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Infantile Botulism

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.

Weight Loss
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]
  • 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]
  • 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.[symptoma.com]
Flaccid Paralysis
  • It is a decending, symmetric, flaccid paralysis, with cranial nerves palsies being the most common complication.[pediatriceducation.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]
  • MRI findings in children with acute flaccid paralysis and cranial nerve dysfunction occurring during the 2014 enterovirus D68 outbreak.[frontiersin.org]
  • It affects ganglionic synapses, parasympathetic synapses and neuromuscular junctions, which explains the clinical picture of descending flaccid paralysis and anticholinergic symptoms.[casereports.bmj.com]
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]
Tonic-Clonic Seizure
  • On day 16 of admission he had abnormal movements in keeping with a generalised tonic-clonic seizure and was treated with midazolam and phenytoin. His electroencephalography was entirely normal.[casereports.bmj.com]

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].

Prolonged PR Interval
  • الصفحة 286 - Clinical findings Arthralgia Fever Laboratory findings Elevated acute phase reactants Erythrocyte sedimentation rate C-reactive protein Prolonged PR interval Supporting Evidence of Antecedent Group A Streptococcal Infection Positive throat[books.google.com]
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]
Pseudomonas
  • A diagnosis of adult-onset “infant” botulism was confirmed by identification of botulinum toxins A and E in stool and serum of a 32-year-old woman with cystic fibrosis treated for a pseudomonas pneumonia and having a percutaneous gastrostomy tube placed[pediatricneurologybriefs.com]

Treatment

  • Review a case of infantile botulism, its diagnosis, and treatment. A 4-month-old healthy Caucasian male presented to the ED with a 6-day history of decreased feeding after referral from the pediatrician.[ncbi.nlm.nih.gov]
  • Treatment with Baby BIG-IV has been available in the USA since 2003.[casereports.bmj.com]
  • Prompt treatment is paramount for these patients and this relies on recognition of the signs and a high index of suspicion.[symptoma.com]
  • She says it's important for areas like Mildura- where there appears to be a higher rate of infantile botulism, to be aware of the treatment options available.[abc.net.au]
  • Well, the toxin is certainly not cheap at around 50,000 for treatment of a 5kg baby.[dontforgetthebubbles.com]

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]
  • Given the deteriorating nature of his condition and negative investigations at that time, he was thought to have a neurodegenerative condition with a poor prognosis. End-of-life care was discussed with parents and arrangements made for baptism.[casereports.bmj.com]
  • What Is the Prognosis? Most children recover fully from botulism, although it can take several weeks to months.[healthychildren.org]

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. Updated May 12, 2017. Accessed January 25, 2018.[rarediseases.org]
  • Understand the epidemiology, risk factors, and management of botulism. Become familiar with the resources to obtain diagnostic aid and antitoxin, when indicated.[pedsinreview.aappublications.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]
Sex distribution
Age distribution

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 C botulinum is a gram-positive, spore-forming anaerobe that naturally inhabits soil, dust, and fresh and cooked agricultural products.[emedicine.medscape.com]
  • 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]

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]
  • Summary Conversation (1) Downloads CME Credits (3.5) Infantile botulism is something we don’t see too often, however because of its potentially devastating effects it is important for us to educate families for prevention and also remind ourselves how[hippoed.com]
  • Breast feeding, though, is a great way to prevent or decrease allergy symptoms.[drgreene.com]

References

Article

  1. 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.
  2. Schwartz KL, Austin JW, Science M. Constipation and poor feeding in an infant with botulism. CMAJ. 2012;184(17):1919-1922.
  3. Thompson JA, Glasgow LA, Warpinski JR, Olson C. Infant botulism: clinical spectrum and epidemiology. Pediatrics. 1980;66(6):936–942.
  4. 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.
  5. Cagan E, Peker E, Dogan M, Caksen H. Infant Botulism. Eurasian J Med. 2010;42(2):92-94.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. Abdulla CO, Ayubi A, Zulfiquer F, Santhanam G, Ahmed MA, Deeb J. Infant botulism following honey ingestion. BMJ Case Rep. 2012; 2012.
  11. Fox C K, Keet C A, Strober J B. Recent advances in infant botulism. Pediatr Neurol. 2005;32(03):149–154.
  12. Brown N, Desai S. Infantile botulism: a case report and review. J Emerg Med. 2013;45(6):842-845.
  13. Long SS. Infant botulism. Pediatr Infect Dis J. 2001;20(7):707–709.

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Last updated: 2019-07-11 21:24