Sudden death of twins: botulism because of contamination by pap vegetables

Botulism is caused by the blockage of the neural transmission in the cholinergic synapses by botulinum neurotoxin (BoNT) which is produced by Clostridium botulinum or other Clostridia. The classic form of botulism occurs after the ingestion of food contaminated by BoNT. The course of the infection can be asymptomatic, mild with subtle paralysis ("failure to thrive") oder severe with generalized paralysis ("floppy infant"). Infected infants can also die sudden and unexpectedly. These deaths often are attributed to Sudden Infant Death Syndrome (SIDS), unless a thorough postmortem examination reveals Botulism. The rate of fatal Botulism falsely attributed to SIDS is not known, because it is difficult in most cases to show the causal relationship between contamination, disease and death. We report the sudden and unexpected simultaneous death of twins of 22 months which could be attributed to Botulism. Contamination of food, colonization of the gut by Clostridia and infection with specific pathomorphological changes could be proven. The initial suspicion of infanticide could be excluded. lt could be shown, that Botulism is a potential cause of simultaneous unexpected deaths in twins.     

Is there a link between infant botulism and sudden infant death? Bacteriological results obtained in Central Germany

Despite the fact that botulism was described in Germany for the first time by Kerner in 1820, the disease is almost forgotten in this country. Only about 10-20 cases of classical botulism (intoxication) are recorded every year, including 1-2 cases of clinical infant botulism. As we assumed a high incidence of botulism to be connected with cases of sudden infant death (SID), we undertook the research work presented here. From every case of unexpected infant death up to 12 months of age, standardised specimens (blood, liver and intestine) were taken at autopsy. They were tested for the presence of botulinum neurotoxin (BoNT) and/or bacterial forms of Clostridium botulinum with subsequent BoNT neutralisation tests by the international standard mouse bioassay. Age, sex, pathological findings and season were recorded. Over a 5-year period, 75 samples including 57 SID cases were tested. Free toxin was found in nine and bacterial forms were detected in six samples. Toxin neutralisation revealed the definite presence of BoNT/BoNT producing bacteria (mainly type E), whereas another 11 toxin tests were inconclusive. According to international literature, these 15 cases are to be interpreted as infant botulism. Conclusion: the results show a remarkable incidence of infant botulism without any known previous medical history, partly hidden as sudden infant death. We propose to systematically search for botulism in connection with sudden infant death.     

Electrodiagnosis reliability in the diagnosis of infant botulism.

Infant botulism is confirmed by isolation of Clostridium botulinum from stool culture or by toxin assay. Although electrodiagnosis has been described as a diagnostic tool in infant botulism, our 11-year review of toxin-confirmed cases suggests that electrodiagnosis is not a reliable tool. In the case report presented, results of electrodiagnosis were negative but enema effluent contained adequate concentrations of organism and toxin to confirm the diagnosis.     

Honey and other environmental risk factors for infant botulism.

Infant botulism results from the in vivo production of toxin by Clostridium botulinum after it has colonized the infant's gut. Epidemiologic and laboratory investigations of this recently recognized disease were undertaken to identify risk factors and routes by which C. botulinum spores might reach susceptible infants. Clostridium botulinum organisms, but no preformed toxin, were identified in six different honey specimens fed to three California patients with infant botulism, as well as from 10% (9/90) of honey specimens studied. By food exposure history, honey was significantly associated with type B infant botulism (P = 0.005). In California, 29.2% (12/41) of hospitalized patients had been fed honey prior to onset of constipation; worldwide, honey exposure occurred in 34.7% (28/75) of hospitalized cases. Of all food items tested, only honey contained C. botulinum organisms. On household vacuum cleaner dust specimens and five soil specimens (three from case homes, two from control homes) contained Clostridium botulinum. The known ubiquitous distribution of C. botulinum implies that exposure to its spores is universal and that host factors contribute importantly to the pathogenesis of infant botulism. However, honey is now an identified and avoidable source of C. botulinum spores, and it therefore should not be fed to infants.     

Two cases of type A infant botulism in Grenoble, France: no honey for infants

We report two severe cases of infant botulism diagnosed at Grenoble University Hospital, France, respectively in 2006 and 2009. Both cases were characterized by a delay in diagnosis, severe neurological manifestations and extended period of hospitalization in intensive care unit, but a complete recovery. Infant botulism is a rare but life-threatening disease. It primarily affects infants, and the main risk factor is honey ingestion. Diagnosis should be systematically evoked by pediatricians in infants suffering from constipation, fatigue, muscle weakness, difficult feeding and altered cry, but before the onset of generalized flaccid paralysis, so as to administer specific treatment (BabyBIG?, a human derived botulinum antitoxin) at an early stage of the disease when it is most effective. In conclusion, parents should be aware of the role of honey as a source of spores of Clostridium botulinum and therefore infant botulism in the first year of life.     

Infant botulism.

Infant botulism is a unique neuromuscular disease affecting infants less than six months old. It is the result of intraintestinal toxin production by C. botulinum (toxi-infection). Characteristic symptoms include constipation, lethargy, and decreased feeding. Physical examination often reveals generalized hypotonia with cranial nerve impairment. Recovery is dependent on supportive care in an intensive care setting. The relationship of this disease to the sudden infant death syndrome requires further study.     

The first case of type B infant botulism in Japan

A six-month-old girl with a 5 consecutive day history of constipation and poor feeding developed generalized weakness, poor head control, difficulties in sucking and swallowing, and cranial nerve dysfunction within a few days. These characteristic manifestations and clinical course prompted examination of the possibility of infant botulism, although no history of eating honey was obtained. Mouse bioassay performed with enema effluent demonstrated type B botulinum toxin. Culture of the effluent was positive for Clostridium botulinum type B. This is the first case of type B infant botulism in Japan.     

Clostridium botulinum and sudden infant death syndrome: A 10 year prospective study

It has been proposed that sudden and unexpected death in infants due to intestinal infection with Clostridium botulinum may mimic the clinicopathological features of sudden infant death syndrome. Between 3.3 and 3.8% of infants in some series have had this neurotoxin-producing bacterium isolated on faecal culture. Prospective screening of 248 infants presenting with the sudden infant death syndrome to the Adelaide Children's Hospital over a 10 year period from 1981 to 1990 was conducted. Faecal samples were obtained from both small and large intestines and cultured specifically for C. botulinum. No samples were positive. The results of this study suggest that routine post-mortem culture of faeces for C. botulinum has been of limited use within the South Australian infant population over the last decade, and that occult botulism has not been a significant factor in the causation of sudden death.     

Infant botulism in Australia — a case report

ABSTRACT. The syndrome of infant botulism was first recognised in late 1975 and the majority of cases reported have been from the United States of America. One case has been reported from the United Kingdom and one from Canada.
A three-month-old male infant from Victoria, Australia presented with constipation, marked hypotonia, limb weakness, ptosis, facial weakness and inability to suck and swallow. These abnormalities resolved and he returned to normal over the ensuing months.
A diagnosis of infant botulism was confirmed after the isolation of Clostridium botulinum type B from the faeces.
Infant botulism has now been recognised in four different countries and it is likely than with increasing awareness of this striking clinical syndrome, more cases will be identified.     

Differential effect of botulinal toxin on esophageal motor function in infants

The effect of Clostridium botulinum toxin on esophageal motor function was studied in four infants (ages 5-9 months) with confirmed infant botulism. Esophageal motility studies using a perfused catheter assembly were performed during the acute phase in all patients, and during the recovery phase in one patient. Motor function of the proximal esophagus and upper esophageal sphincter was abnormal in each, while motor function of the distal esophagus and lower esophageal sphincter was normal. Mean lower esophageal sphincter pressure was 24 mm Hg for the group (normal: 15-30 mm Hg). Sequential studies of proximal esophageal motility in one infant revealed a return of normal motor function which correlated with recovery of peripheral muscle strength and gag reflexes. C. botulinum toxin impairs proximal, but not distal, esophageal motor function in the infant botulism model. This effect appears to be consistent with the known action of the toxin on synaptic acetylcholine release and current concepts regarding distribution of cholinergic and noncholinergic neurotransmitter receptors in the esophagus.     

Infantile botulism: clinical and laboratory observations of a rare neuroparalytic disease

A 3-month-old male infant was admitted to the University Hospital of Los Andes with a history of constipation, weak crying, poor feeding, flaccidity and later bilateral ptosis and hyporeflexia. The admission diagnosis was septicaemia until an electrophysiological study reported postetanic facilitation with 50 Hz/seg stimulations four days later. The Clostridium botulinum toxin type B was isolated from the infant's stool samples and the organism grew in anaerobic cultures. The patient recovered completely and was discharged 2 months later. Although infant botulism is an uncommon disease in our environment, this diagnosis must be suspected in all afebrile infants with constipation, affected cranial nerves and generalized hypotonia. The principal differential diagnoses are Landry-Guillain-Barré syndrome, poliomyelitis, myasthenia gravis and infant muscular atrophy.     

Botulism in infancy

The authors describe a case of botulism in a 3-month-old infant infected with Clostridium botulinum type A. Symptomatology developed within four days, persisted for two weeks, then regressed. Symptoms were paresis of face muscles, hyporeactive pupils, loss of succion and deglutition, axial hypotonia, weakness of peripheral muscles, lability of the autonomic nervous system with acute episodes of bradycardia and constipation. Anomalies of the electroen-cephalogram and of the auditory evoked responses suggest that the toxin penetrated the central nervous system. Treatment was symptomatic, without need for assisted ventilation. It was not possible to detect the source of infection.     

Application of nested polymerase chain reaction for the rapid diagnosis of infant botulism type B

A nested polymerase chain reaction was utilized to successfully detect the type B botulinum neurotoxin gene of Clostridium botulinum in feces from a 6-month-old patient, who had already been diagnosed with type B infant botulism by mouse bio-assay. This method of rapid diagnosis without enrichment culture of feces can be applied to other types of toxins in the use of the type-specific primers. Further investigations, however, are required to define the sensitivity and specificity of the method.     

A case of botulism in a 11-month-old infant

We report a case of botulism in a 11 month-old infant. Hypotonia complicated by progressive bulbar paralysis revealed the disease. Botulism B toxin was present in serum on the 8th day of the disease. On the occasion of this case report the clinical, diagnostic, epidemiological and pathophysiologic aspects of infant botulism are reviewed. The relationship between sudden infant death and botulism is discussed.     

Clinical mimics of infant botulism

Since 1992, Human Botulism Immune Globulin has been provided by the California Department of Health Services to infants with probable infant botulism, the intestinal toxemia form of human botulism. Human Botulism Immune Globulin became available in California in 1992-1997 within a randomized, controlled, double-blinded, pivotal clinical trial and subsequently became available nationwide in 1998-2003 in an open-label study until its licensure in October 2003 as BabyBIG. Thereafter, Human Botulism Immune Globulin remained available nationwide as an approved orphan-drug product. To achieve prompt neutralization of circulating botulinum toxin, the decision to treat with Human Botulism Immune Globulin has been based on clinical criteria that include a consistent history and physical findings of bulbar palsies, hypotonia, and weakness. After licensure, the charts of patients who did not have laboratory-confirmed infant botulism were reviewed to identify their actual diagnoses. The approximately 5% of 681 patients treated with Human Botulism Immune Globulin who did not have infant botulism fell into 5 categories: spinal muscular atrophy, metabolic disorders, other infectious diseases, miscellaneous, and probable infant botulism lacking laboratory confirmation.     

Sudden infant death syndrome and infant mortality in Aboriginal and non-Aboriginal infants

The aim of this study was to investigate sudden infant death syndrome (SIDS) in the context of total infant mortality for Aboriginal and non-Aboriginal infants. Deaths for infants born in Western Australia from 1980 to 1988 inclusive were ascertained from a total population data base. Infant mortality rates and rates by period and cause of death were calculated for both populations. Aboriginal infants had a mortality rate three times that for non-Aboriginal infants (23.6 cf. 7.9 per 1000 live births) and both populations showed a similar rate of decline in mortality over the study period. There were differences in the proportion of deaths occurring neonatally and postneonatally in the two populations. In terms of SIDS, 21% of the deaths in Aboriginal infants occurred neonatally compared with 7% for non-Aboriginal infants. The overall cause of infant death distribution differed significantly between the two populations ( P < 0.001). During the study period, Aboriginal infants showed a significant increase in deaths due to SIDS and a significant decrease in those due to birth defects and low birthweight. These results suggest it would be useful to review the pathology and diagnosis of sudden unexplained death in infancy.     

Infant Botulism with Down Syndrome

A 4-month-old boy with Down syndrome and infant botulism is reported. He was admitted to our hospital with stridor and developed respiratory failure and generalized paralysis. Laboratory examination revealed botulinal toxin type A in his blood and feces. Clostridium botulinum organisms were found in the feces and in the honey which he had been fed. Anti-botulinal toxin was administered on the 10th day of illness. Artifical ventilation was given for 87 days and he survived. This is the 11th known case of infant botulism in Japan. It is possible that other cases remain undiagnosed.     

Clinical, laboratory, and environmental features of infant botulism in Southeastern Pennsylvania

Forty-four cases of botulism occurred in infants in Southeastern Pennsylvania between 1976 and 1983. Forty-three were caused by Clostridium botulinum type B. Progressive weakness necessitated ventilatory support in 39 infants. Complications during hospitalization included otitis media in 13 patients and aspiration pneumonia in 11. Eight infants developed the syndrome of inappropriate secretion of antidiuretic hormone and two developed adult respiratory distress syndrome. One infant died of progressive bradycardia despite adequate control of ventilation. Manifestations of autonomic nervous system dysfunction recognized on admission to the hospital were constipation, distention of the urinary bladder, and decreased salivation and tearing. During hospitalization, some infants had unexpected fluctuations of skin color, blood pressure, and heart rate. Infants' strength improved despite persistent intestinal elaboration of toxin. C botulinum was isolated from seven of nine home or work environments sampled. All 44 infants were white and were receiving breast milk at the time of onset of symptoms. The majority had first feedings of nonhuman food substances within 4 weeks prior to onset of symptoms. Delineation of fecal flora in seven infants revealed predominance of enterobacteriaceae. Perturbations of intestinal flora during infancy, especially at weaning, may cause transient permissiveness to colonization by C botulinum.     

SIDS and other sleep-related infant deaths: expansion of recommendations for a safe infant sleeping environment

Despite a major decrease in the incidence of sudden infant death syndrome (SIDS) since the American Academy of Pediatrics (AAP) released its recommendation in 1992 that infants be placed for sleep in a nonprone position, this decline has plateaued in recent years. Concurrently, other causes of sudden unexpected infant death occurring during sleep (sleep-related deaths), including suffocation, asphyxia, and entrapment, and ill-defined or unspecified causes of death have increased in incidence, particularly since the AAP published its last statement on SIDS in 2005. It has become increasingly important to address these other causes of sleep-related infant death. Many of the modifiable and nonmodifiable risk factors for SIDS and suffocation are strikingly similar. The AAP, therefore, is expanding its recommendations from being only SIDS-focused to focusing on a safe sleep environment that can reduce the risk of all sleep-related infant deaths including SIDS. The recommendations described in this report include supine positioning, use of a firm sleep surface, breastfeeding, room-sharing without bed-sharing, routine immunization, consideration of a pacifier, and avoidance of soft bedding, overheating, and exposure to tobacco smoke, alcohol, and illicit drugs. The rationale for these recommendations is discussed in detail in this technical report. The recommendations are published in the accompanying "Policy Statement--Sudden Infant Death Syndrome and Other Sleep-Related Infant Deaths: Expansion of Recommendations for a Safe Infant Sleeping Environment," which is included in this issue (www.pediatrics.org/cgi/doi/10.1542/peds.2011-2220).     

Etiologia i epidemiologia botulizmu niemowląt

Foodborne botulism is the most common illness caused by anaerobic, spore-forming bacilli – C. botulinum. Besides classic form of botulism there are less known manifestations of the disease such as wound and infant botulism. Infant botulism is a toxicoinfection occurring in children under one year, usually after ingestion of C. botulinum spores. The prevalence of this disease is extremely rare. However, in recent years there has been an increasing participation of infant botulism in total number of botulism cases with more than half of them being a consequence of honey consumption contaminated with C. botulinum spores. The awareness of the symptoms and hazardous agents of infant botulism is extremely important for the proper diagnosis and successful treatment. There is an increasing evidence that the infant botulism is underestimated and not properly diagnosed thus it may be considered as a sudden infant death syndrome. The European Commission together with World Health Organization and Centres for Disease Control and Prevention gave an opinion on microbiological safety of honey, where they recommend that children under one year of age should not be fed with honey.