Creutzfeldt-Jakob disease associated with a deletion of two repeats in the prion protein gene.

A two-octapeptide repeat deletion of the prion protein gene has been recently observed in a patient with a 2-year history of dementia and a clinical diagnosis of possible Creutzfeldt-Jakob disease (CJD). The authors report a similar deletion in a patient with a definitive diagnosis of CJD. Since the two-repeat deletion has not been observed in large, population-based studies, the two cases suggest that this deletion is a new pathogenic mutation associated with CJD.

Cystic teratoma in a supernumerary ovary of the greater omentum. A case report.

Supernumerary ovary is a rarely described gynecologic finding. A 47-year-old woman presented with abdominal pain and a palpable lower abdominal mass. The mass, removed in a partial omentectomy, was found to consist of a supernumerary ovary with a cystic teratoma.

Helicopter wire strike accident and high voltage electrocution: a case report.

Helicopter wire-strike accidents usually involve high-voltage lines or telephone wires where the resulting impact is low velocity with minimal loss of occupiable space. Death and injury in these mishaps is often due to blunt force trauma to the head. In a recent wire strike accident investigated by the Division of Aerospace Pathology at The Armed Forces Institute of Pathology, the circumstances suggest that death of both aviators was due to high-voltage electrocution. Evidence surrounding the case and high-voltage electrocution are discussed.

Sickle-cell trait as a risk factor for sudden death in physical training.

Case reports of sudden death during exertion have not established an association between the sickle-cell trait (hemoglobin AS) and exercise-related death. To test this association, all deaths occurring among 2 million enlisted recruits during basic training in the U.S. Armed Forces in 1977 to 1981 were classified from autopsy and clinical records as non-sudden deaths or as sudden deaths explained or unexplained by preexisting disease. On the basis of known numbers of entering recruits (according to race, age, and sex) and published prevalence rates for hemoglobin AS (8 percent for black and 0.08 percent for nonblack recruits), death rates (per 100,000) were 32.2 for sudden unexplained deaths, 2.7 for sudden explained deaths, and 0 for non-sudden deaths among black recruits with hemoglobin AS, as compared with 1.2, 1.2, and 0.7 among black recruits without hemoglobin S and 0.7, 0.5, and 1.1 among nonblack recruits without hemoglobin S. Among black recruits the relative risk of sudden unexplained death (hemoglobin AS vs. non-hemoglobin S) was 27.6 (95 percent confidence interval, 9 to 100; P less than 0.001), whereas among all recruits this risk was 39.8 (95 percent confidence interval, 17 to 90; P less than 0.001). The relative risk of sudden unexplained death among all recruits increased with age (P less than 0.04), from 13 (ages 17 to 18) to 95 (ages 26 to 30). We conclude that recruits in basic training with the sickle-cell trait have a substantially increased, age-dependent risk of exercise-related sudden death unexplained by any known preexisting cause.

Injury pattern analysis of helicopter wire strike accidents (-Gz load).

Injury patterns in rotary wing aircraft wire strike accidents were reviewed to determine mechanisms of injury. It was found that U.S. Army Safety Center data showed that between 1 January 1974 and 31 August 1981 there were 167 wire strikes involving Army helicopters which resulted in 60 injuries and 34 fatalities at a cost of $12,809,100. Updated data on all military rotary wing aircraft accidents investigated between 1978 and 1982 were screened by the Division of Aerospace Pathology to determine the mechanisms of injury to flight deck personnel. From 13 December 1978 to 23 June 1982, three types of rotary wing aircraft were in eight fatal accidents. These mishaps accounted for 28 casualties: 14 fatalities and 14 injuries. Aviators comprised 64.4% of the fatalities. Injury pattern analysis showed 100% had major head and neck injuries with 66% having basilar skull fractures. Two-thirds had associated mandibular fractures or evidence of impact forces transmitted through the mandible to the skull. The same number had wedge-shaped chin lacerations from impact with the cyclic control stick. We postulate transmission of lethal impact forces primarily in the +Gz direction through the mandible to the skull. This suggests either improper use and/or failure of the seat and restraint systems.