Lympho-monocytic enteroviral myocarditis: traditional, immunohistological and molecularpathological methods for diagnosis in a case of suspected sudden infant death syndrome (SIDS)

A 7-month-old boy was found lifeless in his bed by his grandfather. Near the mouth there were some blood and chyme on the bedlinen. His mother reported a chill during the last 8 days. Autopsy revealed multiple petechia under the capsule of the thymus, subepicardially and subpleurally, the cause of death was tentative supposed to be a sudden infant death. Histologically parts of the myocardium showed a lympho-monocytic myocarditis according to the DALLAS-criteria. Immunohistological investigations demonstrated an enhanced expression of MHC class II antigens, an increased number of leucocytes as well as T-lymphocytes. In situ hybridization and immunohistochemistry revealed myocardial enterovirus infection. Considering that there were regions in the myocardium without the traditional findings of myocardial necrosis and inflammation but with distinct expression of the above mentioned immunohistologic markers, immunohistological investigations turn out to be an appropriate tool to examine infant fatalities with uncertain cause of death for early stages of myocarditis.     

Marfan syndrome presenting as aortic rupture in a young athlete: sudden unexpected death?

A 20-year-old man had a spontaneous rupture of the ascending aorta while exercising. The diagnosis of Marfan syndrome, established by the gross and microscopic autopsy findings, was further supported by review of the autopsy report of the father, who had died at age 26 of a nearly identical aortic rupture. To avoid occurrences such as the above, the responsibility of the forensic pathologist should extend beyond the autopsy to appropriate counseling of families in instances where medically treatable hereditary conditions may be present in the survivors.     

Numerous cortical tubers and rhabdomyomas in a case of sudden unexpected infant death

Sudden infant death syndrome is the leading cause of death in infants between the ages of 1 month to 1 year. Sudden infant death syndrome, a diagnosis of exclusion, can only be made after other explanations for unexpected death have been ruled out. Tuberous sclerosis complex is occasionally the findings in these patients with unexpected infant death. Here, we present a case of an unexpected infant death during sleep with multiple factors that confound the cause of death. We discuss these factors and attempt to delineate their contributions to arrive at a cause and mechanism of death.     

Mechanical failure of a mitral valve prosthesis: an unusual case of sudden unexpected death

Sudden unexpected death caused by the failure of a cardiac valve prosthesis is a rare occurrence. A 45-year-old female suddenly died three years following mitral valve replacement for rheumatic mitral stenosis. At autopsy, death was determined to be due to a metallic fatigue of the mitral valve prosthesis.     

心肌炎猝死者心、脾中柯萨奇 B3病毒基因的检测

目的检测病毒性心肌炎（ viral myocarditis,VMC）猝死者心、脾组织柯萨奇 B3病毒 (CVB3)基因,探索 VMC的病原学诊断方法。方法运用原位逆转录 PCR技术检测实验组（ VMC猝死, 8例）及对照组（非心性死亡, 4例）心、脾组织中的 CVB3基因。结果实验组中, 3例（第 1, 4, 7例）心肌 CVB3基因阳性, 4例（第 2, 4 , 6, 7例）脾组织 CVB3基因阳性;对照组心、脾 CVB3基因均为阴性。结论心、脾组织中 CVB3基因联合阳性可能是 VMC的重要特征;联合检测心、脾中 CVB3基因可提高 VMC的病原学诊断率。     

Cytomegalovirus-induced pneumonia and myocarditis in three cases of suspected sudden infant death syndrome (SIDS): diagnosis by immunohistochemical techniques and molecularpathologic methods

Immunohistochemical and molecularpathologic techniques have improved the diagnosis of myocarditis as compared with conventional histologic staining methods done according to the Dallas criteria. Additionally, immunohistochemistry and in situ-hybridization are able to demonstrate viral infection, e.g. cytomegaloviruses in salivary glands and lungs, locations both known to be involved in cytomegalovirusinfection. However, in many cases of proved cytomegalovirusinfection the cause of death remains unclear. We report on three children younger than 1-year of age, who died suddenly without prodromal symptoms. Their deaths were attributed to SIDS (sudden infant death syndrome). In situ-hybridization, immunohistochemical (LCA, CD45R0, CD68, MHC-class-II-molecules, E-selectine) and molecularpathologic investigations (PCR), however, suggested that death was caused by a cytomegalovirus-induced pneumonia or myocarditis. In the future, these methods should be used for investigating cases with suspicion of SIDS.     

Arrhythmogenesis and diagnosis of cardiac sarcoidosis. An immunohistochemical study in a sudden cardiac death

We present an uncommon case of sudden cardiac death in a 34-year-old white woman. She was found lifeless at home by her parents. Three months before death she was recovered at the Emergency Room for chest pain, palpitation and loss of consciousness. Subsequent cardiological evaluation with ECG showed sinusal rhythm, QRS deviation to the left, QS aspect, asymmetric and rounded T waves and slight length of QT. During hospitalization she presented some episodes of supraventricular paroxysmal tachycardia and non-sustained ventricular tachycardia. No echocardiography alterations were found. An anti-arrhythmic treatment was prescribed. Autopsy revealed some fibrotic scarring in the myocardium of left ventricle. The histological examination of the heart revealed diffuse and extensive fibrosis with non-caseating sarcoid granulomas. The lungs, kidneys and lymph node also showed the same non-caseating granulomas. The diagnosis of sarcoidosis with massive and extensive cardiac involvement was established as cause of death.     

Trophoblastic microemboli as a marker for preeclampsia-eclampsia in sudden unexpected maternal death: a case report and review of the literature

The authors report the case of a 25-year-old white woman at 7 months' gestation who died suddenly and unexpectedly at home. Anatomic findings at autopsy included a tongue contusion, glomerulonephritis, changes indicative of systemic hypertension, and trophoblastic microemboli in the lungs. Review of the prenatal care record disclosed 3+ proteinuria 2 days before death. The features of the postmortem examination were consistent with clinically undiagnosed preeclampsia-eclampsia and glomerulonephritis. The authors discuss the rarity of fatal preeclampsia-eclampsia, the contribution of concomitant glomerulonephritis, and the significance of trophoblastic microemboli in the lungs.     

Asplenia as a cause of sudden unexpected death in childhood

Sudden unexpected death in childhood is rare. The commonest causes of such deaths are a result of fulminating infections of the respiratory or nervous systems. Other causes include unsuspected congenital abnormalities of the heart, acute metabolic disorders, and rarities such as internal hemorrhages and pulmonary thrombosis. Recognition of children with congenital asplenia who are otherwise normal but have an increased susceptibility to overwhelming sepsis is extremely difficult. We reviewed 1763 autopsy files from our institution over 5 years (1990-1995), of which 293 were classified as pediatric cases. The vast majority of the cases were stillbirths and deaths within the first year of life as a result of complex congenital anomalies. Four cases of asplenia were identified in our entire series, 3 of which were of the congenital syndromal variety and 1 of which was a case of isolated sporadic congenital asplenia. All 4 cases of asplenia were analyzed in detail with respect to autopsy findings and cause of death. Severe complex cardiac malformations were present in the congenital syndromal asplenia patients; these other malformations contributed significantly to their death. In this report, we discuss in detail the autopsy findings in a previously healthy 4-year-old girl who presented with a brief 8-hour history of being unwell and died within 4 hours of admission into the hospital. She had sporadic, isolated congenital asplenia complicated by high-grade type 6B pneumococcemia and acute bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). Previously healthy children who clinically deteriorate very rapidly should have a blood smear done as part of their clinical workup. The detection of Howell-Jolly bodies on a peripheral blood smear can be an indicator of asplenia, and this diagnosis can be confirmed by medical imaging of the abdomen. Such steps may aid in the aggressive management of isolated congenital asplenia and thereby avert untimely death.     

An unusual case of sudden unexpected death: postmortem investigation and biomechanical analysis of the cervical spine

A naked man died under peculiar circumstances and the postmortem examination revealed unexpected lesions in the cervical spine. Investigations of the cervical spine (computed tomography, magnetic resonance imaging, and histological examination) showed that a piece of bone was torn of the anterior part of vertebra C6 and that there was fresh bleeding in the surrounding tissue. The cause of death remained unclear but was most likely cardiac arrhythmia initiated by beta-2 agonist inhalation due to an acute asthmatic attack. Data from biomechanical investigation using finite element analysis supported the conclusion that the cervical spine injury was secondary to impact during falling as a consequence of the cardiac arrhythmia.     

Intracranial arteriovenous malformations presenting as sudden unexpected death: a report of 3 cases and review of the literature

Intracranial arteriovenous malformations (AVMs) are cerebrovascular abnormalities due to maldevelopment of the regional capillary bed, producing lesions with arteriovenous shunting. AVMs may account for as many as 1% of all sudden deaths; however, case reports in the literature are exceedingly rare. We present 3 cases of sudden death due to hemorrhage of AVM and discuss the literature and the differential diagnosis in cases of sudden death due to intracranial hemorrhage.     

Mechanisms of unexpected and/or sudden death in Lafora disease

A 23-year-old male was found dead wedged between two chairs at his home address. His past history included a diagnosis of Lafora disease (a type of heritable progressive myoclonic epilepsy) at the age of 16 years. This had been characterised by the development of epilepsy and progressive motor impairment and mental deterioration. Diagnosis had been confirmed by demonstration of mutation in the EPM2A gene on chromosome 6q24. At autopsy, petechial haemorrhages were noted of the face and conjunctivae bilaterally. There were no other significant findings apart from gastric contents within the airways. Death was attributed to positional asphyxia complicated by aspiration of gastric contents. Although death in Lafora disease is usually predictable and often protracted, sudden and/or unexpected death may occur and involve status epilepticus, sudden unexpected epileptic death, choking, aspiration of gastric contents, and cardiac arrhythmias. In addition, the possibility exists of unnatural causes of death, such as accidents, provoked by epilepsy or physical inability of the victims to extricate themselves from dangerous situations, or homicides, provoked by difficulties in caring for individuals with significant and progressive disabilities.     

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.     

Pathologico-anatomic findings in sudden, unexpected death in children and adults with influenza A infection

Thé following observations resulted from studies on forensic autopsy cases: In 76% of the adults and 55% of the infants the cases of unexpected sudden death without morphologically verifiable causes of death showed virologic evidence of recent influenza-A (H3N2)-infection. The pathologic findings corresponded with the findings in lethal infections with influenza-A viruses. Investigation of cases of sudden and unexpected death should always include virologic serum tests. The demonstration of IgM antibodies against influenza-A virus confirms that there was a recent infection. Death from influenza-A infections occurs also in the interepidemic periods.     

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.     

Isolated myocarditis as a cause of sudden death in the first year of life

A series of three cases of isolated myocarditis, presenting as sudden death in infancy, occurred over a period of 3 months. This prompted a review of the autopsy records of the Children's Hospital of Winnipeg. Over a period of 40 years, 24 cases of isolated myocarditis were traced from 3196 autopsies. Most (21 of 24) cases of isolated myocarditis occurred in infants less than 12 months of age. In 16 of the infants there were either no antecedent clinical signs (sudden deaths), or a short clinical history of less than 24 h duration. Heart weights, however, were greater than the 99th percentile of published normals in three infants and above the 95th percentile in a further 16 infants. Areas of hypertrophied fibres were seen even in infants with a short history. These latter findings suggest that a latent phase of myocarditis may exist. The responsible pathogens were identified very rarely, due to a lack of suspicion of the existence of myocarditis, and it is suggested that samples of myocardium should be submitted for virologic examination in all cases of sudden death in the first year of life.     

Cosleeping and sudden unexpected infant deaths in Kentucky: a 10-year retrospective case review

This retrospective case review investigates modifiable risk factors in sudden unexpected infant deaths, including those attributed to sudden infant death syndrome, and examines the impact of cosleeping with adults or siblings. The study examines sudden unexpected infant deaths from 1991 to 2000 in the state of Kentucky, excluding homicides and deaths from identifiable natural causes. Meta-analysis provides a cosleeping prevalence control in normal infants. Based on the findings described herein, we conclude that cosleeping may represent a risk factor in sudden unexpected infant deaths and that a full scene investigation, including whether the infant was cosleeping, should be sought in all cases of sudden infant death.