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.     

Representation of cerebral bridging veins in infants by postmortem computed tomography

The postmortem diagnosis of shaken baby syndrome, a severe form of child abuse, may be difficult, especially when no other visible signs of significant trauma are obvious. An important finding in shaken baby syndrome is subdural haemorrhage, typically originating from ruptured cerebral bridging veins. Since these are difficult to detect at autopsy, we have developed a special postmortem computed tomographic (PMCT) method to demonstrate the intracranial vein system in infants. This method is minimally invasive and can be carried out conveniently and quickly on clinical computed tomography (CT) systems. Firstly, a precontrast CT is made of the infant's head, to document the original state. Secondly, contrast fluid is injected manually via fontanel puncture into the superior sagittal sinus, followed by a repeat CT scan. This allows the depiction of even very small vessels of the deep and superficial cerebral veins, especially the bridging veins, without damaging them. Ruptures appear as extravasation of contrast medium, which helps to locate them at autopsy and examine them histologically, whenever necessary.     

Virological analysis in the diagnosis of sudden children death: a medico-legal approach

Infections are considered to be an important cause of unexpected death in children. It has also been assumed that respiratory viruses are involved in the genesis of sudden infant death syndrome (SIDS). The Spanish National Institute of Toxicology and Forensic Sciences act as the forensic reference centre for Spain. We analyse the experience of this centre in the virological study of 64 cases of sudden children death where viral serology, virological cultures, herpesviruses polymerase chain reaction (PCR) and electron microscopy were performed. According to pathological findings, death could only be attributed to an adenovirus infection in one amygdalitis with upper airways stenosis and asphyxia. Human herpes virus 6 (HHV-6) was detected by PCR in one case with pathological findings characteristic of SIDS. Recent infection by respiratory syncytial virus (RSV), Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were also detected. Meanwhile, 85.9% of the cases yielded negative viral results. Twenty-eight infants were finally categorised as SIDS. Pathological findings of infection were detected in 12 patients despite the negativity of viral analyses. Although viral infection is an uncommon cause of sudden children death, a complete microbiological investigation will help to solve the puzzle of SIDS. Definitive guidelines for microbiological analyses need to be updated whilst new pathogens are discovered or new techniques are implemented in order to clarify unsolved cases.     

Quality assurance review of death certificates: a pilot study

BACKGROUND: Although quality assurance programs for medical examiners are required by the National Association of Medical Examiners' Inspection and Accreditation Checklist, quality assurance programs specifically targeting death certificate completion have not been addressed. The Fulton County Medical Examiner, Atlanta, GA, has implemented a pilot quality assurance program for death certificate information, and this report contains information about 1 year's experience with the program. METHODS: All death certificates are reviewed by the case medical examiner(s) and chief medical examiner prior to their release to funeral homes. Death certificates with errors are retained for quality assurance and review purposes, and needed corrections are made before death certificates are released. During a 1-year period, death certificates with errors were collected and then reviewed and tabulated by type of error. RESULTS: Between May 26, 2003, and May 25, 2004, the Fulton County Medical Examiner certified 1267 deaths. Of these, 47 (4%) were found to contain errors that were corrected and an additional 52 (4%) had been amended for various reasons. The most common errors were misspellings in causes of death or poor or incomplete wording in injury-related information. Forty-seven percent of errors involved omitted, incomplete, or incorrect information that was potentially significant. The most common reason for amended certificates was unexpected detection of acute intoxications among people with significant cardiovascular disease. CONCLUSIONS: Quality assurance review of death certificates can assist in preventing the release of death certificates with incomplete, erroneous, or omitted information and may also be useful as an educational forum regarding completion of the death certificate.     

Lightning fatalities on the South African Highveld: a retrospective descriptive study for the period 1997 to 2000

A review of the Southern Africa medical literature shows a paucity of published data regarding lightning fatalities. The South African Highveld has a lightning ground flash density of 6 to 9 flashes/km/year, with a high incidence of thunderstorm days per year (some 40-70). The Highveld has a largely urban population, many of whom have low socioeconomic status and poor education, housing, and other infrastructures and hence (possibly) are at greater exposure risk. Thirty-eight victims of lightning-related death were identified from the records of the 6 large medicolegal mortuaries on the South African Highveld, serving a population of approximately 7 million, for the period 1997 to 2000. Analysis of the records revealed that 95% of all victims were black, 79% were male, and the average age was 36 years. Lightning strikes occurred from September through to April (normal summer rainfall period), and the most strikes took place in the late afternoon (3:00 pm to 6:00 pm). All except 1 case occurred outdoors. In the autopsy reports, mention was made of singeing of hair in 68% of cases, and mention of damage to clothing was made in 26% of cases. Cutaneous thermal injuries were noted in 34 of the 38 cases, with apparent electrothermal injuries of the feet noted in 4 cases. Fifty-two percent of victims sustained some form of associated blunt-force injury (including abrasions, contusions, etc). Specific keraunopathologic injuries were described in only 2 of the cases. Twenty-one cases had some form of internal organ injury. This study serves to illustrate the relatively high incidence of lightning strikes in the region and calls for a more systematic and detailed investigative protocol in lightning-related deaths.     

"Insuring" the continued solvency of pharmaceutical companies in the face of product liability class actions

Costly product liability lawsuits continue to plague the pharmaceutical industry, and insurance to cover these losses is severely inadequate. Furthermore, questionable regulation of drugs exists once a pharmaceutical has passed FDA approval. This article describes a plan that uses a capitalistic, rather than a governmental, approach to solve both the insurance and the quality control problems. Although the proposed plan has never been used to insure pharmaceutical companies, different permutations of it have been used to insure other litigation-prone industries. Success from the proposed insurance entity results from the combined knowledge of scientists and actuaries to provide both protection from product liability lawsuits for the pharmaceutical industry and enhanced post-market surveillance of pharmaceuticals.     

Constructing Joseph Fredericks: competing narratives of a child sex murderer

Joseph Fredericks--one of Canada's most notorious sex offenders--was defined through the institutions that dealt with him from his infancy to his death to the inquest held after his death. In this paper, we locate in historical context and compare the different narratives that were constructed of his life in each of these institutional settings from unwanted child to 'mental defective' to psychiatric offender to criminal recidivist to victim to iconic sexual predator. We show that each of these narratives claimed to capture the essence of Fredericks in terms of what were his core characteristics and what remedies were necessary for the problems he posed only to be superseded by new narratives based on different assumptions. Finally, we show how one of these conceptions of Frederick's essence influenced a shift in Canadian public policy for sex offenders toward the greater emphasis on community protection characteristic of public policy in the United States.     

Factors related to suicide in New York state prisons

OBJECTIVE: Examine factors related to prison suicides to aid prevention. METHOD: Review the mental health records of all 76 suicides that occurred between 1993 and 2001 in New York State Department of Correctional Services (NYSDOCS) prisons that had some contact with mental health services during their incarceration. (This represented 84% of all NYSDOCS suicides.) Extract data from the psychological autopsies for a sample of 40 of these suicides. RESULTS: Of the suicide victims with some mental health contact, 95% had a substance abuse history, 70% displayed agitation or anxiety prior to the suicide, and 48% had a behavioral change. Common stressors preceding the suicide were inmate-to-inmate conflict (50%), recent disciplinary action (42%), fear (40%), physical illness (42%), and adverse information (65%) such as loss of good time or disruption of family/friendship relationships in the community. Forty-one percent had received a mental health service within 3 days of the suicide. Compared to the about 7200 inmates actively receiving mental health services in state prison, African-Americans and patients with a Major Mood (Bi-polar or Major Depression) were under-represented. Adjustment Disorder, Schizophrenia, and Personality Disorder diagnoses were over-represented. Suicide victims were more likely to have been incarcerated for a violent crime. CONCLUSION: Mental illness, anxiety/agitation, behavior change, stressors, history of substance abuse, and non-African-American were important risk factors.