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.     

Sudden unexpected death in infancy and childhood due to undiagnosed neoplasia: an autopsy study

Sudden unexpected death due to clinically undiagnosed neoplasia in infancy and childhood (SUDNIC) is a rare phenomenon, with only small numbers of cases reported in the literature. In the majority of instances, the tumors involve critical structures within the heart or central nervous system and include gliomas, medulloblastomas, rhabdomyomas, and neoplasms of stromal elements. A 20-year retrospective review of autopsy records from the Hospital for Sick Children, Toronto, was performed (1984-2003, n = 4926), and 7 cases of SUDNIC were identified (0.14%). In addition, 1 case was obtained from the files of the Children's and Women's Health Centre of British Columbia, Vancouver. Diagnoses included 2 cases of acute leukemia (1 myelogenous, 1 lymphoblastic), 2 cases of mediastinal lymphoblastic lymphoma (pre-T cell type), 1 papillary fibroelastoma of the mitral valve prolapsing into and totally occluding the left anterior descending coronary artery, 1 medulloblastoma, 1 Wilms tumor associated with fatal intraperitoneal hemorrhage, and 1 widely disseminated gastric carcinoma. These cases demonstrate that infants and children may have minimal or no symptoms in the presence of significant disease and highlight the need for a thorough autopsy examination in cases of sudden unexpected death in infancy and childhood.     

Fatal ischemic stroke due to dissecting aneurysm of the intracranial arteries presenting as sudden unexpected death in childhood

Sudden unexpected death is any nontraumatic death in healthy individuals with normal activities for age until acute demise. Whereas not uncommon in adults and infants, it is considered a rarity in children, with most cases due to infection or occult cardiovascular abnormalities. Pathologic intracranial findings are rarely found in sudden unexpected death in childhood, with most cases due to occult intracranial neoplasms. Cerebral arterial dissection is an uncommon cause of arterial ischemic stroke in childhood. Outcome is poor, with more than 20% of patients dying. We report 4 cases of sudden unexpected death due to arterial ischemic stroke after cerebral arterial dissection in childhood and present a review of the literature.     

Acute pancreatitis presenting as sudden, unexpected death: an autopsy-based study of 27 cases

Acute pancreatitis represents a spectrum of disease, ranging from a mild, transitory illness to a severe, rapidly progressive hemorrhagic form, with massive necrosis and mortality rates of up to 24%. The reported incidence of acute pancreatitis diagnosed first at clinicopathologic autopsy ranges between 30% and 42%. To better describe outpatient fatalities due to acute pancreatitis that present as sudden, unexpected death, we retrospectively reviewed the autopsy files at the Institute of Legal Medicine, University of Hamburg, Germany, from 2000-2004. Individual cases were analyzed for sex, age, race, circumstances of death, social background of the deceased and previous medical history, seasonal occurrence of the disease, blood alcohol concentration at the time of death, body mass index, autopsy findings, histopathology, and etiology of acute pancreatitis. Among the 6178 autopsies carried out during the 5-year period evaluated, there were 27 cases of acute pancreatitis that presented as sudden, unexpected death. In all cases, the diagnosis was first made at autopsy. The male:female ratio was 1.7:1 and the mean age was 52 years (range, 30-91 years). Etiologies of acute pancreatitis included alcohol (n=19), gall stones (n=2), other identified etiologic factors (n=3), and idiopathic (n=3). Complications of acute pancreatitis included lung edema and/or acute respiratory distress syndrome, peritonitis, disseminated intravascular coagulation, and sepsis. At least 20 subjects (74%) had lived isolated, with no social contacts. Contrary to the clinical observations of a clear seasonal variation in the onset of acute pancreatitis, we found no correlation between death due to acute pancreatitis and a specific month or season. Many prior studies have suggested that the majority of deaths in severe acute pancreatitis occur in the late phase of the disease as a result of pancreatic sepsis. Conversely, in the present study, the majority of affected individuals died during the very early phase of the disease. While gallstones represent the main etiologic factor in most larger clinical series, biliary etiology seems to play only a minor role in outpatient deaths undergoing medicolegal autopsies. Data derived from medicolegal autopsy studies should be included in future population-based studies of acute pancreatitis.     

Sudden death resulting from lesions of the cardiac conduction system

Sudden unexpected deaths in young persons with noncontributory histories, autopsy results, and drug screen results are a common problem in forensic pathology. As part of the evaluation of such cases, the cardiac conduction system (CCS) should be studied. To determine the type and incidence of lethal CCS lesions, the authors reviewed their files of sudden unexpected cardiac deaths with particular attention to cases with causes of death in the conduction system. Cases of sudden cardiac death in patients aged < or=40 years during a 10-year period (Michigan) and a 4 year-period (Spain) were selected from the files. From this group, cases were identified in which the cause of death was a lethal change in the CCS. The portions of the heart containing the CCS were excised, and at least one hematoxylin and eosin slide and at least one trichrome or elastic trichrome slide per block were studied. In the two centers, 381 cases of sudden cardiac death were identified. The most common causes of sudden cardiac death were arteriosclerotic narrowing of the coronary arteries, cardiomyopathy, and myocarditis. In 82 cases, there was no identifiable cause of death even after complete gross and microscopic autopsy was performed, a medical history was obtained, and a drug screen was performed. In 11 cases, the CCS contained lesions that were considered lethal: narrowing of the atrioventricular node artery by fibromuscular hyperplasia (7 cases) and atrioventricular node tumors (4 cases). The 11 cases accounted for 2.9% of the 381 cases of sudden cardiac death and 11.8% of the indeterminable cases. It was concluded that examination of the CCS in deaths in which the gross and microscopic autopsy, history, and drug screen fail to provide a cause of death can yield a cause of death in a significant percentage of cases. If heart block was not documented during life and no explanatory lesions were found during routine cardiac examination, examination of the CCS can yield valuable information.     

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.     

The most severe chronic organ findings in official public health autopsies--a medico-sociologic contribution

At the Vienna Institute, in the year 1986, 79 carcinoma findings were recorded in 1736 cases of natural death. The most common were carcinomas of the respiratory and gastrointestinal tracts. Seventeen unidentified tumors were found to have been the cause of death. Public health autopsies, therefore, contribute to the clarification of tumor disease and thus to cancer statistics. Although diagnosed fatal cancer cases correspond to only 0.33% of all cancer deaths in Vienna, the question arises why a patient with very advanced cancer is not under medical care and why the affliction is revealed only in autopsy, the purpose of which is to elucidate sudden, unexpected deaths. The comparatively few individual cases should nevertheless give cause for reflection and are discussed from a medicosociological view, taking into consideration the statistical and epidemiological data relating to cancer diseases.     

Blood alcohol in sudden and unexpected deaths

Blood alcohol concentration was determined in 1672 sudden and unexpected natural and non-natural out-of-hospital deaths. The material covered all medicolegal autopsies in the province of Uusimaa which has a population of approximately 1.1 million inhabitants. In general, the prevalence of cases with alcohol in the blood at the time of death was high but varied considerably according to sex, age, and the cause and manner of death. The blood alcohol result was positive in 36% of the male and in 15% of the female material. In 59% of the alcohol-positive male and in 54% of the alcohol-positive female cases the actual concentrations were at least 1.5%. Acute use of alcohol was regarded as a significant condition contributing to death in 23% of the whole male and in 8% of the whole female material. These gross results and the details presented indicate that the acute use of excess alcohol is a factor contributing to non-natural but also to sudden and unexpected natural deaths to an extent that is not generally known. The results also emphasize that simple blood alcohol determination should be a routine procedure in the autopsy praxis of all sudden and unexpected natural and non-natural out-of-hospital deaths.     

Mitral valve prolapse syndrome as cause of sudden death in young adults

Mitral valve prolapse syndrome (MVPS) is a relatively common disorder of the mitral valve and most cases take a benign clinical course. Only a subset of patients develop severe clinical symptoms such as arrhythmia, insufficiency of the mitral valve or infective endocarditis. As a consequence, sudden death might occur in these patients, thought to be caused by an arrhythmogenic event. By presenting six cases of sudden unexpected death in young female adults, we point at clinical and pathological data from the literature, which are of interest from the viewpoint of legal medicine. The incidence of MVPS in autopsy series has been reported to be about 4-5%, while clinical data hint at an incidence of about 2.5%. The presented cases suggest that even clinically benign cases of MVPS in young adults might result in sudden unexpected death. Such cases are not included in hospital based studies on the topic. This might lead to an underestimation of the fatal risk associated with the disease, even if sudden death might be a rare event in MVPS.     

Sudden unexpected death in epilepsy: neuropathologic findings

Sudden unexpected death in epilepsy refers to sudden death of an individual with a clinical history of epilepsy, in whom a postmortem examination fails to uncover a gross anatomic, toxicologic, or environmental cause of death. Evidence of terminal seizure activity may not be present. One to two percent of natural deaths certified by the medicolegal death investigator are attributed to epilepsy. Detailed microscopic examination of the brain has increasingly afforded the identification of structural changes representative of epileptogenic foci. The authors present 70 cases of death attributed to sudden unexpected death in epilepsy. These cases were classified as follows: individuals who lacked a gross brain lesion, those who had a brain lesion demonstrable at autopsy, and those who lacked neuropathologic evaluation because of decomposition or because only an external examination was done. All of the subjects had a clinical history of seizures. The authors confirm that various microscopic findings, including neuronal clusters, increased perivascular oligodendroglia, gliosis, cystic gliotic lesions, decreased myelin, cerebellar Bergmann's gliosis, and folial atrophy, are present in a higher percentage of the brains of sudden unexpected death in epilepsy subjects than in the brains of age- and sex-matched control subjects.     

Amniotic fluid embolism-apropos two consecutive cases

Amniotic fluid embolism (AFE) is a sporadic, unpredictable, and usual fatal obstetric complication. The paper deals with two cases of maternal deaths because of AFE verified by medicolegal autopsy. In both the cases, several known risk factors associated with AFE, such as increased maternal age (41 and 35 years), diabetes, augmented labor, and cesarean delivery, were identified. Clinical features were typical, including sudden onset of cardiovascular and respiratory symptoms. In the patient who survived longer, both clinical and autopsy signs of disseminated intravascular coagulopathy were present, while they were absent in the case where death occurred rapidly. This paper describes briefly the particular features to look for at autopsy and stresses the importance of histology examination and staining techniques.     

Sickle cell lung disease and sudden death: a retrospective/prospective study of 21 autopsy cases and literature review

Sudden death in the setting of sickle cell lung disease (SCLD), is periodically seen in the practice of medical examiners. The goal of the present study was to identify the most common pathologic findings of SCLD associated with sudden or unexpected death. A retrospective/prospective review of 21 autopsy cases from sickle cell patients between 1990 and 2004 was performed. Review of medical records, autopsy reports, and H&E-stained slides of lung tissue was performed. Oil-Red-O and elastic staining of lung tissue were evaluated. All cases were screened for both acute and chronic forms of SCLD. Patients admitted for sickle cell pain crisis ranged in age from 8 months to 65 years. Fifteen out of 21 cases (71.4%) showed significant pulmonary pathology. The most frequent lung findings included pulmonary edema (47.6%), pulmonary thromboembolism (38.1%), fat emboli (33.3%), pulmonary hypertension, grades I-IV (33.3%), and microvascular occlusive thrombi (28.5%). Our study demonstrates higher-than-expected percentages of acute and chronic sickle cell-related lung injury such as fat embolism (33.3%) and pulmonary hypertension (33.3%), with right ventricular hypertrophy (33.3%). Therefore, we propose a simple and high-yield autopsy algorithm of ancillary procedures that should be applied on all known and suspected autopsy cases of sickle cell disease.     

Unexpected childhood death due to hemolytic uremic syndrome

Two cases of unexpected childhood death due to hemolytic uremic syndrome are reported. A 21-month-old girl who was discovered dead in bed following a short illness was found at autopsy to have overwhelming sepsis resulting from transmural colitis. Escherichia coli serotype 0157A was isolated from the intestine, and renal changes of hemolytic uremic syndrome were found. A 4-year-old girl died suddenly in hospital from intracranial hemorrhage while being treated for hemolytic uremic syndrome-related renal failure. Culture of urine and feces grew verocytotoxin producing E. coli. These cases demonstrate that hemolytic uremic syndrome may be a rare cause of unexpected childhood death and that the diagnosis may not be established prior to autopsy. Postmortem culture of tissues and fluids in cases of suspected sepsis in children may be essential in establishing this diagnosis, because histologic evaluation may be compromised by profound sepsis and tissue putrefaction. Accuracy in diagnosis may have significant public health and medicolegal consequences.     

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.     

过敏性休克死亡法医学诊断的研究现状

过敏性休克死亡在临床医学及法医学鉴定中较为常见,但过敏性猝死的死后诊断一直是法医病理学鉴定的一大难点。近年来,国内外学者研究了过敏性死亡血清IgE、组胺、肥大细胞类胰蛋白酶和P物质的含量以及其在肺脏和胃肠等组织中的免疫表达,试图为过敏性猝死的法医学鉴定提供客观、准确的形态学依据和诊断指标。本文就过敏性休克死亡法医学诊断的研究进展和存在的问题加以综述。     

Sudden death in a child due to an intrathoracic paraganglioma

A 12-year-old boy under treatment for asthma was found dead in his home. The autopsy revealed a large posterior mediastinal mass that completely compressed the upper lobe of the right lung and the associated airways. This mass extended from the right costovertebral sulcus into the thoracic spinal canal through the spinal foramen and compressed the spinal cord. It was located in the epidural space and was adherent to a nerve root. The histologic and immunocytochemical features were that of a paraganglioma. Although neurogenic tumors are the most common posterior mediastinal masses in the pediatric population, paragangliomas are rare, and spinal involvement has not been described in children. In addition, sudden death has not been reported in association with any of the 13 cases of posterior mediastinal paraganglioma described in the literature as involving the spine. This case illustrates an unusual cause of sudden death in a pediatric patient due to a benign neoplasm.     

Blood alcohol concentrations of sudden unexpected deaths and non natural deaths.

This study reports on the relationships between the blood alcohol concentration (BAC) and the cause of death - which is of course common knowledge for forensic scientists! Our special aim was to gain unselected data for generalizing conclusions. The blood of 2465 consecutive cases (86% of all 2852 sudden unexpected and unnatural fatalities investigated at the Institute for Legal Medicine in Hamburg during the year 1989) was analysed. The BAC was evaluated according to sex, age, cause of death and place of death. In natural causes of death there were 80% cases under 0.05% BAC. Endocrine and digestive system diseases in particular were associated with positive BAC values. In unnatural death cases the BAC under 0.05% was found in 64% of the suicides, 62% of the accidents, 54% of the homicides and 51% of the drug intoxications. The drowning cases showed higher BAC's than traffic accidents. In suicides middle aged males had high BAC's.     

Death caused by myocarditis in Wayne County, Michigan: a 9-year retrospective study

This paper reviews 72 cases of death caused by myocarditis between the years 1996 and 2004, autopsied at the Office of the Wayne County Medical Examiner in Michigan. Myocarditis as a cause of sudden and unexpected death represented 1.3% of all natural deaths in Wayne County during said period. The year 1999 contained the highest number of deaths of this cause (18), where the average number of myocarditis deaths was 8 per year for this 9-year span. In this study, each case was reviewed based on information gathered from investigative, autopsy, and toxicology reports. Significantly, 58% of these cases were male, and 63.4% were African American. Myocarditis caused death in every age group between 7 months and 67 years, but adults between the ages of 19 and 67 were most significantly affected (75%). Flu and/or cold were the most common symptoms experienced in the days directly proceeding death (28%), followed by shortness of breath (17%) and sudden collapse (15%). Sixty-nine percent of these 72 cases were pronounced dead after ACLS (advanced cardiac life support) protocol by emergency medical services or hospital attendants. Cardiomegaly was observed in 24 cases of adults aged 19 or older (54%), and flabby/soft myocardial tissue was observed grossly in 16% of all 72 cases.     

Uncertainty of determining cause of death in a medicolegal material without autopsy. An autopsy study

In constrast to other studies, this investigation was made on cases of medicolegal deaths that would not normally be autopsied. 223 females and 322 males, whose deaths were found to be natural before as well as after autopsy, were studied. The cause of death was estimated by external medicolegal examination, and after autopsy.In 79 females and 109 males, i.e. 35% and 34% respectively, estimated cause of death was found to be different after the autopsy. This was mostly because ischaemic heart disease as a cause of death was overestimated at the external medicolegal examination. No constant relationship between differing causes of death and age group could be demonstrated. Underdiagnoses and overdiagnoses tended to outweigh each other. Pneumonia, pulmonary embolism, cor pulmonale and aortic stenosis were clearly underestimated before autopsy. In addition, a variety of diseases that were not even mentioned at the medicolegal examination was found (subarachnoid haemorrhage, uraemia, perforated and bleeding gastric ulcers, tuberculosis).The same unreliability in the estimated cause of death therefore exists among cases not normally autopsied as found in retrospective studies of cases where autopsy is performed under all circumstances at the request of the police.False information will thus be given to the mortality statistics among the approximately 5000 cases of medicolegal deaths not autopsied in Denmark per year, most of these being natural deaths. Besides, contagious and inherited diseases could be overlooked, relatives given false information and the value of scientific studies in causes of death diminished.The conclusion is that autopsy is still essential to ensure continuous control and correction of causes of death.     

Sudden death of an elderly man with multiple malignant neoplasms.

With the lengthening of the human life span, cancer has become an increasingly important medical problem for the aged. It is not uncommon to find multiple primary neoplasms in elderly individuals. We recently investigated the death of an elderly man who had died suddenly and had three incidental malignant neoplasms, including a pleural mesothelioma, first diagnosed at autopsy. The importance of performing a complete medicolegal autopsy for epidemiological and statistical purposes is emphasized.