Sheep-associated outbreak of Q fever, Idaho.

Between Feb 1 and Aug 31, 1984, an outbreak of 18 symptomatic cases of Q fever occurred in Idaho; these numbers represent an increase over the three cases reported in 1982 and the five reported in 1983. Four of the patients in the outbreak required hospitalization for two to five weeks; there were no fatalities. Eight of the cases had documented Q fever hepatitis, and one had pneumonia. All 18 of the 1984 cases for whom information was available were epidemiologically linked to visiting or working at a sheep research station and/or being exposed to animals from this research station. In this outbreak, patients typically had a hepatitislike illness associated with fever and severe headache. Severity of illness ranged from asymptomatic to life threatening. Cases of pneumonia and hepatitis due to Q fever continue to occur in the United States, especially among persons exposed to livestock.

Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database.

BACKGROUND: Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing. METHODS: National Alzheimer's Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer's Disease Center (ADC), and were coded as having an Alzheimer's disease clinical diagnosis or a nonprion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy. RESULTS: Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases. CONCLUSION: The proportion of patients with dementia with clinically unrecognized but autopsy-confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected clinically atypical cases of prion disease.

Creutzfeldt-Jakob disease in unusually young patients who consumed venison.

BACKGROUND: Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly consumed deer or elk meat created concern about the possible zoonotic transmission of CWD. OBJECTIVE: To examine the possible transmission of CWD to humans. PATIENTS: Three unusually young patients (aged 28, 28, and 30 years) with CJD in the United States during 1997-2000. METHODS: We reviewed medical records and interviewed family members and state wildlife and agriculture officials. Brain tissue samples were tested using histopathologic, immunohistochemical, immunoblot, or prion protein gene analyses. MAIN OUTCOME MEASURES: Presence or absence of established CJD risk factors, deer and elk hunting in CWD-endemic areas, and comparison of the evidence for the 3 patients with that of a zoonotic link between new variant CJD and bovine spongiform encephalopathy. RESULTS: None of the patients had established CJD risk factors or a history of travel to Europe. Two patients hunted game animals and 1 was a daughter of a hunter. Unlike patients with new variant CJD, the 3 patients did not have a unique neuropathologic manifestation, clinicopathologic homogeneity, uniformity in the codon 129 of the prion protein gene, or prion characteristics different from those of classic variants. CONCLUSIONS: Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.

Guillain-Barré syndrome in the United States, 1978-1981: additional observations from the national surveillance system.

During the period January 1978-March 1981, 2,575 cases of Guillain-Barré syndrome (GBS) were reported by participating neurologists in the national GBS surveillance system. The incidence of GBS was highest in the 50- to 74-year-old age group, but a lesser peak was observed in persons aged 15 to 35. The frequencies of antecedent respiratory (43%) and gastrointestinal (21%) illness exceeded frequencies of such illnesses in the US population (10 and 0.8%, respectively), based on survey data compiled by the National Center for Health Statistics; the differences in these frequencies of illness were similar in all seasons of the year, in males and in females, and in persons less than 6, 6 to 16, 17 to 44, and greater than 44 years of age. Nineteen percent of adult patients for whom information was available (67% of the total) reported receiving an A/New Jersey influenza vaccine in 1976, a lower percentage than would be expected on the basis of a survey conducted in that year. The data suggest that persons who received this vaccine have not been at increased risk and may even have been at decreased risk of acquiring GBS during the period covered by this study.

National surveillance for Guillain-Barré syndrome: January 1978-March 1979.

Between January 1, 1978, and March 31, 1979, 1,034 cases of Guillain-Barré syndrome (GBS) were reported to the Centers for Disease Control by the 1,813 American Academy of Neurology sentinel physicians who participated in the national GBS surveillance program. A direct correlation was observed between increasing age and the age-specific attack (incidence) rates. Based on the cases observed and the total US population, age-adjusted attack rates were statistically higher in males (0.52 per 100,000) than in females (0.40). Rates for whites were 0.44 and those for blacks 0.28 per 100,000; although the difference is statistically significant, uncertainties as to the true denominators by race preclude acceptance of these differences as valid. Sixty-seven percent, or 682 of the patients, reported that they had had an antecedent illness within 8 weeks before onset of GBS, and among them the peak period of onset of GBS was in the second week after the onset of the prior illness. There were also 52 patients (5%) who had undergone surgery and 45 (4.5%) who had received vaccinations, both within the 8 weeks before onset of GBS. However, the high proportions of antecedent illness in these groups (45% of those operated and 53% of those vaccinated) made attribution of GBS to the procedures tenuous. Risk of GBS in patients who reported receiving a swine influenza vaccination in 1976 was no greater than in those who reported that they did not receive this vaccine.

Fatal Guillain-Barré syndrome after the national influenza immunization program.

Fifty-eight fatal cases of Guillain-Barré syndrome (GBS) were reported during the 1976 to 1977 National Influenza Program: Thirty-two (58%) of these patients had received the A/New Jersey influenza vaccine. The mean interval from vaccination to onset was 3.9 weeks, and the incidence of preceding illness in vaccinated or unvaccinated patients was similar. Fifty-eight percent had at least one chronic disease before onset. The clinical features were similar in vaccinated and unvaccinated patients. Most deaths followed medical complications of respiratory paralysis: Fifteen had pneumonia, 29 (83%) died suddenly, 15 had sudden arrhythmias or hypotension, and 7 had myocardial infarction or pulmonary embolus.

Results of the national surveillance for Guillain-Barré syndrome.

The preliminary results of the national surveillance for Guillain-Barré syndrome(GBS) are reported. In the first 6 months of 1978, 327 cases of GBS were reported to the Center for Disease Control (CDC). A statistically significant difference was observed between sex-specific attack rates, and a direct correlation also was observed between advancing age and increasing risk of GBS.

Risk factors for Guillain-Barré syndrome.

In 100 cases of Guillain-Barré syndrome (GBS) reported from 10 metropolitan areas to the Centers for Disease Control (CDC) after the 1976-77 influenza vaccination campaign and matched associate or spouse controls, we searched for risk factors for GBS other than A/New Jersey/1976 influenza vaccination and acute respiratory infection. The 47 vaccinated cases recalled influenza vaccination in past years less frequently than did controls (p less than 0.025). Cases and controls did not differ in the number of previous vaccinations or in interval from last vaccination. Cases also gave a history of allergy less frequently than controls. There were no other significant differences.

HLA antigens in Guillain-Barré syndrome.

We compared Guillain-Barré syndrome (GBS) cases reported from cities in the United States in 1976-1977 with spouse or associate controls to detect possible HLA associations. HLA-A11 was somewhat less common among 92 cases than among 100 controls (p = 0.04). The 38 patients and 42 controls vaccinated against A/NJ/76 differed slightly in overall distribution of B locus antigens (p = 0.06), but the individual HLA-B antigen associations were more easily explained by chance. The 54 unvaccinated cases showed no apparent relation to HLA type. These findings should encourage further immunogenetic study of etiologically related GBS cases.

Creutzfeldt-Jakob disease after receipt of a previously unimplicated brand of dura mater graft.

BACKGROUND: Iatrogenic Creutzfeldt-Jakob disease (CJD) transmission via dura mater grafts has been reported in many countries. In September 1998, a 39-year-old Colorado woman was reported as having suspected CJD after receiving a dura mater graft 6 years earlier. METHODS: An investigation was initiated to confirm the diagnosis of CJD and assess the possible source of CJD transmission. The authors determined the presence or absence of other known CJD risk factors, checked for epidemiologic evidence of possible CJD transmission via neurosurgical instruments, and evaluated the procedures used in the collection and processing of the graft, including whether the donor may have had CJD. RESULTS: The CJD diagnosis was confirmed in the dural graft recipient by neuropathologic and immunodiagnostic evaluation of the autopsy brain tissue. She had no history of receipt of cadaveric pituitary hormones or corneal grafts or of CJD in her family. The authors found no patients who underwent a neurosurgical procedure within 6 months before or 5 months after the patient's surgery in 1992 who had been diagnosed with CJD. The dura mater was obtained from a 57-year-old man with a history of dysarthria, ataxia, and behavioral changes of uncertain origin. The graft was commercially prepared by use of a process that included treatment with 0.1 N sodium hydroxide and avoided commingling of dura from different donors. CONCLUSIONS: The patient's age, absence of evidence for other sources of CJD, the latent period, and the report of an unexplained neurologic illness in the donor of the dura mater indicate that the graft was the most likely source of CJD in this patient.

Guillain-Barré syndrome in Larimer County, Colorado: a high-incidence area.

During the period 1981-1983, 19 cases of Guillain-Barré syndrome (GBS) occurred in residents of Larimer County, Colorado, for an incidence of 4.0 cases per 100,000 population per year, compared with 1.2 cases per 100,000 per year in 1975-1980 (p less than 0.05). The higher incidence of GBS in 1981-1983 may represent an unusual chance occurrence, since no patient characteristics or predisposing events could be found to explain the increase. Nevertheless, the findings demonstrate that over a period of as long as 3 years, the crude average annual incidence of GBS in a large, well-defined population may exceed by twofold the upper limit of the previously reported range (0.6 to 1.9 cases per 100,000 per year).

Iatrogenic Creutzfeldt-Jakob disease at the millennium.

The causes and geographic distribution of 267 cases of iatrogenic Creutzfeldt-Jakob disease (CJD) are here updated at the millennium. Small numbers of still-occurring cases result from disease onsets after longer and longer incubation periods following infection by cadaveric human growth hormone or dura mater grafts manufactured and distributed before the mid-1980s. The proportion of recipients acquiring CJD from growth hormone varies from 0.3 to 4.4% in different countries, and acquisition from dura mater varies between 0.02 and 0.05% in Japan (where most cases occurred). Incubation periods can extend up to 30 years, and cerebellar onsets predominate in both hormone and graft recipients (in whom the site of graft placement had no effect on the clinical presentation). Homozygosity at codon 129 of the PRNP gene is over-represented in both forms of disease; it has no effect on the incubation period of graft recipients, but may promote shorter incubation periods in hormone cases. Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.

Risk of respiratory illness associated with day-care attendance: a nationwide study.

The risk of respiratory and other illnesses in children (age groups: 6 weeks through 17 months, 18 through 35 months, and 36 through 59 months) in various types of day-care facilities was studied. Children considered exposed to day care were those who were enrolled in day care with at least one unrelated child for at least 10 hours per week in each of the 4 weeks before the interview; unexposed children were not enrolled in any regular child care with unrelated children and did not have siblings younger than 5 years of age receiving regular care with unrelated children. Although an increased risk of respiratory illness was associated with attending day care for children in all three age groups, this risk was statistically significant only for children 6 weeks through 17 months of age (odds ratio = 1.6; 95% confidence interval = 1.1 to 2.4) and children 18 through 35 months of age who had no older siblings (odds ratio = 3.4; 95% confidence interval = 2.0 to 6.0). In contrast, day-care attendance was not associated with an increased risk of respiratory illness in children 18 through 35 months of age with older siblings (odds ratio = 1.0). For children aged 6 weeks through 17 months, the exposure to older siblings was associated with an increased risk of respiratory illness; however, for children aged 36 through 59 months, older siblings were protective against respiratory illness. In addition, for the children in each age group currently in day care, increased duration of past exposure to day care was associated with a decreased risk of respiratory illness.(ABSTRACT TRUNCATED AT 250 WORDS)

Changing epidemiology of Reye syndrome in the United States.

The average annual incidence of Reye syndrome reported through national surveillance was lower during 1981 to 1984 than during the previous five surveillance years. This is accounted for by a decrease in cases among children younger than 10 years of age; the number of cases in 10- to 19-year-old persons remained relatively stable during this period. The overall decline in incidence and the differing age-specific incidence trends are apparent for both varicella-associated cases and for nonvaricella-associated cases. During 1985, the incidence has been much lower than during any previous year since surveillance was initiated; this most recent decrease includes children 10 to 19 years of age. Independently conducted surveys suggest that the prevalence of salicylate use for viral illnesses has decreased among children in recent years, particularly among children younger than 10 years of age. The changing epidemiology of Reye syndrome may reflect, in part, the declining use of salicylates among children and teenagers in the United States.

Enzymatic alterations in Reye's syndrome: prognostic implications.

Serial studies on serum enzyme and isoenzyme abnormalities in seven cases of Reye's syndrome were found to segregate patients into three categories of organ involvement. The patients with both extensive hepatic and previously unrecognized skeletal muscle involvement died despite therapeutic measures. The other two groups were characterized by enzyme and isoenzyme changes consistent with either predominantly muscular or hepatic involvement. The prognostic value of these abnormalities has been substantiated in subsequent cases of this syndrome. These findings suggest that those patients at greatest risk may be identified early, thus improving evaluation of the efficacy of specific therapeutic interventions in this disease.

National surveillance for Reye syndrome: a five-year review.

National surveillance for Reye syndrome conducted during five years, including the period 1973-1974 and December 1976 through November 1980, has resulted in the reporting of more than 2,000 cases of Reye syndrome. The highest reported incidence of Reye syndrome occurred during years of primary influenza B and A (H1N1) activity; the reported incidence during one period of influenza A (H3N2) activity was somewhat lower. Regional outbreaks of Reye syndrome have been associated with influenza A (H1N1) and B but now with influenza A (H3N2). Cases of Reye syndrome in whites tend to be distributed throughout all age groups whereas a large percentage of cases in blacks have been reported in infants less than 1 year of age in three of the past four years. Nationally, there has been a decline in the case-fatality ratio in recent years.

National Reye syndrome surveillance, 1982.

Despite the fact that influenza B was the primary influenza virus strain during the winter of 1981-1982, only 213 cases of Reye syndrome were reported to the Centers for Disease Control (CDC) between Dec 1, 1981 and Nov 30, 1982. This national reported incidence of 0.33 cases per 100,000 children less than 18 years of age is the lowest reported incidence since the Centers for Disease Control began surveillance in 1973. This relatively low incidence probably reflected, at least in part, the fact the influenza B activity was spotty and the illness relatively mild the winter of 1981-1982. The 213 cases were reported from 43 states; and in 56% of the patients, Reye syndrome occurred following a respiratory illness. The mean age of the children was 7.0 years; there were equal numbers of girls and boys; and 93% were white. Of the ten black patients, 80% were less than 1 year of age compared with 9% of the white patients. Of the 208 patients with reported admission stage, 45% were admitted in stage I or 0, a slightly lower proportion than that observed in the previous 2 years. Salicylate levels were obtained in 55% of the patients and were reported as "detectable" in 81% compared with 96% in 1981 (P = .003, chi 2). Of the 200 patients with known outcome, 70 patients died (a case fatality ratio of 35%).

Outbreak of Kawasaki syndrome in Denver, Colorado: association with rug and carpet cleaning.

Between October 1984 and January 1985, the largest outbreak of Kawasaki syndrome reported to date in the continental United States (62 cases) occurred in the Front Range of the Rocky Mountains, extending from Colorado Springs, Colorado, to Cheyenne, Wyoming. Fifty-two (84%) of these Kawasaki syndrome patients lived in the Denver metropolitan area. A case-control study revealed that 16 (62%) of 26 Kawasaki syndrome patients compared with 10 (20%) of 49 matched control subjects had a history of exposure to shampooed (19%) or spot-cleaned (81%) rugs or carpets within 30 days of the Kawasaki syndrome onset date (odds ratio = 5, P less than .01). The time of exposure to shampooed or spot-cleaned rugs or carpets for 9 of 10 Kawasaki syndrome patients who had a single exposure and for all 6 Kawasaki syndrome patients who had multiple exposures were clustered within an interval 13 to 30 days before the onset of illness. Although the reason for this unusually large outbreak remains obscure, it is the third in which a statistically significant association between Kawasaki syndrome and rug or carpet cleaning has been found.

Kawasaki syndrome among American Indian and Alaska Native children, 1980 through 1995.

BACKGROUND: Kawasaki syndrome (KS) is a leading cause of acquired heart disease among US children, but the epidemiologic features of KS among American Indian and Alaska Native (AI/AN) children have not been described. METHODS: We examined Indian Health Service computerized records of hospital discharges for AI/AN children <18 years of age with KS during 1980 through 1995. RESULTS: During 1980 through 1995, 85 AI/AN children were reported with a hospitalization for KS; 10 of the children had an additional KS hospitalization record within 5 months. The average annual KS hospitalization rate for children <5 years of age, based on first KS hospitalization only, was 4.3 cases per 100000 children; the rate for children age <1 year (n = 21) was 8.6 per 100000 and for children ages 1 to 4 years was 3.6 per 100000. The annual rates for children < 5 years of age ranged from 0 to 8.5 per 100000 children. KS hospitalizations for children peaked in January and February; 50.6% of the children were hospitalized during January through April. The overall median length of hospital stay was 4 days (range, 1 to 29 days); the median duration decreased from 8 days from 1980 through 1982 to 4 days from 1993 through 1995. CONCLUSIONS: The overall annual hospitalization rate of KS among AI/AN children <5 years of age was slightly lower than rates for several majority white populations in the United States. (4.6 to 15.2 cases per 100000) and much lower than rates for blacks and Asians/Pacific Islanders.

Outbreak of poliomyelitis-like paralysis associated with enterovirus 71.

In the summer of 1987 five children were seen at The Children's Hospital of Philadelphia because of acute onset of flaccid paralysis of an arm or leg(s). Although there were documented exposures to oral poliovirus vaccine and coxsackievirus B3 in some of the cases, the clinical, epidemiologic and laboratory findings indicate that enterovirus 71 was the common etiologic agent for this unusual outbreak of poliomyelitis-like paralysis. Of the five children three recovered completely; the other two had residual paralysis with weakness and muscle wasting. Imaging studies of the spinal cord in the two children with residual paralysis revealed defects in the ventral aspect of the spinal cord. This series of paralytic cases attributed to enterovirus 71 is the largest reported in the United States.