US patients (mean age, 67.6 ± 16.5 years; 107 men [61.8%]) who received an ILR after a negative conventional workup were enrolled. During a mean follow-up of 9.4 ± 11.1 months, 52 patients (30.1%) had recurrent syncope, and syncope-correlated arrhythmia was confirmed in 34 patients (19.7%). The ILR analysis showed sinus node dysfunction in 24 patients (70.6%), supraventricular tachyarrhythmia in 4 (11.8%), ventricular arrhythmia in 4 (11.8%), and sudden atrioventricular block in 2 (5.9%). Overall, ILR detected significant arrhythmia in 99 patients (57.2%) irrespective of syncope. Among patients with clinically relevant arrhythmia detected by ILR, PM implantation was performed in 60 (34.7%), an intra-cardiac defibrillator in 5 (2.9%), and catheter ablation in 4 (2.3%). In a Cox regression analysis, history of paroxysmal atrial fibrillation (PAF) (hazard ratio [HR], 2.34; 95% confidence interval [CI], 1.33-4.12; P < 0.01) and any bundle branch block (BBB) (HR, 2.52; 95% CI, 1.09-5.85; P = 0.03) were significantly associated with PM implantation., Conclusion: ILR is useful for detecting syncope-correlated arrhythmia in patients with US. The risk of PM is high in US patients with a history of PAF and any BBB., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2020 The Korean Academy of Medical Sciences.)

Hypertrophic cardiomyopathy (HCM) is associated with an increased risk of sudden cardiac death (SCD), although perhaps not as significantly as previously believed. Given the heterogeneous nature of this disease entity, risk stratification of individuals with HCM remains challenging. The recent HCM risk-SCD prediction model seems to perform well in assessing individual SCD risk. Even though implantable cardiac defibrillators (ICDs) are effective in preventing SCD in patients at increased risk, the importance of shared decision making in deciding whether or not to undergo ICD implantation cannot be understated., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: We evaluated global topology and organization of regional hubs in the brain networks and microstructural abnormalities in the white matter of patients with reflex syncope., Methods: Twenty patients with reflex syncope and thirty healthy subjects were recruited, and they underwent diffusion tensor imaging (DTI) scans. Graph theory was applied to obtain network measures based on extracted DTI data, using DSI Studio. We then investigated differences in the network measures between the patients with reflex syncope and the healthy subjects. We also analyzed microstructural abnormalities of white matter using tract-based spatial statistics analysis (TBSS)., Results: Measures of global topology were not different between patients with reflex syncope and healthy subjects. However, in reflex syncope patients, the strength measures of the right angular, left inferior frontal, left middle orbitofrontal, left superior medial frontal, and left middle temporal gyrus were lower than in healthy subjects. The betweenness centrality measures of the left middle orbitofrontal, left fusiform, and left lingual gyrus in patients were lower than those in healthy subjects. The PageRank centrality measures of the right angular, left middle orbitofrontal, and left superior medial frontal gyrus in patients were lower than those in healthy subjects. Regarding the analysis of the white matter microstructure, there were no differences in the fractional anisotropy and mean diffusivity values between the two groups., Conclusions: We have identified a reorganization of network hubs in the brain network of patients with reflex syncope. These alterations in brain network may play a role in the pathophysiologic mechanism underlying reflex syncope., (© 2018 The Authors. Brain and Behavior published by Wiley Periodicals, Inc.)

A 14-year-old intact male West Highland White Terrier weighing 6.9 kg was admitted to the Tokyo University of Agriculture and Technology Animal Medical Center with the complaint of syncope after showing signs of nausea during feeding. Sinus arrest induced by deglutition was confirmed using a Holter electrocardiography test. However, the clinical symptoms significantly improved after implantation of a permanent pacemaker. Seven months after implantation, the dog died from acute pancreatitis, a cause unrelated to the syncope. Immediately after its death, the heart, lungs, gastrointestinal tract, and other organs were dissected and examined histopathologically. The brain was also examined using magnetic resonance imaging. Examination results led to the diagnosis of swallowing-induced situational syncope.

Objectives: To evaluate the ability of late gadolinium enhancement (LGE) and mapping cardiac magnetic resonance (CMR) including native T1 and global extracellular volume (ECV) to identify hypertrophic cardiomyopathy (HCM) patients at risk for sudden cardiac death (SCD) and to predict syncope or non-sustained ventricular tachycardia (VT)., Methods: A 1.5-T CMR was performed in 73 HCM patients and 16 controls. LGE size was quantified using the 3SD, 5SD and full width at half maximum (FWHM) method. T1 and ECV maps were generated by a 3(3)5 modified Look-Locker inversion recovery sequence. Receiver-operating curve analysis evaluated the best parameter to identify patients with increased SCD risk ≥4% and patients with syncope or non-sustained VT., Results: Global ECV was the best predictor of SCD risk with an area under the curve (AUC) of 0.83. LGE size was significantly inferior to global ECV with an AUC of 0.68, 0.70 and 0.70 (all P < 0.05) for 3SD-, 5SD- and FWHM-LGE, respectively. Combined use of the SCD risk score and global ECV significantly improved the diagnostic accuracy to identify HCM patients with syncope or non-sustained VT., Conclusions: Combined use of the SCD risk score and global ECV has the potential to improve HCM patient selection, benefiting most implantable cardioverter defibrillators., Key Points: • Global ECV identified the best HCM patients with increased SCD risk. • Global ECV performed equally well compared to a SCD risk score. • Combined use of the SCD risk score and global ECV improved test accuracy. • Combined use potentially improves selection of HCM patients for ICD implantation.

Objectives: To determine, using systematic review of the biomedical literature, whether pacing reduces risk of recurrent syncope and relevant clinical outcomes among adult patients with reflex-mediated syncope., Methods: MEDLINE (through PubMed), EMBASE, and the Cochrane Central Register of Controlled Trials (through October 7, 2015) were searched for randomized trials and observational studies examining pacing and syncope, and the bibliographies of known systematic reviews were also examined. Studies were rejected for poor-quality study methods and for the lack of the population, intervention, comparator, or outcome(s) of interest., Results: Of 3188 citations reviewed, 10 studies met the inclusion criteria for systematic review, including a total of 676 patients. These included 9 randomized trials and 1 observational study. Of the 10 studies, 4 addressed patients with carotid sinus hypersensitivity, and the remaining 6 addressed vasovagal syncope. Among the 6 open-label (unblinded) studies, we found that pacing was associated with a 70% reduction in recurrent syncope (relative risk [RR]: 0.30; 95% confidence interval [CI]: 0.15-0.60). When the 2 analyzable studies with double-blinded methodology were considered separately, there was no clear benefit (RR: 0.73; 95% CI: 0.25-2.1), but confidence intervals were wide. The strongest evidence was from the randomized, double-blinded ISSUE-3 (Third International Study on Syncope of Uncertain Etiology) trial, which demonstrated a benefit of pacing among patients with recurrent syncope and asystole documented by implantable loop recorder., Conclusions: There are limited data with substantive evidence of outcome ascertainment bias, and only 2 studies with a double-blinded study design have been conducted. The evidence does not support the use of pacing for reflex-mediated syncope beyond patients with recurrent vasovagal syncope and asystole documented by implantable loop recorder., (© 2017 by the American College of Cardiology Foundation, the American Heart Association, Inc., and the Heart Rhythm Society.)

Objectives: Hypomagnesemia is common among hospitalized patients, particularly those who are critically ill. It can be associated with a number of potentially life-threatening cardiovascular, neurological and behavioral manifestations. As opposed to acute, chronic hypomagnesemia is often underdiagnosed and underreported and as such may pose a diagnostic and therapeutic problem., Case Presentation: We describe a case of magnesium wasting in a middle-aged woman with head and neck cancer who presented with recurrent syncopal episodes complicated by a femur fracture 4 months after completing a course of carboplatin-containing chemotherapy. Fractional excretion of magnesium of 16% was consistent with renal wasting of magnesium. After ruling out all common causes of hypomagnesemia, it was concluded that she sustained carboplatin-induced renal tubular damage making her relatively resistant to magnesium supplementation., Conclusion: Several antineoplastic agents have been linked to chronic hypomagnesemia including anti-epidermal growth factor receptor agents such as cetuximab and panitumumab, cyclosporine, and the platinum-based agents cisplatin and carboplatin. The example case presented here illustrates the importance of chronic hypomagnesemia and its possible debilitating effects following carboplatin-containing chemotherapy. A growing numbers of cancer survivors are treated with these antineoplastic agents, and are hospitalized for non-cancer-related problems. These patients may have prolonged hypomagnesemia, and hence pose a diagnostic dilemma. We review the pathophysiology, etiology, diagnosis, clinical manifestations, monitoring and treatment of hypomagnesemia, with special attention to mechanisms of renal damage caused by platinum-containing chemotherapeutic agents.

Purpose: We set out to determine clinical and EEG features of seizures presenting with falls, epileptic drop attacks and atonia in the video EEG monitoring unit., Methods: We searched the video EEG monitoring reports over a 5-year-period for the terms "drop", "fall" and "atonic"., Results: Seizures presenting as epileptic drop attacks, falls or atonia were found in 23/1112 (2%) admissions. About half of the patients suffering from these seizure types had developmental delay and learning difficulties and in half of the patients a lesion was seen on MRI which was often frontal. The presumed epileptogenic zone was frontal in many cases (43%), unclear with regards to a region or multifocal in 48% and posterior temporal/occipital in 2 patients (9%). EEG patterns recorded were paroxysmal fast activity, spike and wave discharges and EEG attenuation. Seizure related falls were seen in 8 cases (34%) with injuries recorded during Video EEG monitoring in half of those., Conclusion: Clinical and EEG features outlined here can help the clinician to recognise patients at risk for these devastating seizure types., (Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Background: The aim of this study was to investigate the correlation between autonomic dysfunction in multiple sclerosis (MS) and brainstem dysfunction evaluated with the vestibular evoked myogenic potentials (VEMP) score and conventional MRI., Methods: Forty-five patients with the diagnosis of clinically isolated syndrome (CIS) suggestive of MS were enrolled. VEMP, heart rate, and blood pressure responses to the Valsalva maneuver, heart rate response to deep breathing, and pain provoked head-up tilt table test, as well as brain and spinal cord MRI were performed., Results: There was no difference in the VEMP score between patients with and without signs of sympathetic or parasympathetic dysfunction. However, patients with syncope had significantly lower VEMP score compared to patients without syncope (p<0.01). Patients with orthostatic hypotension (OH) showed a trend of higher VEMP score compared to patients without OH (p=0.06). There was no difference in the presence of lesions in the brainstem or cervical spinal cord between patients with or without any of the studied autonomic parameters. The model consisting of a VEMP score of ≤5 and normal MRI of the midbrain and cervical spinal cord has sensitivity and specificity of 83% for the possibility that the patient with MS can develop syncope., Conclusions: Pathophysiological mechanisms underlying functional and structural disorders of autonomic nervous system in MS differ significantly. While preserved brainstem function is needed for development of syncope, structural disorders like OH could be associated with brainstem dysfunction., (Copyright © 2015 Elsevier B.V. All rights reserved.)

Psychogenic pseudosyncope (PPS) is the appearance of transient loss of consciousness (TLOC) in the absence of true loss of consciousness. Psychiatrically, most cases are classified as conversion disorder, which is hypothesized to represent the physical manifestation of internal stressors. The incidence of PPS is likely under-recognized and the disorder is under investigated in the unexplained syncope population, yet it can be diagnosed accurately with a focused history and confirmed with investigations including head-up tilt testing (HUTT), electroencephalogram (EEG; sometimes combined with video) or, in some centers, transcranial Doppler (TCD). Patients are more likely to be young females with an increased number of episodes over the past 6months. They frequently experience symptoms prior to their episodes including light-headedness, shortness of breath and tingling. Conversion disorder is associated with symptomatic chronicity, increased psychiatric and physical impairment, and diminished quality of life. Understanding the epidemiology, biological underpinnings and approach to diagnosis of PPS is important to improve the recognition of this disorder so that patients may be managed appropriately. The general treatment approach involves limiting unnecessary interventions, providing the patient with needed structure, and encouraging functionality. While there are no treatment data available for patients with PPS, studies in related conversion disorder populations support the utility of psychotherapy. Psychotropic medications should be considered in patients with comorbid psychiatric disorders., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: The incidence of syncope increases in individuals over the age of 70 years, but data about this condition in the elderly are limited. Little is known about tilt testing (TT), carotid sinus massage (CSM), or supine and upright blood pressure measurement related to age or about patients with complex diagnoses, for example, those with a double diagnosis, ie, positivity in two of these three tests., Methods: A total of 873 consecutive patients of mean age 66.5±18 years underwent TT, CSM, and blood pressure measurement in the supine and upright positions according to the European Society of Cardiology guidelines on syncope.1 Neuroautonomic evaluation was performed if the first-line evaluation (clinical history, physical examination, electrocardiogram) was suggestive of neurally mediated syncope, or if the first-line evaluation was suggestive of cardiac syncope but this diagnosis was excluded after specific diagnostic tests according to European Society of Cardiology guidelines on syncope, or if certain or suspected diagnostic criteria were not present after the first-line evaluation., Results: A diagnosis was reached in 64.3% of cases. TT was diagnostic in 50.4% of cases, CSM was diagnostic in 11.8% of cases, and orthostatic hypotension was present in 19.9% of cases. Predictors of a positive tilt test were prodromal symptoms and typical situational syncope. Increased age and a pathologic electrocardiogram were predictors of carotid sinus syndrome. Varicose veins and alpha-receptor blockers, nitrates, and benzodiazepines were associated with orthostatic hypotension. Twenty-three percent of the patients had a complex diagnosis. The most frequent association was between vasovagal syncope and orthostatic hypotension (15.8%); 42.9% of patients aged 80 years or older had a complex diagnosis, for which age was the strongest predictor., Conclusion: Neuroautonomic evaluation is useful in older patients with unexplained syncope after the initial evaluation. A complex neurally mediated diagnosis is frequent in older people. Our results suggest that complete neuroautonomic evaluation should be done particularly in older patients.

Brain herniations into dural venous sinuses (DVS) are rare findings recently described and their etiology and clinical significance are controversial. We describe five patients with brain herniations into the DVS or calvarium identified on MRI, and discuss their imaging findings, possible causes, and relationship to the patient's symptoms. All patients were examined with MRI including high resolution pre- and post-contrast T1- and T2-weighted sequences. With respect to brain herniations we documented their locations, signal intensities in different sequences, and size. We then reviewed clinical records in an attempt to establish if any symptoms were related to the presence of these herniations. Three males and two females were examined (age range, 11-68 years). Three patients had unilateral temporal lobe herniations into transverse sinuses, one had a cerebellar herniation into the skull, and one had bilateral temporal lobe herniations into the transverse sinuses as well as a cerebellar herniation into the sigmoid sinus. In all, the herniated brain and surrounding cerebrospinal fluid (CSF) had normal signal intensity on all MRI sequences. When correlated with clinical symptoms, brain herniations were thought to be incidental and asymptomatic in three patients and two patients had histories of headaches. Brain herniations with surrounding CSF into the DVS/skull should be considered potential sources of filling defects in the DVS. We believe that they are probably incidental findings that may be more common than previously recognized and should be not confused with the more common arachnoid granulations, clots, or tumors. Two patients had headaches, but their relation to the presence of herniated brain was uncertain.

A 41 year-old African-American male presented with syncope preceded by shortness of breath at outside facility and transferred to us for management of extensive pulmonary embolism with unstable vital signs. Electrocardiogram showed sinus tachycardia with S1Q3T3 pattern. A transthoracic echocardiogram revealed a freely mobile strand like mass in the left atrium. A transoesophageal echocardiogram showed a very large freely mobile thrombus extending from a patent foramen ovale in to the left atrium. He underwent emergent surgery for the extraction of clot followed by thromboembolectomy from both pulmonary arteries. He made a remarkable recovery and was discharged after seven days of hospital stay., (Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Although it is not uncommon for patients with Chiari I malformations to present with respiratory complaints, cough syncope is a rare presenting symptom. We report an adult patient who had both a Chiari I malformation and atlanto-occipital assimilation, and complained of cough syncope, orthopnea, and central sleep apnea. The patient underwent decompressive craniectomy of the posterior fossa and a cervical level 2 laminectomy. However, due to an initial under-appreciation of the profound narrowing of the foramen magnum as a result of these concomitant pathologies, the patient had continued impaired cerebrospinal fluid flow, leading to a symptomatic pseudomeningocele and required a more extensive decompression that included a cervical level 3 laminectomy as well as a temporary lumbar drain. On 2 year follow-up, he remained asymptomatic., (Published by Elsevier Ltd.)

Objectives: We and others showed that migraineurs are at increased risk of subclinical and clinical ischemic brain lesions. Migraineurs also have a higher prevalence of frequent syncope and orthostatic intolerance, symptoms that are associated with transient reductions in cerebral blood flow. In this study, we assessed whether these autonomic symptoms may contribute to the increased risk of brain lesions in migraine., Methods: Migraineurs (n = 291) and controls (n = 140) from the population-based, cross-sectional CAMERA (Cerebral Abnormalities in Migraine, an Epidemiologic Risk Analysis) cohort (aged 30-60 years, and free of other neurologic symptoms) underwent 1) brain MRI scan, and 2) structured telephone interview including questions on frequent syncope (≥5/lifetime) and orthostatic intolerance., Results: Frequent syncope (odds ratio [OR] = 2.7; 95% confidence interval: 1.3-5.5) and orthostatic intolerance (OR = 2.0 [1.1-3.6]) were independent risk factors for high load of deep white matter lesions. Effects were strongest in women and similar in migraineurs and controls. Migraine diagnosis did not mediate or moderate these associations. Individuals with orthostatic intolerance had higher prevalence of high periventricular white matter lesion load (OR = 1.9 [1.1-3.5]). Syncope and orthostatic intolerance were not related to subclinical infarcts or infratentorial lesions., Conclusions: Frequent syncope, orthostatic intolerance, and migraine independently increase the risk of white matter lesions, particularly in females.

Children and adults with pulmonary arterial hypertension (PAH) have similarities and differences in their background characteristics, hemodynamics, and clinical manifestations. Regarding genetic background, mutations in BMPR2-related pathways seem to be pivotal; however, it is likely that other modifier genes and bioactive mediators have roles in the various forms of PAH in children and adults. In pediatric PAH, there are no clear sex differences in incidence, age at onset, disease severity, or prognosis but, as compared with adults, syncope incidence, pulmonary vascular resistance, and mean pulmonary artery pressure are higher, and vasoreactivity to acute drug testing is more frequent, among children. Nevertheless, the pharmacokinetic effects of 3 major pulmonary vasodilators appear to be similar in children and adults with PAH. This review focuses on the specific pathophysiologic features of PAH in children.

Lower cranial nerve schwannomas are benign tumors of the neurolemmocytes of the cranial nerves. Among children, cranial nerve schwannomas are extremely rare and are predominantly associated with neurofibromatosis (NF) type 2. The purpose of the current case report is to describe a unique giant extra- and intracranial foramen jugular schwannoma in a young boy with lower cranial nerve deficits and glossopharyngeal neuralgia syncope syndrome and to review the pertinent literature. In the current case report, we illustrate the course of disease in a 14-year-old boy with a 4-month history of recurrent syncope and a big bulge on the left side of his neck. Audiometry showed deafness of the left ear. Magnetic resonance imaging (MRI) demonstrated a giant unilateral dumbbell-shaped intra- and extracranial foramen jugular schwannoma with a volume of 156 cm3 causing severe brain stem compression and obstructive hydrocephalus. The tumor was removed completely in a two-step surgery. The tumor was confirmed during surgery to originate from the glossopharyngeal nerve. The histological examination revealed the characteristic features of a schwannoma. The MRI 3 months after the second surgery confirmed complete tumor removal. The genetic examination for NF was negative. Review of literature showed that dumbbell-shaped lower cranial nerve schwannomas in the childhood population are rare., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: 1. Evaluate the otolithic membrane in patients with endolymphatic hydrops (EH) and vestibular drop attacks (VDA) undergoing ablative labyrinthectomy. 2. Correlate intraoperative findings to archival temporal bone specimens of patients with EH., Study Design: Retrospective case review., Setting: Tertiary referral center. SPECIMEN SOURCE: 1. Patients undergoing labyrinthectomy for incapacitating Ménière's disease (MD), delayed EH, VDA, or acoustic neuroma (AN) between 2004 and 2011. 2. Archival temporal bone specimens of patients with MD., Interventions: Ablative labyrinthectomy., Main Outcome Measures: Examination of the utricular otolithic membrane., Results: The otolithic membrane of the utricle was evaluated intraoperatively in 28 patients undergoing labyrinthectomy. Seven (25%) had a history of VDA, 6 (21%) had delayed EH, 9 (32%) had MD, and 6 (21%) had AN. All patients with VDA showed evidence of a disrupted utricular otolithic membrane, whereas only 50% and 56% of patients with delayed EH and MD, respectively, demonstrated otolithic membrane disruption (p = 0.051). None of the patients with AN showed otolithic membrane disruption (p = 0.004). The mean thickness of the otolithic membrane in 5 archival temporal bone MD specimens was 11.45 micrometers versus 38 micrometers in normal specimens (p = 0.001)., Conclusion: The otolithic membrane is consistently damaged in patients with VDA. In addition, there is a significantly higher incidence of otolithic membrane injury in patients with MD and delayed EH compared with patients without hydrops, suggesting that the underlying pathophysiology in VDA results from injury to the otolithic membrane of the saccule and utricle, resulting in free-floating otoliths and atrophy.

Patients commonly present with syncope at emergency departments and other facilities for urgent care. Syncope is understood by physicians to be a transient, self-terminating period of cerebral hypoperfusion that usually results from systemic hypotension, and clinical guidelines for the care of patients with presumed syncope are available. However, the diagnosis and management of such patients continue to pose important diagnostic, therapeutic, and economic challenges, which are the focus of this Review. First, we discuss how to improve symptom characterization to distinguish syncope from other forms of transient loss of consciousness and syncope mimics. Second, we compare methods of risk stratification in patients with suspected syncope, and recommend the introduction of syncope clinics with enhanced interdisciplinary collaboration to optimize patient care at reduced expense. Third, we highlight the importance of the appropriate selection of diagnostic tools and treatment strategies in these syncope clinics. Finally, we address the difficulties associated with therapy for the most-common form of syncope--vasovagal or reflex syncope.

Background: Myocarditis (MC) is an important cause of cardiac dysfunction in children. Fulminant MC is sometimes fatal, and sequelae may develop during follow-up. We conducted a nationwide survey to determine the clinico-epidemiological features of MC in Japanese children and adolescents., Methods and Results: Survey questionnaires were mailed to 627 hospitals, which were asked if they had treated MC patients aged between 1 month and 17 years during the period from January 1997 through December 2002. Responses were collected until December 2005, and data were collected and analyzed until January 2008. A total of 169 patients were reported: 64 fulminant cases, 89 acute cases, and 8 chronic cases. Incidence was 43.5 cases/year and 0.26 cases/100,000. Pathogens were identified in 37 patients; coxsackie virus accounted for 60%. Major cardiovascular manifestations at onset were congestive heart failure, refractory arrhythmia, and syncope in 70, 37, and 17 patients, respectively. Intravenous immunoglobulin was administered to 73 patients. Mechanical support seemed to be effective and life-saving. Among the 169 patients, 123 survived. Cardiovascular sequelae were reported in 49 patients., Conclusions: The survival rate for children with fulminant MC was disappointing. Overall, two-thirds of survivors had no sequelae. Mechanical support may reduce the mortality and the risk of clinical worsening.

Background: It is often reported that clinical symptoms are useful in differentiating cardiac from non-cardiac syncope. Studies in the young are rare. This study was designed to capture the symptoms and signs reported by patients with cardiac syncope before the patients or their attending clinicians knew the final diagnosis., Methods: Retrospective case-note review of 35 consecutive unrelated gene-positive probands with a proven cardiac channelopathy., Results: The presentation leading to diagnosis of cardiac channelopathy was resuscitated sudden cardiac death in 7 patients; syncope in 20; collapse with retained consciousness in 2; palpitations in 1 and an incidental finding in 5. For the 20 patients with syncope (LQTS 18, Brugada syndrome 2), median age at presentation was 13.9 years (1.8 day to 40.8 years). Of the 17 patients able to describe the onset of syncope, 11 (65%) had at least one symptom prior to collapse, though none reported nausea. Dizziness or lightheadedness was the most frequent symptom, being experienced by 8 (47%). Nine (of 20) patients (45%) had witnessed seizure-like activity and 8 (40%) had urinary incontinence. Nineteen patients were capable of describing the post-syncopal period, of whom 15 (79%) reported symptoms, the most common (12; 65%) being drowsiness or exhaustion., Conclusions: Cardiac syncope in the young frequently presents with symptoms and signs that are typically associated with other causes of transient loss of consciousness, including vasovagal syncope and seizure disorders. The presence of symptoms may not be as helpful in differentiating arrhythmic from non-arrhythmic events as is often supposed. A thorough history, appropriate investigations and a high index of suspicion remain essential in the assessment of syncope., (Copyright © 2011 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier B.V. All rights reserved.)

Objectives: The aim of this study was to investigate the clinical characteristics and the long-term course of a large cohort of patients with short QT syndrome (SQTS)., Background: SQTS is a rare channelopathy characterized by an increased risk of sudden death. Data on the long-term outcome of SQTS patients are not available., Methods: Fifty-three patients from the European Short QT Registry (75% males; median age: 26 years) were followed up for 64 ± 27 months., Results: A familial or personal history of cardiac arrest was present in 89%. Sudden death was the clinical presentation in 32%. The average QTc was 314 ± 23 ms. A mutation in genes related to SQTS was found in 23% of the probands; most of them had a gain of function mutation in HERG (SQTS1). Twenty-four patients received an implantable cardioverter defibrillator, and 12 patients received long-term prophylaxis with hydroquinidine (HQ), which was effective in preventing the induction of ventricular arrhythmias. Patients with a HERG mutation had shorter QTc at baseline and a greater QTc prolongation after treatment with HQ. During follow-up, 2 already symptomatic patients received appropriate implantable cardioverter defibrillator shocks and 1 had syncope. Nonsustained polymorphic ventricular tachycardia was recorded in 3 patients. The event rate was 4.9% per year in the patients without antiarrhythmic therapy. No arrhythmic events occurred in patients receiving HQ., Conclusions: SQTS carries a high risk of sudden death in all age groups. Symptomatic patients have a high risk of recurrent arrhythmic events. HQ is effective in preventing ventricular tachyarrhythmia induction and arrhythmic events during long-term follow-up., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Favism is an acute hemolytic syndrome occurring in glucose-6-phosphate dehydrogenase (G6PD)-deficient individuals after the consumption of fava beans. The highest incidence is in boys aged 2-6 years. We report a 56-year-old man presented to the emergency department (ED) with recurrent syncope attacks due to favism. In our knowledge, this is the first report of favism-caused syncope in an adult patient without a G6PD deficiency diagnosis in the past and diagnosed in ED.

Isolated idiopathic right ventricular dilated cardiomyopathy is rare and is a diagnosis by exclusion. There is a distinct male predominance. The usual clinical presentations are syncope, ventricular tachycardia, left bundle branch block on ECG and right heart failure. Diagnosis is usually established based on the clinical and laboratory parameters. Confirmation of the same is done by studying the pathological features of the heart in an endomyocardial biopsy or at post-mortem. Herein we report a case of this rare entity which was diagnosed clinically and was further confirmed at autopsy.

Objective: Traditional electroencephalogram (EEG) recorders reject low frequencies and DC and therefore cannot handle fullband EEG. Dedicated fullband recorders use non-standard file formats, because the standard format (EDF) cannot handle large DC electrode offset voltages. Both facts limit the development and use of fullband EEG. We developed a modification that allows conventional equipment to record fullband EEG, and adapts both types of recorders to EDF., Methods: The modification is a simple filter that attenuates the DC component and thus makes the EEG fit within traditional equipment limitations and EDF. The review software automatically 'de-attenuates' the DC component, without loss of information., Results: DC attenuation by a factor of 10 made both types of recorders store DC attenuated fullband EEG into EDF files. Recordings were made during 0.5-24h in 46 subjects. The DC de-attenuator automatically reconstructed the original fullband EEG within an amplitude range of ±100mV and with a resolution of 0.3μV. Using sintered Ag-AgCl electrodes attached with common procedures, reconstructed DC EEG in spontaneously moving subjects ranged between ±32mV., Conclusions: The modification works. Fullband recordings can now be analyzed by independent software, archived and exchanged., Significance: Any EEG system can be made to record fullband EEG into standard EDF., (Copyright © 2010 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Objective: To determine the use of brain CT as a routine diagnostic tool in patients presenting with syncope., Methods: From March 2006 to April 2008, 292 patients presented with a history of transient loss of consciousness to the emergency department at the King Hussein Medical Center, Amman, Jordan. A neurologist examined all investigated patients and brain scans were obtained. Patients were classified into 4 groups according to brain scan findings and their neurological examination., Results: Out of the 292 patients, 254 (86.9%) patients underwent brain scan, 38 (13%) patients were excluded. The first group included 203 (79.9%) patients, who had normal brain scan, and normal neurological examination. The second group included 10 (3.9%) patients all of which had abnormal brain CT scan findings related to their syncope, and also had abnormal neurological examination. The abnormal neurological findings can be attributed to their syncopal episode and abnormal brain CT findings. Classified into 3 groups based on their brain CT findings and their neurological examination on presentation. The third group included 39 (15.3%) patients who had abnormal brain scan with findings not related to their syncope and either normal or abnormal neurological examination not related and cannot be attributed to the abnormal head CT findings and their syncopal episode. The last group had 2 patients (0.7%) with abnormal scan and normal neurological examination., Conclusion: The use of brain scan as a routine diagnostic tool in patients with syncope is unjustifiable, unless there is an indication in the history or physical examination.

Subchondral and articular collapse following nontraumatic osteonecrosis of the femoral head is an important cause of osteoarthritis in patients between ages 20 and 40. Because hip arthroplasty in the younger population is prone to early wear and failure, it is paramount to prevent collapse once osteonecrosis is detected. Natural remodeling of the osteonecrotic areas by adjacent normal bone is predominated by osteoclastic resorption, which weakens the cancellous bone and allows microfractures to occur before full healing can take place. Current treatment modalities include core decompression and various adjuncts such as bone graft, mesenchymal stem cells and tantalum implants to provide structural integrity and to speed bony creeping substitution. Calcium phosphate cement has been reported in the treatment of fractures, especially depressed tibial plateau fractures. It is slow to resorb and is gradually replaced by bone, allowing prolonged support of periarticular fractures during healing. We hypothesize that calcium phosphate cement in conjunction with standard decompression of osteonecrotic femoral head lesions can prevent collapse., ((c) 2009 Elsevier Ltd. All rights reserved.)

Post-heart transplantation bradycardic syncope and arrest could be due to preferential rejection of the conduction system. We present six heart transplant patients with this presentation, two of whom died. The autopsy of one of those patients demonstrated severe rejection of the conduction system, with only mild rejection throughout the rest of the myocardium. We postulate that aggressive therapy for rejection and pacemaker placement may result in improved survival in heart transplant recipients with this clinical presentation.

Purpose: Some patients with neurally mediated reflex syncope may be misdiagnosed as epilepsy because myoclonic jerky movements are observed during syncope. The seizure-like activities during the head-up tilt test (HUT) have been rarely reported. The purpose of this study was to assess the characteristics of these seizure-like activities and evaluate whether there are differences in the clinical characteristics and hemodynamic parameters of patients with neurally mediated reflex syncope with and without seizure-like activities during HUT-induced syncope., Materials and Methods: The medical records of 1,383 consecutive patients with a positive HUT were retrospectively reviewed, and 226 patients were included in this study., Results: Of 226 patients, 13 (5.75%) showed seizure-like activities, with 5 of these (2.21%) having multifocal myoclonic jerky movements, 5 (2.21%) having focal seizure-like activity involving one extremity, and 3 (1.33%) having upward deviation of eye ball. Comparison of patients with and without seizure-like activities revealed no significant differences in terms of clinical variables and hemodynamic parameters during HUT., Conclusion: Seizure-like activities occurred occasionally during HUT-induced syncope in patients with neurally mediated reflex syncope. The seizure-like activities during HUT might not be related to the severity of the syncopal episodes or hemodynamic changes during HUT.

Neurocardiogenic syncope (NCS, simple fainting) is a common and typically benign familial condition, which rarely may result in traumatic injury or hypoxic convulsions. NCS is associated with emotional triggers, anxiety states and stress. However, the etiology of NCS, as a psychophysiological process, is poorly understood. We therefore investigated the relationship between NCS and brain anatomy. We studied a non-clinical sample of eighteen individuals with histories characteristic of NCS, and nineteen matched controls who had never fainted. We recorded fainting frequency, resting heart rate variability measures and anxiety levels. Structural T1-weighted magnetic resonance images (MRI) were acquired at 1.5 T. Associations between brain morphometry (regional gray and white matter volumes) and NCS, resting physiology and anxiety were tested using voxel-based morphometry (VBM). Compared to controls, NCS participants had lower regional brain volume within medulla and midbrain (a priori regions of interest). Moreover, across NCS individuals, lower gray matter volume in contiguous regions of left caudate nucleus predicted enhanced parasympathetic cardiac tone, fainting frequency and anxiety levels. Our findings provide preliminary evidence for a hierarchical anatomical basis to NCS. First, differences in the volume of brainstem centers supporting cardiovascular homeostasis may relate to constitutional predisposition to NCS. Second, differences in the structural organization of the caudate nucleus in NCS individuals may relate to fainting frequency via interactions between emotional state and parasympathetic control of the heart. These observations highlight the application of VBM to the identification of neurovisceral mechanisms relevant to psychosomatic medicine and the neuroscience of emotion.

We investigated the ability of mutual nonlinear prediction methods to assess causal interactions in short-term cardiovascular variability during normal and impaired conditions. Directional interactions between heart period (RR interval of the ECG) and systolic arterial pressure (SAP) short-term variability series were quantified as the cross-predictability (CP) of one series given the other, and as the predictability improvement (PI) yielded by the inclusion of samples of one series into the prediction of the other series. Nonlinear prediction was performed through global approximation (GA), approximation with locally constant models (LA0) and approximation with locally linear models (LA1) of the nonlinear function linking the samples of the two series, on patients with neurally mediated syncope and control subjects. Causality measures were evaluated in the two directions (from SAP to RR and from RR to SAP) in the supine (SU) position, in the upright position after head-up tilt (early tilt, ET) and after prolonged upright posture (late tilt, LT). While the trends for the GA, LA0 and LA1 methods were substantially superimposable, PI elicited better than CP the prevalence of causal coupling from RR to SAP during SU. Both CP and PI noted a marked decrease in coupling in both causal directions in syncope subjects during LT, documenting the impairment of cardiovascular regulation in the minutes just preceding syncope.

Dynamic left ventricular outflow tract (LVOT) obstruction is seen classically in hypertrophic cardiomyopathy. Cardiac amyloidosis can present with asymmetric hypertrophy that resembles hypertrophic cardiomyopathy, and, in some cases, with dynamic LVOT obstruction. The occurrence of syncope in such patients is not uncommon. The syncope is usually thought to be related to mechanisms other than LVOT obstruction, such as arrhythmias, conduction disturbances, orthostatic hypotension, or vasovagal effects associated with neuropathy.Herein, we report the case of a patient who had immunocyte-derived (primary AL-type) cardiac amyloidosis with the echocardiographic appearance of hypertrophic cardiomyopathy and evidence of LVOT obstruction that caused syncope. We were able to provoke and identify dynamic LVOT obstruction that produced presyncopal symptoms similar to those that typically occur in such patients spontaneously. Dynamic LVOT obstruction as a cause of syncope should be considered in patients who have cardiac amyloidosis and echocardiographic evidence of hypertrophic cardiomyopathy.

A 79-year-old woman with systemic sclerosis was admitted to our hospital because of syncope. On admission, electrocardiogram showed progression of intraventricular conduction defect. Chest radiograph showed marked cardiomegaly. Echocardiogram revealed deterioration of left ventricular systolic function. We suspected progressive myocardial disease with Stokes-Adams attack. When we were preparing a temporary pacemaker, paroxysmal atrioventricular block with asystole for 15 seconds and convulsion occurred. Electrophysiological study showed His-ventricular block and sinus node dysfunction. A permanent pacemaker was implanted. In systemic sclerosis, progression of ventricular conduction defect may warrant prompt electrophysiological study and prophylactic pacemaker implantation.

A 63-year-old man presented with recurrent syncopal attacks associated with swallowing and right-sided throat pain. Immediately after admission, he presented a 16-s asystolia. The patient's clinical history was unremarkable except for previous postimplant periodontitis. Several episodes of severe bradycardia and sinus pauses, always associated with painful deglutition, were recorded subsequently. X-ray orthopanthomography and magnetic resonance imaging of the neck confirmed several areas of periodontitis around the previous dental implants and right mastoid inflammation. A barium swallow and fibre-optic endoscopy also revealed a small sliding hiatus hernia and distal chronic oesophageal inflammation. Despite complete dental curettage, antibiotics and antigastro-oesophageal reflux therapy, only partial relief of the pain and incomplete resolution of the arrhythmic disorder were obtained after 3 weeks, and the patient underwent pacemaker implantation. At 1-month follow-up, however, he reported the complete relief of the throat pain; subsequent Holter monitoring showed normal sinus rhythm, without pacemaker-induced electrical activity.

Echocardiographic findings were reviewed for 106 patients (mean age 41.3 +/- 23.0 years, range 3 to 90 years, 61% female) referred for evaluation of unexplained syncope. Abnormal echocardiographic findings were seen in 36/106 (34%) patients, of which 12/106 (11%) may have an abnormality that contributed to symptoms. Abnormal echocardiographic findings (64 vs 6%, p < 0.01) and those possibly causing syncope (22 vs 0%, p < 0.05) were significantly more likely in the oldest tercile of patients compared with the youngest. No patient less than 35 years old had a possibly diagnostic abnormality.

A 31-year-old lady presented with anemia and syncope. Echocardiography revealed massive pericardial effusion with a right atrial mass. Transesophageal echocardiography, computed tomography and magnetic resonance imaging scans confirmed presence of a right atrial mass. Histopathology revealed a high grade angiosarcoma. Complete resection was done and the patient was referred to an oncology unit for further management. After three months the patient had extensive metastasis and succumbed to the disease. This case report highlights the clinical presentation, rapid and aggressive course of cardiac angiosarcomas.

Wide variability in patient selection, extent of callosal section and definition of successful outcome between studies make impact of corpus callosotomy on patients with medically refractory epilepsies difficult to interpret. Severe mental retardation is considered to be predictive of unfavorable seizure outcome after callosotomy. Very little attention has been paid on the influence of callosotomy on the psychosocial burden on the patients' families. We evaluated the seizure outcome, and parental perception about change in cognition and behavior of 17 children (median age 9.5 years, range 3.5-18 years) with severe mental retardation (IQ<30 in all, except one) and injurious drop attacks, who have completed >or=1-year postoperative follow-up after callosotomy. Nearly two-thirds of our patients had >or=90% reduction in drop attacks and generalized tonic-clonic seizures. In the one-stage total callosotomy group, 9 of 11 (82%) patients had favorable outcome, compared to 2 of the 6 (33%) in the partial callosotomy group. Absence of generalized epileptiform discharges on the 1-year postoperative EEG was significantly associated with a favorable seizure outcome. The mean duration of epilepsy prior to callosotomy tended to be shorter among patients with favorable seizure outcome. Postoperative complications were trivial and transient. Nearly three-fourths of the parents appreciated improvements in behavior and attentiveness of their children and were satisfied with the outcome. We conclude that, in children with severe mental retardation and injurious drop attacks, total callosotomy can be undertaken as a one-stage procedure with insignificant morbidity and results in highly favorable seizure outcome.

Hypertrophic cardiomyopathy (HCM) is an inherited disease with marked phenotypic variability that includes the extent of hypertrophy, the presence and severity of symptoms, and the natural history of the disease. Symptoms of impaired consciousness (syncope and pre-syncope) occur in approximately 15-25% of patients with hypertrophic cardiomyopathy (HCM). In young patients a history of recurrent syncope is associated with an increased risk of sudden death. Detailed investigations identify a probable mechanism in a minority of these, usually paroxysmal atrial fibrillation or ventricular tachycardia. In the majority, however, no likely mechanism is found despite extensive investigation. Although this may be the case, it is still of vital importance to exclude potentially treatable causes of syncope.