Infantile botulism is a form of botulism that affects children less than a year old. Risk factors include ingestion of honey (or other products) and exposure to construction sites in which Clostridium botulinum spores may be found. The clinical presentation is significant for descending paralysis, acute hypotonia, lethargy, ptosis, and other neurologic manifestations. The diagnosis is clinical and relies upon the history, risk factors, and physical exam. Detection of the spore or toxins in the stool is one method for confirmation.
Presentation
Infant botulism is one of 4 types of botulism along with foodborne, wound and intestinal [1]. Although rare, infant botulism is the most predominant type in the United States, where it has been traditionally associated with the ingestion of honey [1] [2] [3]. However, the etiology and source of spores remain unknown in the majority of American cases [4]. Other sources include corn syrup, grape molasses [5], infant cereal formulas, infant powdered milk, and medicinal plants [6]. Spores may also originate from soil and dust found in construction areas [7].
Infantile botulism occurs in children less than a year old because this age group has low gastric acid production, a smaller pool of flora, and an immature immune system, all of which render them vulnerable to toxin production following the ingestion of Clostridium botulinum spores [8]. The botulinum toxin inhibits the release of acetylcholine at the neuromuscular junction [1], which is the mechanism of action responsible for the signs and symptoms observed.
The clinical manifestations of infant botulism include descending paralysis, constipation (which may precede other symptoms), hypotonia, lethargy, weak sucking, weak crying, irritability, and weakness of the bilateral extremities and face [1] [7] [9]. Note that a clinical presentation of acute hypotonia should raise suspicion for this condition [7]. Note that, in many cases, descending paralysis may be absent.
Complications
Patients may present with hypoventilation that ultimately progresses to respiratory failure requiring endotracheal intubation [1] [7]. Specifically, 60% of the infants eventually require mechanical ventilation as the disease advances [2]. Additionally, impaired gag reflex can cause aspiration.
Physical exam
Infants with infant botulism appear floppy [10]. On the neurologic exam, remarkable findings include hypotonia, reduced deep tendon reflexes, ptosis, sluggish pupils, reduced gag reflex, and significant head lag [1] [7] [9] [11]. Patients may also exhibit signs of respiratory distress. They typically do not have a fever.
Entire Body System
- Sepsis
Early severe infantile botulism may be rare but should be considered in neonates who have hypotonia and respiratory arrest or a sepsis-like clinical picture. [ncbi.nlm.nih.gov]
Congenital heart disease Hematologic – bleeding secondary to sepsis, Vitamin K deficiency, etc. Metabolic abnormalities – endocrinopathies, nephropathies, inborn error of metabolism, etc. [pediatriceducation.org]
He is hospitalized for possible sepsis. A sepsis work up is done and he is started on IV fluids and empiric antibiotics of ampicillin and gentamicin for sepsis. [hawaii.edu]
View/Print Table TABLE 2 Differential Diagnosis of Hypotonia in Infants Infectious Sepsis Meningitis Encephalitis Metabolic Electrolyte abnormalities (hyponatremia) Reye's syndrome Hepatic encephalopathy Hypothyroidism Organic acidurias Subacute necrotizing [aafp.org]
CT, MRI and EEG (electroencephalogram) scans were all normal, and the patient was broadly treated for sepsis, meningitis and encephalitis while investigations continued. [adc.bmj.com]
- Feeding Difficulties
Symptoms of infant botulism include: Constipation Weakness Feeding difficulties Dropping Anorexia Irritability Weak cry If you would like to contribute to Cole's recovery click here. Copyright 2017 Scripps Media, Inc. All rights reserved. [abc15.com]
In Argentina and some southern Mediterranean countries infant botulism has been associated with feeding infants herbal infusions or teas. [gov.uk]
- Acutely Ill Patient
Samples to be taken from acutely ill patients include: serum: At least 10ml. Serum samples must be collected before antitoxin is administered. wound. Pus: As much as possible in a sterile container. [gov.uk]
Respiratoric
- Aspiration
Additionally, impaired gag reflex can cause aspiration. Physical exam Infants with infant botulism appear floppy. [symptoma.com]
Observation for and management of complications such as aspiration pneumonia, urinary tract infection, acute otitis media and other complications of long term hospitalization. 2. [clinicaladvisor.com]
If infant botulism is not treated, death can result.7,8 Observed complications of infant botulism include adult respiratory distress syndrome; anemia; aspiration; bacteremia; C. difficile colitis, including toxic megacolon; fractures; osteopenia; funguria [the-medical-dictionary.com]
Gastrointestinal
- Constipation
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. [ncbi.nlm.nih.gov]
Method 1 Catching Signs and Symptoms Early 1 Take note of unexplained constipation. This is first sign of your baby being infected with botulism. If your infant has gone more than 3 days without defecating, it indicates your baby is constipated. [wikihow.com]
In view of constipation, patients may need a saline washout to get a stool sample. Specific assays can be used to test for the toxin. [dontforgetthebubbles.com]
- Dysphagia
The diagnosis should be considered in a child admitted to the intensive care unit in respiratory distress, and presenting with nausea, vomiting, and fever that precede the onset of ptosis, nonreactive pupils, dysphagia, dysphonia, weakness of facial and [pediatricneurologybriefs.com]
A characteristic pattern of dysarthria, dysphagia, dry mouth, diplopia, and blurred vision with ptosis, evolves during the onset of disease. [hawaii.edu]
Urinary retention, constipation and dysphagia disappeared by day 40, whereas the mydriasis and muscle weakness lasted until day 90. He successfully underwent bone marrow transplantation on day 49. [ann-clinmicrob.biomedcentral.com]
This can lead to regurgitation as well as dysphagia. Other early symptoms: dysarthria, a lack of sweating, severe dry mouth and throat, and finally postural hypotension. Food-borne botulism may initially present with nausea and vomiting. [physio-pedia.com]
The additional bulbar palsies of ophthalmoplegia, weak cry, weak sucking, and dysphagia (drooling) are not apparent in the photograph. [nejm.org]
Cardiovascular
- Hypertension
Her maternal grandfather is deceased from lung cancer, and her paternal grandmother and grandfather have hypercholesterolemia and hypertension. [the-medical-dictionary.com]
Neurologic
- Flaccid Paralysis
MRI findings in children with acute flaccid paralysis and cranial nerve dysfunction occurring during the 2014 enterovirus D68 outbreak. [frontiersin.org]
It is characterized by symmetric cranial nerve palsy, commonly followed by symmetric, descending, flaccid paralysis of involuntary muscles, which may result in respiratory compromise and death. ( 1 ) The sensorium remains intact because the botulism toxin [pedsinreview.aappublications.org]
Botulinum neurotoxin is absorbed through the intestine into the circulation and binds at its target site, the neuromuscular junction, blocking release of the neurotransmitter acetylcholine leading to flaccid paralysis. [wales.nhs.uk]
The diagnosis may not be considered initially because signs of an evolving bulbar palsy, flaccid paralysis, and hypotonia may be subtle. [hawaii.edu]
- Descending Paralysis
Note that, in many cases, descending paralysis may be absent. Complications Patients may present with hypoventilation that ultimately progresses to respiratory failure requiring endotracheal intubation. [symptoma.com]
paralysis (3) bilateral symptoms (4) no fever (5) clear sensorium (6) lack of sensory findings Food-borne toxin absorbed from small intestine home-canned fruit and vegetable ingestion symptoms within 12-36 hrs GI distress -> neuro symptoms parasympathetic [lifeinthefastlane.com]
The classic syndrome of botulism is a symmetrical, descending motor paralysis in an alert patient, with no sensory deficits. [emedicine.medscape.com]
This organism produces a heat-labile neurotoxin blocking pre-synaptic cholinergic transmission leading to descending paralysis and potential respiratory collapse. [clinicaladvisor.com]
- Mydriasis
Symptoms and Signs See Botulism Early symptoms and Signs Constipation (65%) May precede weakness by weeks Cranial Nerve Dysfunction Weak cry and weak sucking Decreased oral intake (79%) Decreased Gag Reflex Cranial Nerve 6 palsy (unable to abduct eye) Mydriasis [fpnotebook.com]
Case presentation We report a 5-year-old boy, who developed general muscle weakness, constipation, ptosis and mydriasis during the third induction therapy for relapsed acute myeloid leukemia. [ann-clinmicrob.biomedcentral.com]
Loss of ocular motility ptosis, mydriasis and facial weakness also may occur. 14, 15 The weakness progresses in a descending fashion in a matter of days. Affected infants become irritable and lethargic. [aafp.org]
- Cranial Nerve Involvement
Signs of weakness in the infant with botulism begin with cranial nerve involvement and loss of head control. The infant may develop a weak cry, poor sucking ability, impaired gag reflex, pooling of secretions and decreased oral intake. [aafp.org]
- Paresis
Abstract Infantile botulism is a recently recognized cause of acute hypotonic paresis and respiratory failure in young infants. [ncbi.nlm.nih.gov]
Workup
The diagnosis of infant botulism is largely based on the patient's clinical presentation, medical history, risk factors, and physical exam. A crucial component of the workup includes a history of all patient intake with regards to honey, baby formulas and powdered milk, plants, and other sources of spore exposure. Additionally, the clinician should inquire whether family members were recently ill [1]. Note that this diagnosis should still be considered in patients who lack exposure to honey or construction locations [1]. Prompt treatment is paramount for these patients and this relies on recognition of the signs and a high index of suspicion [12].
Diagnostic tests
Confirmation is best made through the detection of botulinum toxin or spores in the stool whereas serum testing for toxins is not as sensitive [1]. Also, isolation of the implicated pathogen in the stool is another method.
Laboratory tests
To help rule out differential diagnoses and attain a complete clinical picture, laboratory tests such as a complete blood count (CBC), complete metabolic panel (CMP) which includes electrolyte levels, and spinal fluid analysis should be obtained [5].
Electromyogram (EMG)
If abnormal, findings will feature a pattern of short-lasting, small-amplitude motor-unit action potentials in abundance [13]. This is not pathognomonic for infant botulism as it occurs in other neurological diseases as well [1] [11].
Microbiology
- Clostridium Botulinum in Stool
The confirmatory testing for infant botulism relies on detection of Clostridium botulinum in stool samples, either through culture and isolation of the organism and spores or by detection of toxin. [clinicaladvisor.com]
Treatment
Specific treatment of infant botulism is with human botulism immune globulin (BabyBIG®), which is available from the Infant Botulism Treatment and Prevention Program (IBTPP—call 510-231-7600 or see the IBTPP web site). [msdmanuals.com]
Get clear, concise descriptions and evidence-based treatment guidelines for a full range of clinical conditions, ranging from the common to the unusual. [books.google.ro]
Human botulism immune globulin for the treatment of infant botulism. N Engl J Med 2006;354(5):462-71 [ Abstract ] See also: California Department of Health Services' Infant Botulism Treatment and Prevention Program http://infantbotulism.org/ [cidrap.umn.edu]
However, treatment with BIG-IV did not always result in a shortened hospital stay. [nejm.org]
Prognosis
In most cases, the prognosis is excellent with institution of appropriate supportive care. The recognition of cranial nerve palsies or a history of constipation should raise the suspicion of infantile botulism. [ncbi.nlm.nih.gov]
What’s the prognosis? The prognosis is excellent. In the study mentioned above, all patients (both groups) survived to discharge and none had any serious sequelae. Complications are essentially secondary to being in hospital. [dontforgetthebubbles.com]
Prognosis Case fatality rate of treated patients: Excellent long-term prognosis without residual changes X. Course Mechanical Ventilation : 23 days Hospital stay on average: 44 days Relapses, if they occur, usually do so within 13 days XI. [fpnotebook.com]
What Is the Prognosis? Most children recover fully from botulism, although it can take several weeks to months. [healthychildren.org]
If you noticed any of the previously mentioned symptoms, even 1 or 2, don’t hesitate to seek medical care, as this medical condition can easily be treated and has a good prognosis if immediate medical intervention is received. [wikihow.com]
Etiology
However, the etiology and source of spores remain unknown in the majority of American cases. Other sources include corn syrup, grape molasses, infant cereal formulas, infant powdered milk, and medicinal plants. [symptoma.com]
Discussion Given the history and exam consistent with infant botulism along with CSF reassuring against infectious or autoimmune etiologies for the infant’s weakness, the infant was treated empirically with BabyBIG on hospital day 2 despite the abnormal [frontiersin.org]
Etiology/Causes Clostridium botulinum [1] A gram-positive bacteria that produces spores anaerobically. This bacteria is found globally in soil and aquatic sediments. [8] Produces 7 immunologically distinct toxins designated by letters A-G. [physio-pedia.com]
Epidemiology
Descriptive Epidemiology 587 Mechanisms and Routes of Transmission 7 Pathogenesis and Immunity 590 Patterns of Host Response 591 Control and Prevention 592 References 593 Suggested Reading 595 Rocky Mountain Spotted Fever Theodore E Woodward and J Stephen [books.google.com]
Botulism in the Very Young Neonate: A Case Series. ( 28765793 ) Jackson L....Riley M. 2017 6 Type F Infant Botulism: Investigation of Recent Clusters and Overview of This Exceedingly Rare Disease. ( 29293930 ) Halpin A.L....Rao A.K. 2017 7 Molecular and Epidemiological [malacards.org]
National Surveillance Team – Enteric Diseases Epidemiology Branch. Centers for Disease Control and Prevention. http://www.cdc.gov/nationalsurveillance/botulism-surveillance.html Updated May 12, 2017. Accessed January 25, 2018. [rarediseases.org]
Thompson JA, Glasgow LA, Warpinski JR, Olson C (1980) Infant botulism: clinical spectrum and epidemiology. Pediatrics 66: 936–942 PubMed Google Scholar 7. [link.springer.com]
Epidemiology Incidence : Estimated at 250 cases in U.S. per year More cases than foodborne or Wound Botulism States with highest rates California (50%) Utah Pennsylvania Age of Onset Age 6 weeks to 9 months Peaks at 2-3 months (90% are under 6 months [fpnotebook.com]
Pathophysiology
Pathophysiology Sources Contaminated soil (e.g. construction site, farm) Contaminated honey (10% of samples) Contaminated corn syrup (0.5% of samples) Other related factors Infants under 2 months living in rural farming area Infants over 2 months are [fpnotebook.com]
Physician awareness of infant botulism is paramount to early recognition and intervention, because more than 70 percent of these infants will eventually require mechanical ventilation. 2 Pathophysiology Infant botulism occurs when ingested spores germinate [aafp.org]
Pathophysiology Infant botulism differs from adult forms of the disease in that active colonization of the gastrointestinal tract by viable organisms leads to disease, rather than ingestion or exposure to pre-formed toxins. [clinicaladvisor.com]
Pathophysiology C botulinum is a gram-positive, spore-forming anaerobe that naturally inhabits soil, dust, and fresh and cooked agricultural products. [emedicine.medscape.com]
Prevention
Descriptive Epidemiology 587 Mechanisms and Routes of Transmission 7 Pathogenesis and Immunity 590 Patterns of Host Response 591 Control and Prevention 592 References 593 Suggested Reading 595 Rocky Mountain Spotted Fever Theodore E Woodward and J Stephen [books.google.com]
Breast feeding, though, is a great way to prevent or decrease allergy symptoms. [drgreene.com]
Infant Botulism Treatment and Prevention Program: Web site or call 510-231-7600: Provides information about treatment, prevention, and support groups [msdmanuals.com]
Healthy adults and children over one year of age have a more mature digestive system that prevents the Clostridium bacteria from surviving. Foodborne botulism, while also rare, is different from infant botulism. [edis.ifas.ufl.edu]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.ro]
References
- Jackson L, Madan-Khetarpal S, Naik M, Michaels MG, Riley M. Infant Botulism in the Very Young Neonate: A Case Series. AJP Reports. 2017;7(3):e163-e166.
- Schwartz KL, Austin JW, Science M. Constipation and poor feeding in an infant with botulism. CMAJ. 2012;184(17):1919-1922.
- Thompson JA, Glasgow LA, Warpinski JR, Olson C. Infant botulism: clinical spectrum and epidemiology. Pediatrics. 1980;66(6):936–942.
- Pifko E, Price A, Sterner S. Infant botulism and indications for administration of botulism immune globulin. Pediatr Emerg Care. 2014;30(2):120-4; quiz 125-127.
- Cagan E, Peker E, Dogan M, Caksen H. Infant Botulism. Eurasian J Med. 2010;42(2):92-94.
- Kobayashi T, Haginoya K, Morimoto T, Hatakeyama T, Tsuchiya S. A case of infant botulism infection due to consumption of untreated well-water. J Pediatr. 2014;164(4):931-933.
- DiPrisco BE, Chhabria S, Forem SL, Rosenberg RE. Ten-week-old girl with lethargy, weakness, and poor feeding. Clin Pediatr (Phila). 2013;52(2):190-193.
- Ramroop S, Williams B, Vora S, Moshal K. Infant botulism and botulism immune globulin in the UK: a case series of four infants. Arch Dis Child. 2012;7(5):459-460.
- Quinn KK, Cherry JD, Shah NR, Christie LJ. A 3-month-old boy with concomitant respiratory syncytial virus bronchiolitis and infant botulism. Pediatr Infect Dis J. 2013; 32(2):195.
- Abdulla CO, Ayubi A, Zulfiquer F, Santhanam G, Ahmed MA, Deeb J. Infant botulism following honey ingestion. BMJ Case Rep. 2012; 2012.
- Fox C K, Keet C A, Strober J B. Recent advances in infant botulism. Pediatr Neurol. 2005;32(03):149–154.
- Brown N, Desai S. Infantile botulism: a case report and review. J Emerg Med. 2013;45(6):842-845.
- Long SS. Infant botulism. Pediatr Infect Dis J. 2001;20(7):707–709.