Outbreak of type C botulism in captive wild birds.

In late summer 2010, an outbreak of type C botulism affected the birds kept in a dam at a southern Brazilian zoo. A total of 14(10 black-necked swans, Cygnus melancoryphus; 3 Muscovy ducks, Cairina moschata; and 1 fulvous whistling-duck, Dendrocygna bicolor) out of 100 birds died after showing flaccid paralysis of the skeletal muscles characterized by general locomotion deficit, flight and swimming disorders, dropped neck, and severe dyspnea. Carcasses of dead birds (some infested by larvae of sarcophagus fly) scattered in the bird enclosure, and oxygen-free, organically rich mud and/or shallow standing waters present at the edges of the weir were identified as possible toxin sources. Postmortem examinations revealed no significant pathological changes. Epidemiologic and clinical findings indicated the diagnosis of type C botulism toxin, which was confirmed by mouse bioassay and seroneutralization.

A controlled evaluation of case clinical effect coding by poison center specialists for detection of WMD scenarios.

BACKGROUND: Many public health entities employ computer-based syndromic surveillance to monitor for aberrations including possible exposures to weapons of mass destruction (WMD). Often, this is done by screening signs and symptoms reported for cases against syndromic definitions. Poison centers (PCs) may offer significant contributions to public health surveillance because of their detailed clinical effect data field coding and real-time data entry. Because improper clinical effect coding may impede syndromic surveillance, it is important to assess this accuracy for PCs. METHODS: An AAPCC-certified regional PC assessed the accuracy of clinical effect coding by specialists in poison information (SPIs) listening to audio recordings of standard cases. Eighteen different standardized cases were used, consisting of six cyanide, six botulism, and six control cases. Cases were scripted to simulate clinically relevant telephone conversations and converted to audio recordings. Ten SPIs were randomly selected from the center's staff to listen to and code case information from the recorded cases. Kappa scores and the percentage of correctly coding a present clinical effect were calculated for individual clinical effects summed over all test cases along with corresponding 95% confidence intervals. The rate of the case coding by the SPIs triggering the PC's automated botulism and cyanide alerts was also determined. RESULTS: The kappa scores and the percentage of correctly coding a present clinical effect varied depending on the specific clinical effect, with greater accuracy observed for the clinical effects of vomiting and agitation/irritability, and poor accuracy observed for the clinical effects of visual defect and anion gap increase. Lack of correct coding resulted in only 60 and 86% of the cases that met the botulism and cyanide surveillance definitions, respectively, triggering the corresponding alert. There was no difference observed in the percentage of coding a present clinical effect between certified (9.0 years experience) and non-certified (2.4 years experience) specialists. There were no cases of coding errors that resulted in the triggering of a false positive alert. CONCLUSION: The success of syndromic surveillance depends on accurate coding of signs and symptoms. Although PCs generally contribute high-quality data to public health surveillance, it is important to recognize this potential weak link in surveillance methods.

Denmark: botulism in an infant or infant botulism?

A 4.5 months old, previously healthy Danish girl was admitted to a paediatric department after six days of passive behaviour and weak suck. Over the next days she became increasingly weak, developed bilateral ptosis, the muscle stretch reflexes were lost, and mydriasis with slow pupillary responses was noted. Botulism was suspected and confirmed by testing of patient serum in a bioassay. The condition of the patient improved following administration of botulism antiserum. The clinical picture was suggestive of intestinal (infant) botulism. However, botulism acquired from consumption of food with preformed neurotoxin could not be excluded.

Botulinum toxins--cause of botulism and systemic diseases?

Toxins of Clostridium botulinum (types A-G) are known as 'neurotoxins', causing the clinically well-known picture of flaccid muscular paralysis. The molecular biological background is the blocking of acetylcholine secretion in neuromuscular junctions by enzymatic cleavage of molecules forming the machinery of exocytosis. Two 'non-neurotoxins' (types C2, C3) are produced by some strains of C. botulinum types C and D. These affect the cytoskeleton by ribosylating actin filaments. All these toxins are used as cell biological tools for the study of specific actions and effects in different eukaryotic cells. Pharmaceutical and molecular biological research has shown their influence on several crucial organs (or cell cultures thereof) of humans and animals (brain and spinal cord, cerebellum, hippocampus, hypophysis, pancreas, adrenal glands, salivary glands and others). Under natural conditions, botulinum toxins may pass the intestinal barrier and circulate in the bloodstream for a certain time. Carriers occurring naturally in food, such as wheat germ agglutinin, digitonin or saponin, and bacterial toxins such as streptolysin O, perfringolysins, C2 toxin or botulinolysin may also form pores in cell walls. They facilitate the entry of botulinum toxins into cells that may not have natural binding receptors. It is concluded that in vivo actions of different botulinum toxins after their entry into the organism may contribute to the onset of different diseases of hitherto cryptogenic origin. Some examples are given and future problems are discussed.

Medical aspects of biologic toxins.

Biologic toxins are molecules produced by living organisms that are poisonous to other species, such as humans. Some biologic toxins are so potent and relatively easy to produce that they have been classified as biothreat agents. These include the botulinum neurotoxins, ricin, staphylococcal enterotoxin B, and Clostridium perfringens epsilon toxin. This article focuses on these four biothreat toxins and their medical aspects. The majority of the article is spent on the botulinum neurotoxins, because these are the most poisonous substances known and are the only toxins classified as Category A threat agents-the highest level of threat agent. The remainder of the article is devoted to sections on the other three biothreat toxins: ricin, staphylococcal enterotoxin B, and C perfringens epsilon toxin.

Botulism: update and review.

Botulism is both an old and an emerging disease. Over 100 years ago, the classic food-borne type was found to be caused by ingesting contaminated food containing the toxin produced by a bacteria. In the first half of the 20th century a second form, wound botulism, was discovered. Three additional forms (infant, hidden, and inadvertent) were first described in the last quarter of the 20th century. Our understanding of how botulinum toxin blocks the release of acetylcholine at the neuromuscular junction has been clarified in the past 10 years. In the past 20 years, we have witnessed one of the strangest of all ironies in the history of medicine. The very lethal botulinum toxin is now being used as a treatment in an expanding list of disorders. Research is advancing in several directions. These new avenues include improved methods of preventing and treating botulism and additional novel uses of botulinum toxin as a therapeutic agent. In this article, the five clinical forms of botulism, the actions of botulinum toxins, electrodiagnostic methods, treatments, and possible future directions are discussed.

Clostridium botulinum and the clinical laboratorian: a detailed review of botulism, including biological warfare ramifications of botulinum toxin.

OBJECTIVE: This review article is designed to thoroughly familiarize all health care professionals with the history, classification, epidemiology, clinical characteristics, differential diagnosis, diagnostic evaluation (including laboratory-based testing), treatment, and prognosis of botulism. It is especially targeted toward clinical laboratorians and includes a detailed enumeration of the important clinical laboratory contributions to the diagnosis, treatment, and monitoring of patients with botulism. Finally, the bioterrorism potential for botulism is discussed, with an emphasis on the clinical laboratory ramifications of this possibility. DATA SOURCES: Included medical periodicals and textbooks accessioned from computerized and manual medical literature searches. More than 1000 medical works published from the 1800s through 2003 were retrieved and reviewed in this process. DATA SYNTHESIS: Pertinent data are presented in textual and tabular formats, the latter including 6 tables presenting detailed information regarding the clinical parameters, differential diagnosis, diagnostic studies, laboratory testing, and therapeutic approaches to botulism. CONCLUSIONS: Because botulism is such a rare disease, a keen awareness of its manifestations and prompt diagnosis are absolutely crucial for its successful treatment. The bioterrorism potential of botulism adds further urgency to the need for all health care professionals to be familiar with this disease, its proper evaluation, and timely treatment; the need for such urgency clearly includes the clinical laboratory.

Botulism type B presenting as pure autonomic dysfunction.

Botulism nowadays is a rare mostly food-borne disease caused by the toxin of Clostridium botulinum. On the other hand, botulinum toxin blocking cholinergic transmission has become a most powerful treatment option for many focal movement disorders and is increasingly used to treat autonomic disorders [2, 7, 8]. Although muscle weakness is the hallmark of botulism, autonomic dysfunction may be the leading clinical symptom in rare cases and should be included in the differential diagnosis of pure dysautonomia. Here, we describe a patient with autonomic dysfunction as the leading symptom of botulism type B.

Botulismo y toxina botulínica / Botulism and botulinum toxin

El botulismo es una toxi-infección producida por la bacteria anaerobia Clostridium botulinum por medio de una potentísima toxina, la toxina botulínica, de la cual existen ocho serotipos diferentes. Ésta es capaz de provocar en humanos al menos cuatro cuadros clínicos diferentes por bloqueo de la transmisión neuromuscular, y que pueden variar en gravedad desde la casi ausencia de síntomas hasta la muerte por parálisis respiratoria. Paradójicamente, la toxina botulínica se presenta también como un arma terapéutica eficaz y segura en decenas de enfermedades, si bien gran parte de estas potenciales aplicaciones está aún en fase de investigación. (AU)

Evento aparentemente amenazador para la vida / Apparently threatening event for the life

Esta determinación se utiliza para describir aquellos episodios en los que un niño menor de un año,(en aparente estado de buena salud), sufre un cambio en su apariencia que es interpretado por los padres o adultos circundantes como un estado demuerte inminente. Definiremos al ALTE como aquel evento en que la madre o responsable del cuidado de un niño menor de 1 año refiere que éste presentó: 1- Un súbito cambio de color (palidáz, cianosis, rubicundez) 2- Hipotonía o menos frecuentemente hipertoníia 3- Alteraciones en la respiración 4- Luego de realizar alguna estimulación intensa (sacudida, respiración boca a boca) ligera o espontáneamente

[Type B botulism: a family outbreak]. / Botulisme de type B: à propos d'une épidémie intrafamiliale.

CASE REPORTS: Three cases of an outbreak of familial foodborne botulism are reported. The food incriminated could not be identified despite a careful investigation into the food history of the patients. The outcome was good following endotracheal ventilation and botulism antitoxin trivalent therapy. CONCLUSION: In France, foodborne botulism is an uncommon public health disease, and with a good prognosis when the diagnosis is promptly performed. The value of emergency electromyographic findings is emphasized, particularly when the repetitive stimulation of the motor nerve shows a presynaptic block of neuromuscular transmission. Management depends on the symptomatology, and trivalent antitoxin therapy is the only specific treatment.

Clinical spectrum of botulism.

Botulism is a paralyzing disease caused by the toxin of Clostridium botulinum. The toxin produces skeletal muscle paralysis by producing a presynaptic blockade to the release of acetylcholine. Recent studies have pinpointed the site of action of the several types of botulinum neurotoxin at the nerve terminal. Since the discovery of the toxin about 100 years ago, five clinical forms of botulism have been described: 1) classic or foodborne botulism; 2) wound botulism; 3) infant botulism; 4) hidden botulism; 5) inadvertent botulism. A clinical pattern of descending weakness is characteristic of all five forms. Almost all human cases of botulism are caused by one of three serotypes (A, B, or E). Classic and wound botulism were the only two forms known until the last quarter of this century. Wound botulism was rare until the past decade. Now there are increasing numbers of cases of wound botulism in injecting drug users. Infant botulism, first described in 1976, is now the most frequently reported form. In infant botulism spores of Clostridium botulinum are ingested and germinate in the intestinal tract. Hidden botulism, the adult variant of infant botulism, occurs in adult patients who usually have an abnormality of the intestinal tract that allows colonization by Clostridium botulinum. Inadvertent botulism is the most recent form to be described. It occurs in patients who have been treated with injections of botulinum toxin for dystonic and other movement disorders. Laboratory proof of botulism is established with the detection of toxin in the patient's serum, stool, or wound. The detection of Clostridium botulinum bacteria in the stool or wound should also be considered evidence of clinical botulism. Electrophysiologic studies can provide presumptive of botulism in patients with the clinical signs of botulism. Electrophysiologic testing can be especially helpful when bioassay studies are negative. The most consistent electrophysiologic abnormality is a small evoked muscle action potential in response to a single supramaximal nerve stimulus in a clinically affected muscle. Posttetanic facilitation can be found in some affected muscles. Single-fiber EMG studies typically reveal increased jitter and blocking, which become less marked following activation. The major treatment for severe botulism is advance medical and nursing supportive care with special attention to respiratory status.

Botulinum neurotoxins: mechanism of action and therapeutic applications.

Recent studies have led to the discovery of the molecular lesions in motor neurons caused by botulinum neurotoxins. These neurotoxins are metalloproteinases that enter the cytosol and very specifically cleave protein components of the neuroexocytosis apparatus. Consequently, acetylcholine cannot be released and the muscle is paralysed. For this reason, botulinum neurotoxins are increasingly being used to treat a variety of conditions where a functional paralysis of neuromuscular junctions is useful as therapy.

Investigation of animal botulism outbreaks by PCR and standard methods.

A double PCR procedure is proposed for identification of Clostridium botulinum C and D. This method consists of a first PCR amplification with a degenerate primer pair able to amplify a 340 bp common DNA fragment from botulinum neurotoxin (BoNT) C1 and D genes, followed by two subsequent PCR amplifications with two primer pairs specific for BoNT/C1 and D respectively (198 bp DNA fragment). This method was found to be specific for C. botulinum C and D, amongst 81 strains of C. botulinum and 21 different species of other Clostridium and bacteria tested. The detection limit ranged from 10 to 10(3) bacteria in the reaction volume according to the C. botulinum C and D strains. In 160 naturally contaminated animal and food samples submitted to a 48 h enrichment culture, the double PCR showed an 89.4% correlation rate with the standard mouse bioassay. A clear distinction between botulism type C and D was obtained. The double PCR provides a reliable alternative for detection and identification of C. botulinum C and D in clinical and food samples.

Type F botulism due to neurotoxigenic Clostridium baratii from an unknown source in an adult.

Type F botulism was confirmed in a 54-year-old male with signs compatible with botulism who reported to the emergency unit of a hospital. Botulinal neurotoxin was detected in the patient's serum and fecal specimens, and a neurotoxigenic organism whose physiologic characteristics correspond to those of Clostridium baratii was isolated. The toxin produced by the isolate was neutralized by type F botulinal antitoxin and cross-neutralized with lower efficiency by type E antitoxin. The patient's food history was not suggestive of botulism, and it seems likely that the illness was due to colonization of the gut.