INFANTILE BOTULISM
Infantile Botulism, as the name suggests, is an infantile disorder that is uncommon, life threatening, and neuromuscular disorder. It is, in fact, one of the five naturally acquired types of Botulism, other ones being – Food borne Botulism, Wound Botulism, Iatrogenic Botulism, and Intestinal Botulism. On the other hand, it can also be acquired through some un-natural ways (with malicious intent) in the form of Bioterrorism and Biologic war (Arnon et al., 2001) (Atlas, 2002). Infantile Botulism comprises 70% of all the cases of Botulism (Van Horn & Street, 2019).
As far as the diseases’ etiology is concerned, the causative agent is identified as Clostridium botulinum (or rarely, C. butyricum and C. baratii), which is an anaerobic bacillus, having spore forming ability, gram positive in nature. Strain ‘B’ of C. botulinum was found to be associated with Infantile Botulism after conducting a characterization study in the United Kingdom (Johnson et al., 2005).
Concerning the pathophysiology of the disease, it is seen to result from an unusual infectious condition which is called ‘intestinal toxemia.’ This infectious condition is attained after either swallowing the spores of Clostridium botulinum or through the neurotoxins produced by C. butyricum or C. baratii (Arnon, Schechter, Maslanka, Jewell, & Hatheway, 2006). The ingested spores then germinate (and become vegetative cells) and temporarily colonize the lumen of the large intestine of an infant (Wilcke Jr, Midura, & Arnon, 1980). After colonizing, the spores start producing botulinum neurotoxin. Importantly, C. botulinum has the ability to produce all seven known serotypes of the botulinum toxin (A to G), whereas C. baratii and C. butyricum are only capable of producing one serotype each – F and E, respectively (Simpson, 2004).
The toxin they produce is one of the most dangerous and poisonous toxins having a lethal toxin dosage as low as 1mcg/kg (Van Horn & Street, 2019). It is absorbed through the gut and transported by the blood to the neuromuscular junction. Serotypes B, D, F, and G of the toxin act on the vesicle associated membrane protein (VAMP) and thus exerts its effect on the neuromuscular junction by binding irreversibly (Simpson, 2004).
By acting on VAMP, in particular, botulinum toxin blocks the process of exocytosis primarily at all the peripheral cholinergic sites including – neuromuscular junctions, autonomic ganglia of the sympathetic and parasympathetic nervous systems, sites of postganglionic parasympathetic, and other unusual postganglionic sympathetic sites that are capable of releasing acetylcholine as the neurotransmitter. As a result of this blockade phenomenon, the characteristic flaccid paralysis and autonomic dysfunction in the form of anticholinergic symptoms are observed (Simpson, 2004).
As the toxin is known to act on the autonomic system and neuromuscular junction, it can be well anticipated that the clinical manifestations would be related to autonomic dysfunction.
Clinical features of the infantile Botulism lead to the name “Floppy baby syndrome” and are manifested in the form of feeding and sucking difficulties, drooling, acute and severe hypotonia, bulbar palsies (ptosis, abnormal eye movements, diplopia, dysphonia, dysarthria, etc.), decreased tone of the anal sphincter, constipation and the most dreadful one being respiratory insufficiency secondary to paralysis of the diaphragm (Bernardor et al., 2018). All of this starts from bulbar musculature and then finally develops into a progressive descending scheme of paralysis. On the other hand, atypical manifestations of the disease include severe hyponatremia, metabolic acidosis, coagulopathy pertaining to sepsis, and a full cardiopulmonary arrest (Mitchell & Tseng-Ong, 2005). Furthermore, infantile Botulism has also been found to be correlated to Sudden Infant Death Syndrome (SIDS) (Nevas et al., 2005).
Talking about the risk factors for the infantile Botulism it is seen that they are mostly present in – increased birth weights infants, infants born to the women of advanced maternal age, and the infants being breastfed (Van Horn & Street, 2019). The reason for the susceptibility of the infants is justified by the fact that infants have a sterile intestine and immature flora of the gut. Moreover, a notable highlight is infants particularly lack bile acids capable of possessing Clostridium-inhibiting properties (Smith et al., 2010).
On the other hand, the foods having the potential to cause the disease are popularly known to be – ingestion of honey either direct or as a pacifier and infant formula (King et al., 2010). Moreover, dust particles are also believed to be the causative factor for the disease (Bernardor et al., 2018). There is little awareness regarding the use of honey as a medicine and as a traditional remedy by the parents. The term used for this practice has been coined as – Traditional and Complementary Healthcare Approaches (TCA). Basis of TCA is on the cultural, historical, and religious backgrounds. According to this study, 29% of the parents used honey for their children as they believed it to be acceptable, natural, and safe. In fact, one General Practitioner also specifically mentioned honey as a ‘safe’ remedy. Overall, only five health care practitioners out of 13 (40%) and two parents out of 92 (2%) had awareness about infantile botulism and hence discouraged the use of honey (Kumar, Lorenc, Robinson & Blair, 2011).
Similarly, in another study conducted in Italy, it was reported that 25% of the mothers were using honey for their infants and were completely unaware of the fact that it can lead to Infantile Botulism (Aureli, Franciosa & Fenicia, 2002).
Another study also reported that there is little knowledge and awareness among health care practitioners for the use of honey as TCA (Suchard, Suchard & Steinfeldt, 2004). The reason being that they think it is the placebo effect, or they completely lack the knowledge about TCA in general (Kumar, Lorenc, Robinson & Blair, 2011).
Moving on to the diagnosis of the infantile Botulism, it is observed that as the disease is life threatening, therefore prompt response from the clinician’s is quite necessary. A high level of clinical suspicion is needed whenever a case of ‘floppy baby’ presents. Furthermore, it is imperative to send both stool samples as well as gastric or serum contents for making cultures and performing a direct assay of the botulinum toxin. The latter method of toxin assay is more reliable and gives prompt results while the stool test has a sensitivity of 60%. PCR can also be performed (Van Horn & Street, 2019). Recently, another crucial diagnostic test – electroneuromyography (EMG) – has proven to be an essential support to confirm and reach to the diagnosis even earlier than toxin detection. It is basically an electrodiagnostic test that detects motor and sensory nerve conduction velocity in one arm and leg with the help of 2 Hz stimulation of nerve at two distal muscles (Bernardor et al., 2018). Typically there are three electro-physiologic changes which are diagnostic for Infantile Botulism; they are 1) decreased amplitude of compound muscle action potential (CMAP) in response to supramaximal stimulation of the nerve (2) facilitations seen in tetanic or post tetanic phase after stimulation of 20-50 Hz (3) prolonged post tetanic facilitation or absence of post tetanic exhaustion of compound muscle action potential (CAMP) (Yanay et al., 2004) (Smith et al., 2010).
Although these diagnostic techniques have been mentioned in the sequence of increased reliability but they may not always be present, and hence it becomes difficult for the physicians to rule out the differentials or even pick the diagnosis in the first place. This is because similar characteristics are present in other diseases or conditions like – Electrolyte disturbances; conditions causing dehydration (hypoglycemia, hyponatremia, hyperkalemia); Metabolic abnormalities; musculoskeletal conditions such as Congenital myopathy; Leigh disease, Myasthenia gravis, Guillain-Barre Syndrome; and states like encephalopathy and sepsis (Van Horn & Street, 2019). Therefore, it is imperative for physicians to keenly monitor the cases and keep themselves aware regarding the spectrum of manifestations, extremely narrow time frame to diagnose, and variation in the reliability and sensitivity of the diagnostic tools. They must not look for the history of honey ingestion alone and thus put infantile Botulism above in the differentials whenever a case of weakness in infancy presents even in the absence of opposing diagnostic tests (Koepke, Sobel & Arnon, 2008).
As far as treatment of the disease is concerned, supportive treatment is given initially. At the same time, antibiotics are contraindicated because they cause lysis of the cells and lead to an increased surge of the toxin in the bloodstream hence causing more harm than good. Another treatment option for the severe form of the disease is Botulism Human Immune Globulin Intravenous (BIG-IV). The half-life of this drug is about 28 days in vivo, and it holds a huge potential to neutralize the botulinum toxin. An impressive feature is that a single infusion of BIG-IV is capable of neutralizing the toxin that may be absorbed from the gut of an infant for at least six months (Arnon et al., 2006).
Moreover, BIG-IV has been able to decrease the mean length of the hospital stay, the mean duration of intensive care, the mean duration of mechanical ventilation, the mean duration of nasogastric and intravenous feeding, and mean hospital charges per patient, all without any serious adverse effect (Arnon et al., 2006).
Hence, the main challenge of Infantile Botulism lay in the diagnostic domain of the disease; therefore, training and increased awareness must be provided to the health professional dealing with the conditions of infancy as the disease is controllable if it is diagnosed at an initial stage.
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