A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea

Kuru is so far the principal human epidemic prion disease. While its incidence has steadily declined since the cessation of its route of transmission, endocannibalism, in Papua New Guinea in the 1950s, the arrival of variant Creutzfeldt–Jakob disease (vCJD), also thought to be transmitted by dietary prion exposure, has given kuru a new global relevance. We investigated all suspected cases of kuru from July 1996 to June 2004 and identified 11 kuru patients. There were four females and seven males, with an age range of 46–63 years at the onset of disease, in marked contrast to the age and sex distribution when kuru was first investigated 50 years ago. We obtained detailed histories of residence and exposure to mortuary feasts and performed serial neurological examination and genetic studies where possible. All patients were born a significant period before the mortuary practice of transumption ceased and their estimated incubation periods in some cases exceeded 50 years. The principal clinical features of kuru in the studied patients showed the same progressive cerebellar syndrome that had been previously described. Two patients showed marked cognitive impairment well before preterminal stages, in contrast to earlier clinical descriptions. In these patients, the mean clinical duration of 17 months was longer than the overall average in kuru but similar to that previously reported for the same age group, and this may relate to the effects of both patient age and PRNP codon 129 genotype. Importantly, no evidence for lymphoreticular colonization with prions, seen uniformly in vCJD, was observed in a patient with kuru at tonsil biopsy

Nurses\u27 Alumnae Association Bulletin - Volume 7 Number 11

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Genetic susceptibility, evolution and the kuru epidemic

The acquired prion disease kuru was restricted to the Fore and neighbouring linguistic groups of the Papua New Guinea highlands and largely affected children and adult women. Oral history documents the onset of the epidemic in the early twentieth century, followed by a peak in the mid-twentieth century and subsequently a well-documented decline in frequency. In the context of these strong associations (gender, region and time), we have considered the genetic factors associated with susceptibility and resistance to kuru. Heterozygosity at codon 129 of the human prion protein gene (PRNP) is known to confer relative resistance to both sporadic and acquired prion diseases. In kuru, heterozygosity is associated with older patients and longer incubation times. Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are predominantly PRNP codon 129 heterozygotes and this group show marked Hardy–Weinberg disequilibrium. The deviation from Hardy–Weinberg equilibrium is most marked in elderly women, but is also significant in a slightly younger cohort of men, consistent with their exposure to kuru as boys. Young Fore and the elderly from populations with no history of kuru show Hardy–Weinberg equilibrium. An increasing cline in 129V allele frequency centres on the kuru region, consistent with the effect of selection in elevating the frequency of resistant genotypes in the exposed population. The genetic data are thus strikingly correlated with exposure. Considering the strong coding sequence conservation of primate prion protein genes, the number of global coding polymorphisms in man is surprising. By intronic resequencing in a European population, we have shown that haplotype diversity at PRNP comprises two major and divergent clades associated with 129M and 129V. Kuru may have imposed the strongest episode of recent human balancing selection, which may not have been an isolated episode in human history

Episodic memories among irritable bowel syndrome (IBS) patients: An important aspect of the IBS symptom experience

Objective: Some IBS patients possess detailed memories of the events surrounding their bowel symptom onset ( episodic memories ). In this exploratory study we sought to: (1) examine memory relationship with gastrointestinal (GI) symptom severity, extraintestinal symptoms, and mood; (2) qualitatively explore memory valence and content in IBS patients with or without episodic memories. Methods: Referral IBS patients Results: 14/29 (48.3%) of IBS subjects endorsed episodic memories of IBS symptom onset, often GI infections/enteritis (35.7%). Recall of the exact year (69%) and month (60%) of symptom onset were common. Episodic memories were associated with greater IBS symptom severity/bother, higher anxiety/depression, and poorer HRQOL. Though AMT and SCEPT memory specificity were not different based on episodic memories, overgeneralization to negatively-valenced cues in the AMT was associated with more severe IBS in those without episodic memory. Qualitative analysis revealed no observable differences in topic focus of IBS patients with and without episodic memories. Conclusions: IBS patients often endorse episodic memories associated with symptom onset, and this recall seems to associate with more severe symptoms. Overgeneralization responses to negative stimuli may lead to worse bowel symptoms in those without episodic memories. IBS memory specificity may associate with qualitative differences in processing psychosocial experiences and might be important to IBS pathophysiology

What´s the risk? Fearful individuals generally overestimate negative outcomes and they dread outcomes of specific events

Although it is an adaptive mechanism that danger usually elicits fear, it seems that fearful individuals overestimate the danger associated with their feared objects or situations. Previous research has not systematically distinguished between the estimated risk of an encounter with fear-relevant stimuli and the expected unpleasant outcomes of such encounters. Furthermore, it is not clear if biased risk perception is specific to an individual’s fear or generalized to all negative events. In an online-survey (N = 630) we assessed the estimated risk to encounter fear-relevant stimuli and the expectations of negative outcomes of such encounters. Items contained three domains (spiders, snakes, and everyday fear triggers). In regression analyses we examined the specific associations between fear and risk estimations. In addition, we compared subgroups with specific fears and low fearful individuals. While an individual’s fear score was not related to the estimated risk of an encounter with fear-specific stimuli, it was related to an overestimation of negative outcomes in all domains. The perceived risk of aversive outcomes was most pronounced for an individual’s specific fear. Furthermore, an individual’s specific fear was most predictive of the estimated risk of a negative fearrelevant outcome. Highly fearful individuals overestimate the risk of negative outcomes of fear-relevant encounters. Specifically, they dread outcomes of encounters with their feared object. Differentiating fear-relevant components of risk perception provides insights into the cognitions which may motivate maladaptive avoidance behavior

Summary of Total Mercury Concentrations in Fillets of Selected Sport Fishes Collected during 2000–2003 from Lake Natoma, Sacramento County, California

This report summarizes results of total mercury measurements in skinless fillets of sport fishes collected during August 2000, September–October 2002, and July 2003 from Lake Natoma, a small (8,760 acre-feet) afterbay for Folsom Dam on the lower American River. The primary objective of the study was to determine if mercury concentrations in fillets approached or exceeded guidelines for human consumption. The Food and Drug Administration (FDA) human-health action level for methylmercury in commercially caught fish is 1.0 μg/g (microgram per gram); the U.S. Environmental Protection Agency (USEPA) human-health criterion for methylmercury residue in fish tissue is 0.30 μg/g. Wet weight concentrations of total mercury in skinless fillets were as high as 0.19 μg/g in bluegill (Lepomis macrochirus), 0.39 μg/g in redear sunfish (L. microlophus), 1.02 μg/g in largemouth bass (Micropterus salmoides), and 1.89 μg/g in channel catfish (Ictalurus punctatus). Maximum concentrations of mercury in other fish species varied from 0.10 μg/g in rainbow trout (Oncorhynchus mykiss) to 0.56 μg/g in white catfish (Ameiurus catus). Altogether, 1 of 86 largemouth bass and 11 of 11 channel catfish exceeded the FDA human-health action level. In addition, 1 of 20 redear sunfish, 26 of 86 largemouth bass, 2 of 3 spotted bass (M. punctulatus), 1 of 1 brown bullhead (A. nebulosus), and 1 of 1 white catfish exceeded the USEPA human-health criterion. These results indicate that some fish species inhabiting Lake Natoma contain undesirably high concentrations of mercury in their skinless fillets

Summary of Total Mercury Concentrations in Fillets of Selected Sport Fishes Collected during 2000–2003 from Lake Natoma, Sacramento County, California

This report summarizes results of total mercury measurements in skinless fillets of sport fishes collected during August 2000, September–October 2002, and July 2003 from Lake Natoma, a small (8,760 acre-feet) afterbay for Folsom Dam on the lower American River. The primary objective of the study was to determine if mercury concentrations in fillets approached or exceeded guidelines for human consumption. The Food and Drug Administration (FDA) human-health action level for methylmercury in commercially caught fish is 1.0 μg/g (microgram per gram); the U.S. Environmental Protection Agency (USEPA) human-health criterion for methylmercury residue in fish tissue is 0.30 μg/g. Wet weight concentrations of total mercury in skinless fillets were as high as 0.19 μg/g in bluegill (Lepomis macrochirus), 0.39 μg/g in redear sunfish (L. microlophus), 1.02 μg/g in largemouth bass (Micropterus salmoides), and 1.89 μg/g in channel catfish (Ictalurus punctatus). Maximum concentrations of mercury in other fish species varied from 0.10 μg/g in rainbow trout (Oncorhynchus mykiss) to 0.56 μg/g in white catfish (Ameiurus catus). Altogether, 1 of 86 largemouth bass and 11 of 11 channel catfish exceeded the FDA human-health action level. In addition, 1 of 20 redear sunfish, 26 of 86 largemouth bass, 2 of 3 spotted bass (M. punctulatus), 1 of 1 brown bullhead (A. nebulosus), and 1 of 1 white catfish exceeded the USEPA human-health criterion. These results indicate that some fish species inhabiting Lake Natoma contain undesirably high concentrations of mercury in their skinless fillets