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The Epidemiology of Primary Amoebic Meningoencephalitis in the USA, 1962–2008

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The Epidemiology of Primary Amoebic Meningoencephalitis in the USA, 1962–2008 Epidemiol. Infect. (2010), 138, 968–975. f Cambridge University Press 2009 This is a work of the U.S. Government and is not subject to copyright protection in the United States doi:10.1017/S0950268809991014 The epidemiology of primary amoebic meningoencephalitis in the USA, 1962–2008 J. S. YODER 1*, B. A. EDDY1,2,G.S.VISVESVARA1,L.CAPEWELL1 AND M. J. BEACH 1 1 Division of Parasitic Diseases, Centers for Disease Control and Prevention, Atlanta, GA, USA 2 Rollins School of Public Health, Emory University, Atlanta, GA, USA (Accepted 18 September 2009; first published online 22 October 2009) SUMMARY Naegleria fowleri, a free-living, thermophilic amoeba ubiquitous in the environment, causes primary amoebic meningoencephalitis (PAM), a rare but nearly always fatal disease of the central nervous system. While case reports of PAM have been documented worldwide, very few individuals have been diagnosed with PAM despite the vast number of people who have contact with fresh water where N. fowleri may be present. In the USA, 111 PAM case-patients have been prospectively diagnosed, reported, and verified by state health officials since 1962. Consistent with the literature, case reports reveal that N. fowleri infections occur primarily in previously healthy young males exposed to warm recreational waters, especially lakes and ponds, in warm-weather locations during summer months. The annual number of PAM case reports varied, but does not appear to be increasing over time. Because PAM is a rare disease, it is challenging to understand the environmental and host-specific factors associated with infection in order to develop science-based, risk reduction messages for swimmers. Key words: Infectious disease epidemiology, parasitic disease epidemiology and control, water-borne infections. INTRODUCTION via the olfactory nerve. The incubation period may vary from 2 to 15 days. Signs and symptoms of Primary amoebic meningoencephalitis (PAM) is a infection are similar to those of bacterial or viral rare but nearly always fatal disease caused by infec- meningitis and include headache, fever, stiff neck, tion with Naegleria fowleri, a thermophilic, free-living anorexia, vomiting, altered mental status, seizures, amoeba found in freshwater environments [1, 2]. and coma. Death typically occurs 3–7 days after onset PAM occurs in otherwise healthy individuals with of symptoms. Autopsy findings show acute haemor- exposure to warm, untreated or poorly disinfected rhagic necrosis of the olfactory bulbs and cerebral water. Infection results from water containing N. cortex [3]. fowleri entering the nose, followed by migration of While there are more than 40 species of Naegleria the amoebae through the cribriform plate to the brain [4], only one species, N. fowleri, causes PAM. N. fowleri can tolerate temperatures of up to 45 xC and proliferate in natural bodies of water (e.g. lakes * Author for correspondence: J. S. Yoder, 4770 Buford Highway, NE Mail Stop F-22, Atlanta, GA 30341, USA. and rivers) during warmer months of the year when (Email: [email protected]) the ambient temperature increases. N. fowleri is Downloaded from https://www.cambridge.org/core. IP address: 170.106.33.14, on 25 Sep 2021 at 20:20:41, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0950268809991014 Epidemiology of N. fowleri infections 969 typically free-living and feeds on bacteria in the Disease and Outbreak Surveillance System, which has water; PAM occurs when there is accidental intro- tracked PAM case reports since 1989; (2) the com- duction of the amoeba into the human nose. N. fowleri pressed mortality file of the National Vital Statistics has been detected in water sampling from freshwater System, searching on International Classification of lakes, ponds, and rivers [5–7], thermally polluted Diseases, Ninth Revision (ICD-9) code 136.2 (specific water [8–10], geothermally heated water such as hot infections by free-living amoebae) and ICD-10 code springs [11], warm groundwater [12], and soil [13]. B60.2 (naegleriasis) for the period 1979–2008; (3) No reliable estimates exist on the occurrence of medical literature review of reported PAM case- PAM worldwide. Since being first described in 1965 patients; (4) searches of media reports since 1990; and [14], N. fowleri has been identified as the cause of (5) CDC laboratory test requests and results. Case- PAM in >16 countries [15]. However, relatively few patients identified from these sources were verified persons are diagnosed with PAM despite the millions with public health officials from the state of diagnosis, of persons who have contact with warm freshwater and methods of diagnoses were reviewed by the CDC annually. The common finding of antibodies to the parasitic disease laboratory. amoeba in humans [16, 17] and the frequent finding of Case reports were included if laboratory-confirmed N. fowleri in these waters [5–12] suggests that ex- detection of N. fowleri organisms or nucleic acid was posure to the amoeba is much more common than the reported in CSF, biopsy, or tissue specimens. Case- incidence of PAM suggests. patients were classified on the basis of their state Previous reports have linked N. fowleri infections in of exposure instead of their state of diagnosis or the USA to water exposure in warm-weather states, residence. The method of identifying, verifying, and particularly among young males [15]. Water-related classifying case-patients and the amount of infor- activities previously identified as risk factors include mation included differs from an earlier N. fowleri re- swimming [18, 19], diving [20], use of watercraft [21], view [15] and a brief summary of N. fowleri cases [24], exposure to hot springs [22], and submersion in un- producing slightly different results. An additional 11 treated drinking water [23]. Because of the low num- case-patients died after N. fowleri infection during ber of reported case reports, it is difficult to quantify 1937–1961 [15, 25, 26]. These case reports met CDC’s the risk of these or other water-related activities. laboratory confirmation criteria and were state- While N. fowleri infections are rare, they are nearly verified but are not included in this analysis because always fatal, prompt diagnosis is difficult, and com- they were retrospectively diagnosed from preserved municating risk reduction messages to the public is autopsy specimens from a single state. Since this type challenging. In order to effectively address these con- of retrospective diagnosis was not conducted in other cerns, medical professionals and public health prac- states, the inclusion of those case-patients was felt titioners need science-based and consistent approaches to create a state-specific bias in the interpretation of to N. fowleri diagnosis and treatment, case reporting, these results. environmental sampling, and risk-reduction mess- ages. It is also critical to understand whether the RESULTS number and geographic distribution of case-patients is changing over time and whether there is potential During 1962–2008, 111 case reports of PAM (range for global climate change to play a role in increasing 0–8 cases per year) occurred in the USA (Fig. 1). The the risk of future N. fowleri infections. annual number of reported case reports probably in- creased in 1978 due to the establishment of the free- living amoeba registry at CDC. The six highest annual METHODS totals of case reports were 1980 (eight case reports), While N. fowleri is not a notifiable disease in the USA, 1978, 1987, 1995, 2002, and 2007 (six case reports CDC has maintained a free-living amoeba laboratory each). During 1962–2008, the median age of case- and registry since 1978 with a decided focus on iden- patients was 12 years (range 8 months to 66 years) tifying cases occurring in the USA. This registry and with 62.2% (n=69) being children (aged f13 years) other data sources were used to identify and describe (Fig. 2). Of the 111 case-patients, males accounted for all PAM case-patients prospectively diagnosed and 88 (79.3%) of the cases-patients (Fig. 2). Where race reported in the USA. The additional data sources and/or ethnicity was recorded, race was documented utilized for this review included: (1) the Waterborne as White for 28 case-patients and Black for three Downloaded from https://www.cambridge.org/core. IP address: 170.106.33.14, on 25 Sep 2021 at 20:20:41, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0950268809991014 970 J. S. Yoder and others 9 AR Unknown F year 6 8 AR 18 F 7 FL M 10 6 CA FL AZ FL AZ AZ F M F M M M 9 15 9 14 5 14 5 FL FL LA CA TX FL AZ AZ FL M M F M M M M M M 10 6 2 29 11 14 66 5 10 4 FL FL NM FL TX NC OK FL FL Number of case reports M M M M M M M M M 12 12 30 19 9 16 11 12 11 3 VA CA GA FL TX MO SC NC TX OK CA TX FL OK FL M F F M F F M M M F M M M M M 17 16 14 14 23 11 12 4 4 3 15 8 8 9 14 2 FL FL FL VA GA GA FL SC FL FL TX SC TX FL TX AR TX NC TX TX TX FL TX GA OK AZ TX CA M M M M M F M M M M F M M F M M M F M F M M F M M M M M 10 16 7 14 7 16 1 13 45 54 37 13 12 8 5 23 4 3 13 6 10 14 9 11 7 6 12 9 1 FL FL VA VA VA VA SC MS U* FL FL U* TX SC TX U* TX CA TX TX TX FL U* NV TX AR TX AZ TX TX CA TX FL TX TX TX NC TX GA TX OK M M M M F M M M M M M M F M M F M M M M M M M M M M M F M M M M M M M F M F M M F 18 18 14 27 15 24 16 7 1 11 16 30 17 8 6 14 3 12 9 3 10 2 11 31 12 9 27 <1 7 11 23 9 19 13 9 11 12 7 12 22 14 0 1962 1964 1966 1968 1970 1972 1974 1976 1978 1980 1982 1984 1986 1988 1990 1992 1994 1996 1998 2000 2002 2004 2006 2008 Year Fig.
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