Sudden unexpected nocturnal death syndrome in the Mariana Islands

Sudden unexpected nocturnal death syndrome (SUNDS) is a distinct clinical entity in previously healthy, young, Southeast Asian males. It is well known in the Philippines and more recently recognized in the U.S. by nonspecific autopsy findings, with no evidence of underlying disease and absence of toxic drug or alcohol levels. In 1973-89, 14 cases of apparent SUNDS came to coroner's autopsy in the Commonwealth of the Northern Marianas (CNMI) and Guam. All 14 cases, with the exception of one Yapese, were previously healthy, male Filipinos, aged 23 to 55, who were either found dead in bed, or described by their colleagues as having nocturnal seizure activity consisting of gurgling, frothing, and tongue biting immediately prior to death. Autopsy findings showed no anatomic findings to account for death. Comprehensive serum and urine drug analyses were negative. All decedents showed absence of significant atherosclerosis or grossly detectable structural cardiac anomaly, while four showed cardiomegaly. Migrants from Southeast Asia carry with them a pre-disposition to this syndrome, which appears to decline with longer residence in the new country. The mechanism of death in SUNDS is believed to be ventricular fibrillation, possibly precipitated by sudden sympathetic discharge. Studies suggest at least some deaths may be associated with an abnormal cardiac conduction system. Acute pancreatitis has been a finding in some series, but not our cases. Why the condition is virtually limited to males and seemingly sleep-triggered, has not been adequately explained. Stress and depression are believed to be predisposing factors.     

Sudden unexpected death in a patient with splenic sequestration and sickle cell-beta+-thalassemia syndrome

Acute splenic sequestration crisis is a rare disorder that usually occurs in children, with sickle cell anemia, who are under the age of five years. A few cases have been described in adults with heterozygous sickle cell syndromes. Though this entity can be fatal there have been no reported cases associated with sudden death. We describe a case of sudden, unexpected death, associated with splenic sequestration, in a 29-year-old African-American man with undiagnosed sickle cell-beta-thalassemia syndrome.     

Sudden unexpected death in epilepsy: a retrospective study

Patients with epilepsy have a mortality rate higher than that of the general population; sudden unexpected death represents a significant category of mortality in these patients. The precise frequency of occurrence of sudden unexpected death in epilepsy (SUDEP) is not well defined, with a range of 1 in 370 to 1100 in the general epileptic population. A major difficulty with incidence studies is the continued reluctance in using the term SUDEP as a cause of death, making reliance solely on death certificates inconsistent and incomplete. Knowledge about SUDEP remains limited, as no single common risk factor has yet been identified, although predisposing conditions have been suggested. The purpose of this study is to review the association between several clinical variables and SUDEP to elucidate risk factors. The characteristics of the 67 cases in this series correlate with published findings in previous studies. Attributes that may be used to define an at-risk group of epileptics include age less than 40 years, male gender, long history of seizure disorder, undermedication or poorly controlled seizure activity, and mental or physical stress. Education of physicians as to the existence of SUDEP and risk factors is imperative in improving patient education and reduction in mortality.     

Sudden unexpected death due to a brainstem glioma in an adult

Sudden death due to undiagnosed central nervous system tumors is an uncommon, but well-described occurrence. Most of the tumors in these circumstances are supratentorial and occur in a wide spectrum of ages. Brainstem tumors are more rare and occur predominantly in the pediatric and adolescent populations. We present the case of a 48-year-old man who died suddenly and unexpectedly of a brainstem glioma. This case is unusual because of his age and the paucity of antecedent symptoms.     

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.     

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.     

Sudden death in an infant caused by rupture of a basilar artery aneurysm

Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. Seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.     

Sudden death from hyponatremia and hypokalemia in a woman with Gardner syndrome.

The authors present the case of a 39-year-old woman with Gardner syndrome who died from marked hyponatremia and hypokalemia. Gardner syndrome is a rare variant of the familial adenomatous polyposis syndrome in which the affected individual develops thousands of polyps within the gastrointestinal tract, with a 100% risk of eventual malignant change. Individuals with Gardner syndrome also develop a variety of extra gastrointestinal abnormalities. In the case presented, a woman with a clinical history of Gardner syndrome who had previously undergone a total colectomy with ileorectal anastomosis presented to the hospital with a recent history of sore throat, fever, diarrhea, and abdominal pain. The symptoms were considered clinically to be due to a viral gastroenteritis. She was admitted to the hospital, where she had episodes of collapse believed to be vasovagal in origin. She suffered a cardiorespiratory arrest and died 24 hours after admission. After her death, electrolyte estimation performed on blood taken shortly before death revealed severe hyponatremia and hypokalemia. Postmortem examination showed the gastric mucosa to be virtually covered by innumerable adenomatous and hyperplastic polyps. Fewer polyps were seen within the small bowel. There was no evidence of malignancy. The features were consistent with Gardner syndrome. Hyponatremia and hypokalemia have been described in patients with villous adenomas and in familial adenomatous polyposis syndromes associated with numerous colonic polyps. The cause of death in this case was considered to be hyponatremia and hypokalemia associated with florid gastric polyps in a woman with Gardner syndrome. Viral gastroenteritis contributed to the death by causing further electrolyte depletion. To the best of the authors' knowledge, death in Gardner syndrome has not been described as attributable to such metabolic disturbance, in particular in those who have only gastric, small bowel, and rectal polyps remaining after total colectomy.     

Sudden death in an infant caused by rupture of a basilar artery aneurysm.

Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. Seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.     

Sudden death in an 8-week-old infant with Beckwith-Wiedemann syndrome

The authors report a case of a 2-month-old girl diagnosed with Beckwith-Wiedemann syndrome (BWS) who was born prematurely and died suddenly in the hospital just before being discharged. BWS is a malformation syndrome associated with an increased risk of childhood tumors. The major features of BWS are macroglossia, abdominal wall defects, and visceromegaly, frequently leading to premature birth. Due to complex inheritance patterns, a predominance of nonfamilial cases, and the variability in expression of the features (termed incomplete penetrance), the risk of delayed diagnosis is evident. Secondary to hyperplastic pancreatic islands, hypoglycemia occurs frequently, and if not anticipated, adequate measures for prevention of hypoglycemic episodes may be delayed, resulting in possible intellectual deficits. The infant presented here died of natural causes: immaturity of the lungs resulting in marginal respiratory function and compounded by increased risk for asphyxia secondary to the enlarged tongue. The clinical history and findings in this infant are discussed in respect to the genetic syndrome with their relevance to medicolegal examination and the causes and manner of death.     

Sudden death caused by coronary artery aneurysms: a late complication of Kawasaki disease

The authors describe three cases (two blacks and one Latin American) of sudden death caused by late complications of Kawasaki disease (mucocutaneous lymph node syndrome). At autopsy each heart contained multiple coronary artery aneurysms with luminal stenosis caused by intimal hyperplasia and thrombi. Although virtually all fatal cases of Kawasaki disease occur within six months of the onset of symptoms, there have been other reported deaths up to 14 years after the acute illness. The coronary artery aneurysms of Kawasaki disease may persist and cause death years after the acute illness.     

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.     

Sudden death caused by myocardial tuberculosis: case report and review of the literature

A 25-year-old fit man died suddenly while playing social soccer. Autopsy revealed an infiltrative lesion involving the left ventricle with overlying pericarditis. No other significant pathologic changes were observed. Histologic examination showed necrotizing granulomatous inflammation. No acid-fast bacilli were demonstrated in the pericardial fluid or on histologic examination. The presence of Mycobacterium tuberculosis DNA complex was confirmed by use of the ligase chain reaction technique. The differential diagnosis of myocardial tuberculosis includes sarcoidosis, rheumatic fever, rheumatoid arthritis, giant-cell-containing tumors, idiopathic (giant-cell) myocarditis, and bacterial infections such as tularemia and brucellosis. This case illustrates the protean manifestations of tuberculosis and highlights the use of molecular biologic techniques in arriving at a definitive diagnosis in cases of suspected tuberculosis.     

Sudden, unexpected death in a 5-year-old boy with an unusual primary intracranial neoplasm. Ganglioglioma of the medulla

This report documents sudden, unexpected death in a 5-year-old boy from a ganglioglioma of the medulla. Gangliogliomas are rare, primary brain tumors that are encountered predominantly in childhood. We are unaware of any previous report of a similar case.     

Sudden death caused by tension pneumothorax after rupture of a thoracic aortic aneurysm. Case report

A rare case of fatal tension pneumothorax is reported. An aged Japanese man with marked subcutaneous emphysema of the neck was found collapsed in a betting office. He was ascertained to have left tension pneumothorax, based on radiographic examinations carried out before his death. At autopsy, severe pneumomediastinum was observed, and the descending thoracic aorta with a ruptured dissecting aneurysm was closely adhered to the left lung pleura. The hemorrhage spread into the pulmonary parenchyma and finally spouted out from the surface of the lung apex. Because the blood loss itself was not fatal in quantity, it is concluded that the patient died of tension pneumothorax caused by a lung penetration from the rupture of an aortic aneurysm.     

Sudden death in a patient with Von Recklinghausen's neurofibromatosis

Sudden death caused by tumors is uncommon. Death attributable to an unsuspected primary intracranial neoplasm is especially rare. An undiagnosed brain tumor is particularly unusual in a patient with Von Recklinghausen's neurofibromatosis (NF). A young man with known NF, with no manifest central nervous system involvement, died less than 24 hours after plastic surgery. A glioblastoma multiforme was discovered at autopsy.     

Sudden, unexpected death due to cardiac fibroma. Report of a case and review of the literature

A case of cardiac fibroma in a 58-year-old woman with a history of atrioventricular block is described. The patient died suddenly at home 6 days following the insertion of a pacemaker. Review of the pertinent literature is presented.     

Sudden death caused by parasites: postmortem cerebral malaria discoveries in the african endemic zone

This survey presents several cases of sudden deaths in Africa, specifically in Cote d'Ivoire, where the postmortem diagnosis of human cerebral malaria was determined after autopsy followed by pathologic examination of the brain. It is thought that cerebral malaria may be the primary cause of sudden death in nonimmunized persons during or after traveling in such endemic areas of Africa. The target population is composed of tourists, business travelers, and sailors. Because death caused by cerebral malaria occurs so suddenly, it can lead to forensic issues. Therefore, in any cases involving sudden death, it is very important for the forensic scientist to do a systematic evaluation, including pathologic examination of brain tissue, to rule in or exclude cerebral malaria. This practice will reinforce and aid research in progress directed at developing a vaccine and elucidating the role of tumor necrosis factor in this disease. Furthermore, this study will alert the physician to the importance of an effective and well-followed prophylaxis.