成年人癫痫猝死(附两例病例报告)

目的 探讨成年人癫痫猝死（sudden unexpected death in epilepsy,SUDEP）的临床表现及可能发生的机制。方法回顾性分析2例成年人SUDEP的临床资料并复习有关文献。结果2例成年男性患者均在数分钟内发生突然死亡,均为特发性全面性强直-阵挛发作类型;均为长期服用抗痫药物且癫痫控制不良。1例在睡眠中死亡,另1例在午饭后死亡。结论成年人SUDEP的发生机制可能为：心律失常,神经源性肺水肿和中枢性呼吸抑制。强直-阵挛性发作、联合用药及癫痫控制不良是SUDEP的危险因素。较好地控制强直-阵挛发作是减少SUDEP的有效措施。     

Sudden unexpected death in a toddler with Williams syndrome

We report a toddler with Williams syndrome (WS) complicated by congenital heart disease and a seizure-like episode who died suddenly several hours after uneventful cardiac catheterization. The cardiovascular anomalies and their association with sudden death as well as the clinical neurological and neuropathologic abnormalities identified in WS are delineated and discussed.     

Sudden unexpected death due to undiagnosed glioblastoma

Although glioblastomas are among the most common primary cerebral neoplasms, sudden death due to these tumors is an uncommon event. Due to the usual rapid increase in intracranial pressure, patients develop symptoms rather early, leading to medical attention in time. A search for cases of sudden unexpected death due to undiagnosed glioblastoma from a total of 14,482 cases from the archives of the Institute of Legal Medicine in Hamburg in the period of 1991–2003 revealed only one such case. Out of a total of 5,432 cases from the Institute of Neuropathology, Hamburg, during the same period, two further cases were found. A comprehensive literature review on cases of sudden death due to primary cerebral neoplasms published so far revealed a total of 83 cases with only ten cases of glioblastoma (12%), whereas 55 of these cases were due to histological benign tumors (66%).     

Sudden unexpected death in childhood due to eosinophilic myocarditis

A 12-year-old boy with no previous serious medical history experienced abdominal discomfort and chest pains for 5 days and suddenly died. The autopsy revealed diffuse and extensive infiltration of eosinophils into the myocardium, with poorly formed granulomas and few fibrotic changes. The necrotic change was so extensive that Charcot-Leyden crystals formed. The other visceral organs had no specific pathologic changes except for mild lymphocytic infiltration with an increase in goblet cells in the bronchial areas and eosinocytosis in the blood vessels. An initial viral infection. seemed to have caused subsequent eosinophil activation due to an allergic condition. Eosinophilic myocarditis is a rare cause of sudden death in apparently healthy children. Cardiac toxicity of eosinophils is, however, well established and dominates the ultimate prognosis of patients with complicated eosinophilia.     

Sudden unexpected child death associated with ingestion of fluid dish detergent

Summary A case of sudden unexpected death of a 13-month-old child is reported. The immediate cause of death was aspiration of stomach contents. The autopsy and microscopical examinations revealed cerebral oedema, organ congestion and an increased number of neutrophils in germinal centres of mesenterial lymph nodes and spleen. The finding of ethanol and methanol (5.2 and 0.1) and of significant amounts of a surface-active agent (linear alkyl benzene sulphonate) in the stomach contents indicated consumption of a fluid dish detergent prior to death. The blood ethanol concentration was below 0.1 and urine ethanol and methanol concentrations were 0.28 and 0.04. The possibility that ethanol-containing detergents may trigger hypoglycemic attacks is discussed.     

A forensic autopsy case: Sudden unexpected death due to cardiac inflammatory myofibroblastic tumor

We report an autopsy case of a 25-year-old man with no medical history who died suddenly in an Internet cafe. He was found in cardiorespiratory arrest and did not respond to cardiopulmonary resuscitation. Traumatic lesions were not observed on his body. An autopsy was performed to investigate the cause of death. Upon examination, we discovered a heart tumor that infiltrated from the outside wall to the outflow tract of the left ventricle. Left ventricular outflow tract obstruction due to a cardiac tumor was considered the mechanism of death. Histological examination identified an inflammatory myofibroblastic tumor (IMT). The final diagnosis was death secondary to circulatory failure due to a cardiac IMT. Additionally, a cardiac tumor was diagnosed using post-mortem computed tomography. Only few cases of sudden unexpected death due to cardiac IMT have been reported; we report this case along with a review of the literature.     

Sudden unexpected death with dysplastic change in the atrioventricular node artery

We report an autopsy case of a 19-year-old male who died suddenly. Death happened while he was sleeping after drinking alcoholic beverages. His heart revealed concentric hypertrophy of the left ventricle without asymmetric septal hypertrophy or mitral regurgitation. Upon microscopic examination, the epicardial atrioventricular (AV) node artery revealed stenosis with intimal thickening before it entered the ventricular septum and acute ischemic change was observed beneath the conduction system in the upper ventricular septum. This finding suggests that death occurred after some preceding localized ischemic event within the ventricular septum. We therefore consider that the cause of death was fatal arrhythmia due to dysplastic change to the AV node artery.     

Sudden neonatal death from congenital cystic adenomatoid malformation

We report a case of congenital cystic adenomatoid malformation (CCAM) of the lungs resulting in sudden death immediately after birth. The case is extremely unusual because of the diffuse bilateral involvement. The extensive involvement of both lungs could explain the abrupt onset of the symptoms and the ineffectiveness of resuscitation attempts. The presence of cartilage as a part of the malformation adds interest to the case, since it is seldom found in this malformation and to the best of ¶our knowledge has been reported only exceptionally in a ¶type II CCAM.     

Sudden and unexpected deaths due to non-traumatic abdominal disorders: A forensic perspective

Sudden and unexpected deaths due to non-traumatic abdominal disorders are a disregarded topic in the forensic field. These conditions are very heterogenous, accounting for 1–5% of sudden deaths. Specifically, acute gastrointestinal hemorrhages, bowel obstructions, liver cirrhosis, and necrotizing pancreatitis are frequently reported, whereas there are also unusual conditions which deserve careful analysis. Furthermore, the postmortem examinations of such disorders are not straightforward in the forensic setting since advanced putrefaction of the body, alleged medical malpractice, or the absence of any medical records could complicate autopsy procedures. Thus, the use of forensic histopathology, toxicology, microbiology, and postmortem radiology may help the pathologists to identify the abdominal cause of death. In this paper, the authors aim to discuss most of congenital and acquired abdominal conditions that have resulted in sudden and unexpected deaths. Hence, the discussion of the pathological features of each abdominal organ or system is prefaced with practical examples from caseworks of the Milan Institute of Legal Medicine. However, this paper also shows an extensive literature review with selected medicolegal articles to detailly describe the lethal mechanisms related to each condition, along with any associated risk factors or other diseases. Finally, each section shows a focus on either novel forensic approach (when available) or medicolegal implications, such as the use of postmortem radiology, peculiar histopathologic findings, or allegations in medical malpractices.     

Sudden unexpected death due to Fournier’s gangrene

A 43-year-old Japanese male vagrant collapsed suddenly in a police station. The man was resuscitated in hospital but died about 13 h later. An autopsy revealed necrotizing fasciitis in the genitalia and lower abdominal wall. The man was considered to have died from endotoxic shock following on Fournier’s gangrene. Received: 16 July 1996 / Received in revised form: 23 December 1996     

Sudden,unexpected infant death due to pulmonary arterial hypertension

A 3-year-old girl with no particular medical history complained of a stomachache and died on the way to the hospital. The autopsy revealed marked right ventricular hypertrophy and dilation with no other cardiac abnormalities. Microscopically, the pulmonary small arteries showed marked medial hypertrophy and varying degrees of intimal and adventitial thickening. We supposed that the cause of death was attributable to pulmonary arterial hypertension (PAH). PAH is a rare disease that can cause sudden, unexpected death at any age. Forensic pathologists should consider PAH in the differential diagnosis of sudden death.     

Sudden cardiac death from parvovirus B19 myocarditis in a young man with Brugada syndrome

Cardiovascular diseases are the leading cause of sudden death all over the world. The aetiology of sudden cardiac death among young adults includes Brugada syndrome and myocarditis.Brugada syndrome is a genetic abnormality of sodium channels in the myocardium with a characteristic electrocardiographic pattern.Myocarditis has several aetiologies including infections. One of the most common cardiotropic viruses is parvovirus B19. This infection presents as a febrile illness in childhood and may result in fatal outcome, more frequently in adults. In this report we present a case of a young man who suffered from a mild upper respiratory tract infection. After recovery he had an episode of syncope and was diagnosed with Brugada syndrome. Some weeks later he died suddenly at home while sleeping. The detailed forensic pathological, histological and microbiological investigation revealed a parvovirus B19-associated myocarditis. Synergic effect of structural and functional abnormalities of the myocardium may lead to death. The cause and potential complications (eg. myocarditis) of even mild infections should be monitored carefully.     

Sudden death in case of syringomyelia

Summary Report of a case of sudden death in a 28 year old man, with known disease of the spinal cord. Autopsy showed hydromyelia, syringomyelia and a moderate intoxication by ethyl alcohol, which in combination are considered to have caused the death.

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Zusammenfassung Bericht über den plötzlichen Tod eines 28jährigen Mannes mit einer klinisch bekannten Erkrankung des Rückenmarks. Bei der Autopsie wurden eine Hydromyelie und Syringomyelie festgestellt. Es bestand eine mittelgradige Alkoholisierung. Die Kombination dieser Gegebenheiten wird als Todesursache angesehen.

     

Association between sudden unexpected deaths in bathtubs and ambient temperature among elderly Japanese adults: A time-series regression study

BackgroundSudden unexpected deaths in bathtubs among elderly Japanese adults occur predominantly during the cold season. This study investigated the relationship between these deaths and bathing day temperature among elderly adults in Tokyo.MethodsData for 1408 cases of bath-related deaths from January 1 to December 31, 2015 were obtained from the Tokyo Medical Examiner’s Office. We excluded 409 cases for the following reasons: criminal death, injury-related death, suicide, intoxication, non-sudden death, not bathtub-related death, out-of-bathroom death, subject aged under 65 years, undetermined bathing date, institutional housing, and bathing not at subject’s home. Ultimately, 999 cases were analyzed. Daily mean temperature data were collected. A time-series regression study was performed to estimate the influence of sex, age, and bathing day temperature. Monthly changes in the population bathing in a bathtub were considered in the model.ResultsThe relative risk (RR) of sudden unexpected death in a bathtub was 1.381 for males (95% confidence interval [CI]: 1.218–1.564) compared to females. The RRs were 4.182 (95% CI: 3.523–4.986) and 9.382 (95% CI: 7.836–11.273) among those aged 75–84 years and ≥85 years, respectively, compared to among those aged 65–74 years. The RR increased to 1.092 (95% CI: 1.082–1.102) as the daily mean temperature decreased by 1 °C.ConclusionSudden unexpected death in a bathtub correlated with bathing day temperature among elderly Japanese adults, and extremely low temperature, male sex, and older age increased the risk of such death. Our findings provide insight into preventing sudden unexpected deaths in bathtubs.     

A case of sudden unexpected infant death involving a homozygotic twin with the thermolabile CPT2 variant,accompanied by rotavirus infection and treatment with an antibiotic containing pivalic acid

We investigated a case of sudden unexpected death involving a 22-month-old male homozygotic twin infant. After both of the twins had suffered from gastroenteritis, one was found dead in his bed, but his brother survived and has since been healthy. Notably, only the deceased had been treated with an antibiotic containing pivalic acid, which may sometimes cause hypocarnitinemia. Postmortem computed tomography and medicolegal autopsy demonstrated severe liver steatosis, and subsequent genetic analysis revealed that the twin had the thermolabile variant of carnitine palmitoyl transferase 2 (CPT2). On the basis of these facts, we concluded that the cause of death had been fatty acid oxidation deficiency accelerated by an antibiotic containing pivalic acid and virus infection in this infant harboring the thermolabile genetic variant of CPT2. Although each factor alone was not fatal, their combination appeared to have resulted in sudden unexpected infant death.     

Sudden death associated with myxomatous transformation of the mitral valve in an 8-year-old boy

An 8-year-old boy suffered a cardiac arrest while playing soccer. In contrast to a similar event at the age of 5 years, resuscitation was not successful. At autopsy, the cardiac findings were of a myxomatous transformation of the mitral valve with lacerations of the posterior cusp and the left vestibular endocardium and left ventricular hypertrophy. Sudden death due to arrhythmias in association with a myxomatous mitral valve is a rare event with only about 100 cases published world-wide. To our knowledge, the present case probably reports the youngest affected individual who died of this pathological condition. Received: 5 November 1997 / Received in revised form: 5 January 1998     

Sudden adult death: An autopsy series of 534 cases with gender and control comparison

The aim of our study was to highlight the epidemiological difference in adult sudden death between males and females. The type of pathologies found in adult victims of sudden death was compared to control cases in order to determine the most significant pathologies involved in sudden death.Among all autopsies performed between 1995 and 2009, 534 adult cases of sudden death and 154 cases who violently died were respectively selected. For each case, a complete autopsy was carried out, including systematic histological examination of all major organs.The sudden death population was composed of 369 males and 165 females. There was no statistical difference regarding age between males and female. Sudden death took place more often at home in women than in men (p < 0.0001). A stressful event was more frequently found in men than in women (p = 0.03). Deaths caused by cardiovascular diseases were more frequent in males than in females, especially Coronary Artery Disease (CAD) (p < 0.0001). Cardiomyopathy was more often the cause of death in women, particularly Arrythmogenic Right Ventricular Cardiomyopathy (ARVC). Cardiac pathologies were found in 45% of the control cases. CAD and ARVC were statistically more frequent in the sudden death group than in the control group.According to our study, profile of sudden death is different between males and females. Those data seem to be important for clinicians involved in prevention programs of sudden death, as they can adapt their screening according to the gender.     

Sudden death in a child caused by a giant cavernous hemangioma of the anterior mediastinum

A 4-year-old girl who had been treated for asthma since the age of 2 years had a severe coughing fit and died suddenly. The patient had a history of occasional severe coughing fits, and these fits had been worsening in severity during the week before her death. Prior to death, she was taken to a clinician, and thymic hypertrophy was suspected based on chest X-ray findings. The clinician recommended that she visit a general hospital at a later date; however, she died that night. Postmortem radiology and autopsy revealed a large mass in the anterior mediastinum compressing the heart and airway, and no other findings attributable to sudden death were observed. Therefore, we concluded that the patient's death was attributable to acute respiratory and cardiac circulatory failure secondary to the pressure induced by the mass. Microscopically, the mass showed a cavernous structure composed of cystically dilated, thin-walled large vessels filled with blood. The final diagnosis was a cavernous hemangioma. Hemangiomas are the most common benign vascular anomalies seen in young children; however, mediastinal hemangiomas are rare and can cause life-threatening complications because of their size and location. Therefore, forensic pathologists should include hemangioma as a differential diagnosis in children with anterior mediastinal masses.     

Sudden death following AV node ablation in a man with Fabry disease mimicking hypertrophic cardiomyopathy

We present a case of Fabry disease with an uncommon pattern of asymmetrical hypertrophy with septal prominence resulting in an erroneous diagnosis of hypertrophic cardilmyopathy clinically. The deceased presented for a medicolegal autopsy following his sudden death after an AV node ablation. Fabry disease continues to be an important misdiagnosis of hypertrophic cardiomyopathy in a clinical setting. Early diagnosis of Fabry disease is essential so that early treatment can be instituted.     

Cardiac sudden death in a young cannabis user

We report a case of sudden death due to acute coronary syndrome (ACS) in a young cannabis user. A man in his late thirties died at home, and marijuana was found. The autopsy revealed severe occlusion by an atherosclerotic plaque in the left anterior descending artery. The histopathological examination revealed ischemic changes, likely caused by cannabis-induced sympathetic β-adrenergic stimulation. Both cannabinoid receptors (CB1 and CB2) were expressed in the atherosclerotic lesions. The CB2 expression was higher than CB1 expression in the atherosclerotic plaque, corresponding to macrophage infiltration. Since cannabis is regarded as a casual drug due to its lower levels of dependency, some individuals have supported legalized marijuana use. However, this case report will provide cautions on the casual use of cannabis.