Sudden Unexpected Deaths Due to Intracranial Meningioma: Presentation of Six Fatal Cases,Review of the Literature,and A Discussion of the Mechanisms of Death

Deaths due to meningiomas are routinely diagnosed in clinical practice because this neoplasm tends to present with the typical progression of neurological deficits. On the other hand, sudden unexpected deaths due to meningiomas are rarely described in the literature. The study presents six fatal cases of previously undiagnosed intracranial meningiomas from the Cook County Medical Examiner's Office from 1998 to 2014. The most common explanation of the mechanism of sudden death due to intracranial neoplasms is a rapid increase in intracranial pressure produced by the mass effect of the neoplasm. Other mechanisms of death include acute intracranial and intratumoral hemorrhage, and benign neoplasms that grow in the vicinity of vital centers altering neural discharge in autonomic pathways leading to cardiac suppression or lethal arrhythmia. Forensic pathologists must keep in mind that sudden unexpected death caused by intracranial meningiomas, although extremely rare, may be encountered in the forensic setting.     

过敏性和冠心病猝死者心肌组织中肥大细胞类胰蛋白酶、脑利钠肽的表达

目的探讨肥大细胞类胰蛋白酶、脑利钠肽(brain natriuretic peptide,BNP)在过敏性猝死和冠心病猝死鉴别诊断中的意义。方法选取山西医科大学法医病理学教研室2010—2015年尸检案例心肌标本共30例,分为颅脑损伤致死组、过敏性猝死组、冠心病猝死组,每组各10例。采用免疫荧光染色和Western印迹法分析各组心肌组织肥大细胞类胰蛋白酶和BNP的表达。结果过敏性猝死组、冠心病猝死组心肌组织内肥大细胞类胰蛋白酶免疫荧光染色均出现阳性染色;三组间两两比较,表达差异均具有统计学意义(P0.05)。冠心病猝死组心肌组织内BNP的表达量高于过敏性猝死组、颅脑损伤致死组(P0.05),过敏性猝死组与颅脑损伤致死组之间差异无统计学意义(P0.05)。结论联合检测心肌组织内肥大细胞类胰蛋白酶、BNP有望为过敏性猝死和冠心病猝死的法医学鉴别诊断提供帮助。     

Adrenal hypofunction and sudden death

Adrenal insufficiency is an infrequent and easily overlooked cause of sudden death, often occurring in individuals being treated for other serious disease processes. Three cases are presented to demonstrate some of the clinical presentations encountered, and the symptoms and signs are discussed.     

Association of right coronary artery hypoplasia with sudden death in an eleven-year-old child

Congenital coronary artery abnormalities are a rare but well-documented cause of sudden and unexpected death in the pediatric age group. Most reported cases involve both an aberrant origin and course of the abnormal vessel. A case of unexpected death occurring in an otherwise healthy eleven-year-old boy, who had been previously investigated for sudden collapse, is described. The major finding at autopsy was marked disparity in diameter between the coronary arteries due to diffuse hypoplasia of the right coronary artery. This case demonstrates the importance of meticulous examination of the coronary artery system in cases of sudden death in childhood so that significant reduction in luminal cross section will be adequately documented. The possible role played by this finding in the etiology of sudden death is discussed.     

Is there progress in the autopsy diagnosis of sudden unexpected death in adults?

Sudden death is now currently described as natural unexpected death occurring within 1h of new symptoms. Most studies on the subject focused on cardiac causes of death because most of the cases are related to cardiovascular disease, especially coronary artery disease. The incidence of sudden death varies largely as a function of coronary heart disease prevalence and is underestimated. Although cardiac causes are the leading cause of sudden death, the exact incidence of the other causes is not well established because in some countries, many sudden deaths are not autopsied. Many risk factors of sudden cardiac death are identified: age, gender, heredity factors such as malignant mutations, left ventricular hypertrophy and left ventricle function impairment. The role of the police surgeon in the investigation of sudden death is very important. This investigation requires the interrogation of witnesses and of the family members of the deceased. The interrogation of physicians of the rescue team who attempted resuscitation is also useful. Recent symptoms before death and past medical history must be searched. Other sudden deaths in the family must be noted. The distinction between sudden death at rest and during effort is very important because some lethal arrhythmia are triggered by catecholamines during stressful activity. The type of drugs taken by the deceased may indicate a particular disease linked with sudden death. Sudden death in the young always requires systematic forensic autopsy performed by at least one forensic pathologist. According to recent autopsy studies, coronary artery disease is still the major cause of death in people aged more than 35 years. Cardiomyopathies are more frequently encountered in people aged less than 35 years. The most frequent cardiomyopathy revealed by sudden death is now arrhythmogenic right ventricular cardiomyopathy also known simply as right ventricular cardiomyopathy (RVC). The postmortem diagnosis of cardiomyopathies is very important because the family of the deceased will need counseling and the first-degree relatives may undergo a possible screening to prevent other sudden deaths. In each case of sudden death, one important duty of the forensic pathologist is to inform the family of all autopsy results within 1 month after the autopsy. Most of the recent progress in autopsy diagnosis of sudden unexpected death in the adults comes from molecular biology, especially in case of sudden death without significant morphological anomalies. Searching mutations linked with functional cardiac pathology such as long-QT syndrome, Brugada syndrome or idiopathic ventricular fibrillation is now the best way in order to explain such sudden death. Moreover, new syndromes have been described by cardiologists, such as short-QT syndrome and revealed in some cases by a sudden death. Molecular biology is now needed when limits of morphological diagnosis have been reached.     

TB-related sudden death (TBRSD) due to myocarditis complicating miliary TB: a case report and review of the literature

TB-related sudden death (TBRSD) is rarely reported in the literature and in the majority of cases is due to bronchopneumonia and hemoptysis. Cardiac complications of tuberculosis causing sudden death can take many forms and are rarer still, with only a handful of cases reported. We describe a case of a previously fit and healthy 20-year-old Asian female who, after returning from a holiday in India, collapsed while getting off a bus. At postmortem, the only macroscopic finding of note was a localized area of fibrosis on the anterior wall of the left ventricle. Microscopic examination of this area showed Langhans giant cells; noncaseating epithelioid granulomas and acid-fast bacilli were demonstrated on Ziehl Nielsen staining. In addition, the lungs, liver, and kidneys contained multiple noncaseating granulomas. The case serves to highlight the protean nature in the presentation of this disease and the importance of postmortem histology in autopsy work.     

Sudden, unexpected death due to undiagnosed frontal glioblastoma in a schizophrenic patient

The incidence of sudden death due to undiagnosed primary intracranial tumor is low in forensic autopsy. We report a case of a 48-year-old white male, known to be a schizophrenic patient for several years, and in whom a medico-legal autopsy disclosed a large, previously undiagnosed, bilateral frontal glioblastoma infiltrating the genu of corpus callosum. We emphasize the importance of performing complete autopsy, including a thorough neuropathological examination, in all cases of sudden unexpected death, especially in those cases in which no extracerebral cause of death had been established and whose clinical history was primarily of a psychiatric nature.     

6例甲状腺功能亢进性心脏病猝死法医学分析

目的分析甲状腺功能亢进性心脏病(以下简称甲亢性心脏病)猝死案例,探讨其死亡的一般情况及法医病理学特点,为此类案件的法医病理学鉴定提供参考。方法收集中国医科大学法医学院2001—2016年6例甲亢性心脏病猝死案例,回顾性分析基本信息(性别与年龄)、临床表现、病史、解剖所见和组织病理学所见、生物化学检测指标、死亡原因。结果 6例案例多具有明确的甲状腺功能亢进病史,并表现出不同程度的心血管病症状;均具有明显的死亡诱因;甲状腺病理学检验符合弥漫性毒性甲状腺肿的表现;心脏质量均增加,心腔扩张,心肌肥大,灶状坏死;死后心包液的生物化学检测可作为甲亢性心脏病猝死的辅助手段。结论对甲亢性心脏病猝死案例进行诊断时应参考临床病史、尸体检验、组织病理学检验、死后毒(药)物检验等结果综合判定,必要时进行死后甲状腺和心功能的生物化学检测。     

Sudden unexpected death due to a previously undiagnosed plasma cell dyscrasia

The plasma cell dyscrasias are a diverse group of disorders characterized by the production of a clonal paraprotein. Sudden death is a recognized complication of the plasma cell dyscrasias, most commonly in individuals with cardiac involvement by amyloidosis. However, the current forensic literature has no reported cases in which sudden death resulted from complications of a plasma cell dyscrasia that was first diagnosed by postmortem histologic examination. We present the case of a woman whose sudden and unexpected death resulted from a seizure. Postmortem examination revealed no evidence of trauma or a grossly identifiable natural disease process that would have accounted for her death. However, microscopic and immunohistologic studies revealed a previously undiagnosed plasma cell dyscrasia, the clonality of which was determined by immunohistochemical studies for immunoglobulin light chains, that was not associated with amyloid deposition. This case elucidates a previously unrecognized cause of sudden unexpected death and illustrates the importance of microscopic studies in selected cases examined in medical examiner/coroner offices.     

Neoplastic disease in a medicolegal autopsy material. A retrospective study in northern Sweden

Only a small fraction of sudden unexpected deaths are caused by neoplastic disease and thus subject ot medicolegal autopsy. The medicolegal autopsy forms an opportunity to study not only medically diagnosed and treated neoplasms, but also the natural evolution of untreated disease. In a series of 7,020 consecutive medicolegal autopsies in northern Sweden, we found 171 cases with malignant and/or intracranial neoplasms. In 41 cases, sudden death was caused by previously unknown tumors. The most common mechanisms of death in this group were disseminated cancer, intracranial tumors, pulmonary thromboembolism, hemoptysis, and aspiration of blood, and the most common locations were the bronchi and the lung. In some of these cases, the mechanism was sometimes dramatic, raising a question of violent death or intoxication. In 30 cases, sudden unexpected death was caused by previously known tumors, and also in this group disseminated cancer was the most common cause of death, and the most common locations were the bronchi and the lung. In 22 cases, tumors were found suicidal cases; in 14 of these, the tumor was considered to be a major causative factor to the suicide, while in eight cases the tumor was considered to be an incidental finding. The expected number of cancers in the 1,060 suicides investigated in this series was 27, according to the official cancer prevalence data. Thus, a possible over-representation of suicides among persons with cancer seems doubtful and needs further exploration.     

A Case of Sudden Infant Death Due to Incomplete Kawasaki Disease

Although Kawasaki disease (KD) is a self‐limiting disease, it may cause sudden cardiac death. Diagnosis of KD is principally based on clinical signs; however, some infant cases do not meet the criteria. Such cases are identified as incomplete KD. The sudden death risk in incomplete KD cases is similar to conventional KD. In our 5‐month‐old case, he had been admitted to a hospital for a fever and suppuration at the site of Bacille de Calmette et Guerin (BCG) vaccination. However, after discharge from the hospital, his C‐reactive protein (CRP) levels declined, he got indisposed and died suddenly. A medico‐legal autopsy revealed myocarditis, coronaritis, platelet‐aggregated emboli in coronary arteries, and myocardial degeneration, suggesting that the fatal myocardial infarction was due to thrombus emboli in the coronary arteries. Forensic pathologists therefore should pay attention to the cardiac pathology originated from incomplete KD as a potential cause in cases of sudden infant death.     

Mallory-Weiss syndrome as the cause of sudden, unexpected death

The Mallory-Weiss-syndrome is an upper gastrointestinal haemorrhage due to longitudinal mucosal lacerations in the oesophagogastric junction. The mucosal lacerations occur as a sequel of vomiting or any other increase in intraabdominal pressure and account for up to 15% of all upper gastrointestinal bleedings. If death is due to a bleeding Mallory-Weiss-tear, however, massive blood stains on the corpse and around it frequently give rise to the suspicion of an unnatural cause of death. For this reason, autopsy is usually indispensable to elucidate the circumstances in which death occurred. The authors carried out a retrospective analysis of 5958 autopsies performed between 1997 and 2001 at the Institute of Legal Medicine, University of Hamburg, in order to identify all cases of Mallory-Weiss-syndrome as cause of sudden, unexpected death. The results (9 cases, amounting to 0.15% of all autopsies) suggest that Mallory-Weiss-syndrome is probably much more common as a cause of sudden death than previously described. Regarding epidemiological aspects, men clearly predominate; the average age was 48 years. It should also be stressed that in 8 of the 9 cases there was a previous history of chronic alcohol abuse.     

Evaluation of diagnostic tools applied in the examination of sudden unexpected deaths in infancy and early childhood

During the period between 1984 and 1999, 309 cases of sudden unexpected death in infancy and early childhood (0-3 years) were investigated at the Institute of Forensic Medicine in Oslo. In 73 cases, an explainable cause of death was found. In this non-sudden infant death syndrome (SIDS) group, 42 cases were due to disease, 14 to accidents, 7 to neglect/abuse and 10 cases were due to homicide. In 43 cases, there were pathological findings at the autopsy or suspect features in the history and/or circumstances, which were, however, insufficient to explain death ("borderline" SIDS). In the remaining 193 cases, nothing of significance was detected ("pure" SIDS).The purpose of the present study was to evaluate the importance of the different diagnostic tools used in diagnosing non-SIDS and borderline SIDS cases. The definition of SIDS requires a negative history as well as a negative autopsy result. Thus, the following variables were analysed: circumstances, medical history and autopsy, which included a gross pathological investigation, histology, neuropathology, microbiology, radiology and toxicology. In diagnosing deaths due to disease, histology, neuropathology and microbiology were the most important diagnostic tools. In contrast, information about the circumstances of death and the gross pathological findings at autopsy most often revealed the cause of death in accidents and cases of neglect/abuse and homicide.Following the drop in SIDS rate in Norway after 1989, the share of pure SIDS in proportion to the total population of sudden unexpected deaths in infancy and early childhood has decreased. The increasing proportion of non-SIDS and borderline SIDS cases presents a challenge to improve the quality of the investigation in cases of sudden death in infancy and early childhood.     

Sudden Death Due to Recent Hemorrhage From an Intrathoracic Chronic Expanding Hematoma

Chronic expanding hematoma (CEH) is a rare disease that can develop in any region of the body, but it most frequently develops in the thorax. When intrathoracic CEH is left untreated, gradually expanding hematoma can be life‐threatening, leading to respiratory failure or hemoptysis. We encountered an 89‐year‐old man with cardiopulmonary arrest on arrival. He had been healthy, and it was unclear whether CEH had previously been detected. A very large mass was observed on chest computed tomography (CT), but the cause of death could not be determined. In the autopsy, this mass was identified as CEH and no malignant findings were noted. A fresh hemorrhage had occurred in the hematoma and perforated the bronchial lumen, which caused airway obstruction/asphyxia and resulted in sudden death. CEH should be suspected when a very large tumorous lesion occupying the entire hemithorax is observed on chest imaging, and it is important to recognize that sudden death can occur in the natural course of CEH.     

原发性心肌病猝死心肌mtDNA~(4977)缺失变化

目的探讨原发性心肌病(PCM)猝死者心肌线粒体DNA(m tDNA4977)缺失情况及其与猝死的关系。方法对18例PCM猝死和28例对照组病例心肌组织蜡块,用常规方法提取心肌m tDNA,以PCR、琼脂糖紫外凝胶成像技术确定扩增产物激光密度,初步定量检测m tDNA4977缺失率。结果PCM猝死18例中,检见13例m tDNA4977缺失,占72.44%。对照组28例中,检见3例m tDNA4977缺失,占10.71%;两组病例m tDNA4977缺失率均值分别为0.5795和0.0744,差异有非常显著性意义。结论多数PCM,特别是扩张型心肌病猝死者心肌可检见m tDNA4977缺失;提示其心肌m tDNA4977缺失变化与PCM猝死的发生可能有一定关系。     

Sudden death due to unsuspected coronary vasculitis.

Coronary artery vasculitis is a well-recognized complication of polyarteritis nodosa and is occasionally seen in other forms of systemic vasculitis. However, involvement of the major epicardial coronary arteries leading to myocardial infarction and death is uncommon. Isolated coronary arteritis is even more rare. We report three cases of sudden death due to myocardial ischemia associated with arteritis of the major coronary arteries. All three decedents were previously healthy young to middle-aged men who had died suddenly after complaints of chest pain and shortness of breath. The autopsy findings and differential diagnoses are presented. Such cases are of particular interest to the medical examiner because of the sudden, unexpected nature of the deaths. An approach to the correct diagnosis is discussed.     

Sudden death due to an unrecognized cardiac hydatid cyst: three medicolegal autopsy cases

Echinococcosis is a human infection caused by the larval stage of Echinococcocus granulosus. The most common sites of infection are the liver and the lungs. Cardiac hydatid cysts are very rare, even in regions where hydatic cysts are endemic (the Mediterranean, South America, Africa, and Australia). It has been reported that cardiac involvement is seen in about 0.5-3% of human echinococcosis cases. Three cases of cardiac hydatid disease that caused sudden death and which were histopathologically diagnosed are reported. Cardiac echinococcosis is rare, but due to its insidious presentation and affinity to cause sudden death, it is important that it be identified in the histopathological examination.     

Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old Boy

Abstract:  Sickle cell disease (SCD) and sickle cell trait (SCT) can be associated with sudden unexpected death in the pediatric population, usually due to pulmonary complications occurring within the acute chest syndrome (ACS). Musculoskeletal complications can occur and are classically limited to bone infarcts. The occurrence of bone pathology centered upon the epiphyseal growth plate in SCD/SCT is extremely rare, and multiple such injuries in a single patient have not been previously reported. Herein, we describe a case of sudden unexpected death in a 5-year-old child with undiagnosed SCT due to the ACS, with widespread epiphyseal and periosteal bone lesions mimicking multiple inflicted injuries at autopsy. This case highlights the importance of clinicopathological correlation and is the first to describe SCT pathology as a mimic of nonaccidental injury.     

Cardiac pathological study of sudden death due to myocarditis: analysis of 17 cases

17 cases of sudden death due to myocarditis were presented. The problem of the classification and mechanism of sudden death is discussed. It is concluded that heart function failure is the main reason for the sudden death of myocarditis.     

Sudden, unexpected death due to fourth ventricular subependymoma

A case of sudden, unexpected death due to fourth ventricular subependymoma (subependymal glomerate astrocytoma) in a 60-year-old man is presented. Sudden, unexpected death due to uncomplicated subependymoma has not been previously described.