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.

IgA plasma cell infiltration of proximal respiratory tract, pancreas, kidney, and coronary artery in acute Kawasaki disease.

The etiology and pathogenesis of Kawasaki disease (KD) remain unknown. As previously reported, in US patients with acute KD, IgA plasma cells (PCs) infiltrate the vascular wall. To determine whether IgA PCs are increased at mucosal sites in KD and to determine whether other nonvascular KD tissues are infiltrated by IgA PCs, the cells were immunolocalized and quantitated in tissue sections taken from 18 US and Japanese patients who died of acute KD and from 10 age-matched controls. IgA PCs were significantly increased in the trachea of patients who died of acute KD, compared with controls (P<.01), a finding that was similar to findings in children with fatal respiratory viral infection. IgA PCs also infiltrated coronary artery, pancreas, and kidney in all KD patients. These findings strongly support entry of the KD etiologic agent through the upper respiratory tract, resulting in an IgA immune response, with systemic spread to vascular tissue, pancreas, and kidney.

Homicidal commotio cordis in two children.

This paper's objective is to describe two cases of fatal commotio cordis resulting from the deliberate striking of children's chests by adults with their fists. These deaths involve two male children, ages 3 years and 14 months. The clinical histories, events in the households prior to the deaths, behaviors of the children, autopsy findings, and investigation results are all similar. In both cases, fatal blows were delivered to the anterior chest with a closed fist. Both children collapsed immediately, unable to be resuscitated. Confessions were obtained in both cases by investigators soon after the children's deaths. Autopsies showed chest contusions in only one child, presumably due to knuckle impact. The cardiac rhythms noted by paramedics were ventricular fibrillation and asystole. Due to the lack of physical findings, an immediate and thorough investigation is critical. An accurate history of events preceding death must be obtained.

Unexpected death due to gestational choriocarcinoma: a report of two cases.

We report two cases of unexpected death due to metastatic gestational choriocarcinoma and provide a review of the literature regarding these neoplasms. Our cases illustrate the extreme importance of medical follow-up of patients after an ectopic pregnancy or therapeutic abortion.

A prospective study on sudden unexpected death in epilepsy.

Sudden unexpected death accounts for a substantial portion of deaths among epileptics. The incidence of this phenomenon is probably 1 in 370 to 1 in 1,110 in the general epileptic population but may be even higher in the 20- to 40-year age group, and still higher if epileptics with symptomatic epilepsy are selected. Sudden unexpected death in epileptics has been observed at least once weekly by the Office of the Medical Examiner of Cook County (Chicago), Illinois, for many years. A year-long prospective study revealed that victims of this complication of epilepsy are most commonly black males averaging 35 years of age who have infrequent generalized seizures and usually have some structural lesion in the brain responsible for their seizures. They tend to abuse alcohol and have poor compliance with anticonvulsant medication. The electroencephalograms display considerable variability from record to record. At autopsy the heart, lung, and liver weights were heavier and the brain weights were lighter than expected. The mechanisms involved in sudden unexpected death in epileptics may include autonomically mediated cardiac arrhythmia alone or in combination with sudden "neurogenic" pulmonary edema and "backward" cardiac failure.

Sudden epilepsy deaths and the forensic pathologist.

Sudden unexpected deaths in epileptic persons are not rare events, most commonly encountered by the forensic pathologist rather than the clinician. Such deaths may represent 1-1.5% of all "natural" deaths certified by the medical examiner or coroner. The typical victim is a black male about 30 years of age who tends to abuse alcohol, with a history of generalized epilepsy for more than 1 year and likely for more than 10 years. There are a lack of obvious anatomic causes for the death at autopsy, but 60-70% of cases will have a lesion in the brain (most commonly old trauma) to explain the epilepsy. Most victims have no blood levels of anticonvulsant medications at the time of death. We have evolved a form for use by medical examiner/coroner's investigators at the scene to collect relevant information which will be of assistance to the pathologist in interpreting the case. Estimated prevalence of sudden epilepsy death, mechanisms, and other features of such cases are reviewed briefly.

Atypical gunshot wounds of entrance: an empirical study.

Atypical gunshot wounds of entrance occur when bullets deviate from their stable nose-on trajectory before entering the body. When this occurs, the resulting wound may have an atypical D-shaped appearance. Ray-like abrasions or bruises may radiate from the corners of the wound. Unstable nonaxial flight may be caused by intermediate targets, ricochets , inappropriate weapon/ ammunition combinations, poor weapon construction, or use of misaligned silencers . If a bullet is deformed before entrance the configuration of the resulting wound may be bizarre, and the wound configuration may closely resemble the configuration of the striking bullet. Nine cases are presented showing the effects of various intermediate targets. A number of methods for the investigation of atypical wounds are discussed.

Sudden unexpected death associated with seizures: analysis of 66 cases.

We have analyzed 66 cases of sudden unexpected death (SUD) in persons with seizure disorders, which were examined by the Office of the Medical Examiner, Cook County (Chicago), Illinois. The individuals ranged in age from 10 months to 60 years (mean age, 28 years). Autopsy findings were insufficient to explain death, and there was no evidence of major systemic pathology. Approximately 40% of victims were found dead in bed, and the remainder in some other room at home, apparently having been engaged in normal activity. Several died in an emergency room following a seizure at home. Cardiopulmonary resuscitation was attempted but was ineffective. Neuropathological examination revealed brain lesions, which probably caused the seizures, in 60% of the cases. In 68% the anticonvulsant blood level was subtherapeutic or below detectable levels. The prevalence of seizure-associated SUD may be between 1:525 and 1:2,100 among epileptics. The mechanism of death in these cases probably involves cardiac arrhythmias mediated by sympathetic autonomic events occurring during the seizure.

Ponto-medullary avulsion associated with cervical hyperextension.

19 cases of partial or complete ponto-medullary avulsion are reported. This type of damage seems to be produced by severe hyperextension of the head on the neck with or without an additional rotational component.