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DESCRIPTION
The infant botulism syndrome is an infectious neuroparalytic disease resulting from the ingestion of spores of "Clostridum botulinsum" which germinate, multiply and produce botulinal toxin within the infant's large intestine. First described in 1976 it has now been reported from all inhabited continents except Africa.
Infant botulism is an illness that can occur when a newborn ingests bacteria that produce a toxin inside the body. the condition is frightening because it can cause muscle weakness and breathing problems. But it is rare; Fewer than 100 cases of infant botulism occur each year in the United States, and most babies who do get botulism recover fully.
Infant botulism is treatable, but because of its severity, it is important to learn the symptoms so you can recognize it early. It's also important to know that honey is a known source of the bacteria spores that cause botulism. For this reason, avoid giving your baby honey.
This illness typically affects babies who are between 3 weeks and 6 months old, but they are susceptible to it until their first birthdays. When babies are born their immune and digestive systems are not fully developed. It takes the first year of life for these systems to mature. Extra care must be taken during that first year to make sure the baby is protected from invading microorganisms and infections.
ABOUT INFANT BOTULISM
Infant botulism can occur when a child ingests "Clostridium botulinum" bacteria, which are found in dirt and dust and can contaminate honey.
These bacteria are typically harmless to older children and adults because their mature digestive systems can move the bacteria through the body before they cause any harm. But very young babies haven't developed the ability to handle the bacteria yet. So once an infant ingests them, the bacteria can produce a toxin. That toxin interferes with the normal interaction between the muscles and nerves and can hamper an infant's ability to move, eat, and breathe.
Two other types of botulism tend to affect older children and adults: wound botulism occurs when the bacteria infect a wound and produce the toxin inside of it; food-borne botulism is usually caused by eating home-canned foods that contain the toxin.
A CASE HISTORY
A 3-month-old Caucasian female infant presented to a children's hospital with a 3-week history of constipation, 1-week history of decreased activity, decreased oral intake, weight loss and a 3-day history of fever. She was the product of a full-term delivery with no perinatal complications. She had been regularly breast-fed up to the day of presentation. She had also received supplemental formula feeding. She had not been given any other foods, specifically neither honey nor corn syrup. She had received her 2-month immunizations including live attenuated oral polio vaccine (OPV), DTP, and conjugated Hib vaccine.
The infant lived with both her parents and a 3-year old sister in Louisiana, having moved to Georgia a week prior to hospitalization. At the time the family was moving, road construction was being done near their house.
On her admission the patient had a temperature of 37°C, heart rate of 148/min, a respiratory rate of 48/min, and a blood pressure of 134/85 mm Hg. She appeared listless and weak, but otherwise well-nourished, with weight and height above the 50th percentile for age. Positive findings included dry oral mucous membranes, mildly sunken anterior fontanelle, weak suck, and generalized hypotonia, including poor head control and significant head lag. Deep tendon reflexes were present and normal in all extremities. The remainder of the physical exam was negative.
Initial abnormal labs included a leukocyte count of 24,000/mm3, with 56 percent segmented neutrophils, 2 percent band forms, 33 percent lymphocytes, 7 percent monocytes and 2 percent eosinophils. U/A results were normal except for 1+ ketones. Electrolytes were within normal limits with a blood urea nitrogen of 21 mg/dL and a creatinine of 0.4 mg/dL. CSF examination revealed 1 white blood cell, no red blood cells, protein of 54 mg/dL, and a glucose of 52 mg/dL. Blood, urine, and CSF cultures were obtained.
The patient was admitted with an initial diagnosis of sepsis with dehydration and started on IV fluids at 1.5 times maintenance. Also IV ceftriaxone at 75 mg/kg/day was started. During the next 48-72 hours, the patient developed ptosis, exhibited sluggish pupillary response, continued to be severely hypotonic, and had poor gag reflex with increasing difficulty handling oral secretions. There was marked decrease in the use of antigravity muscles.
Further laboratory evaluation included a thyroid profile, urine and serum examination for inborn metabolic disorders, and a tensilon test for myasthenia gravis, all of which were negative. Nerve conduction velocity was measured and electromyography (EMG) studies were performed, but the findings were nondiagnostic. A MRI of the head was not contributory.
At this point, a diagnosis of infantile botulism was strongly considered and a stool sample was sent to the CDC in Atlanta to determine the presence of Clostridium botulinum toxin.
SIGNS & SYMPTOMS
Infant botulism can be seen in children under 6 months of age. The mother usually notices listlessness, lethargy and poor feeding as the first signs. The weakness increases over 1 to 4 days (typically between 18 to 36 hours).
- Constipation is often the first symptom of botulism that parents notice. But many other illnesses can also cause constipation. So if your infant has not had a bowel movement in 3 days, it's a good idea to call your child's health care provider. The children usually receive medical attention because of the constipation.
- Flat facial expression. The descending paralysis is symmetrical. The first signs of illness are in the cranial nerves. The typical patient has expressionless face.
- Poor feeding (weak sucking action).
- A weak, feeble cry.
- Muscle weakness and decreased movement. Poor head control, and generalized weakness and hypotonia. Deep tendon reflex are normal initially and diminished subsequently as the paralysis extends and increases. The "frog-leg" sign is also seen. A general, progressive muscle weakness (hypotonia, dysphagia and in severe cases, flaccid paralysis).
- Trouble swallowing with excessive drooling. The gag, suck, and swallow reflex are impaired.
- The corneal reflex is impaired, if tested repetitively.
- Breathing problems and respiratory failure.
Infant botulism can be treated. But, it is important to get your child medical care as soon as possible. So if you see warning signs of the illness, call your health care provider right away.
CLINICAL OBSERVATIONS
The clinical spectrum ranges from asymptomatic "carriage" of infant botulism to a fulminant forms of the disease that may mimic sudden infant death syndrome. Most infants suffer from the moderate to severe form of the disease and require hospitalization. Constipation is a characteristically early sign, reflecting formation of toxin within the GI tract, although this is frequently overlooked. The child is usually afebrile. Infants then become listless and progressively weak over a period of days to weeks with decreased suck, weak cry, and hypotonia. Symmetric descending paralysis is the rule, and the cranial nerves are the first to be affected. The typical patient manifests hypoactive tendon reflexes, decreased suck and gag, poorly reactive pupils, bilateral ptosis, oculomotor palsies, and facial diplegia. In severe cases, flaccid paralysis with respiratory failure may occur. Occasionally, the onset of lethargy and weakness may be so abrupt as to mimic bacterial sepsis or meningitis, thus leading to a delayed diagnosis. It has been postulated that breast-fed infants with botulism are identified because they have milder disease with later onset, while non breast-fed infants present earlier with a clinical picture identical to that of sudden infant death syndrome.
CAUSE
Infant botulism is caused by "clostridium bacteria" that live in soil and dust. These bacteria may also contaminate foods, especially honey. Clostridium bacteria produce a toxin (poison) called botulinum toxin, which blocks the normal messages between muscles and nerves and affects muscles everywhere in the body. The toxin usually affects intestinal muscles first.
Infant botulism occurs worldwide, and 95-98 percent of cases occur in infants between 1 to 6 months of age. The remaining 2-5 percent are distributed over the subsequent 6 months. In the United States, most cases of infant botulism cannot be prevented, since the spores of clostridium bacteria are found in soil everywhere.
The illness has occurred in all major racial and ethnic groups in equal proportions in males and females. The diagnosis should be considered as a possibility in any season of the year given a compatible history. The illness occurs with increased frequency (more than 90 percent of reported cases) in the USA come from California, Utah, and southeastern Pennsylvania. Besides honey, the soil in Pennsylvania, the soil and cistern water in Australia and the soil and vacuum cleaners in California were also identified as a source of spores. This is likely a consequence of high concentrations of C. botulinum spores in the soil of these regions. But Clostridium b. is a ubiquitous gram-positive, spore forming, obligate anaerobe with soil and dust as its natural habitat. However, for the majority of infant botulism, no source of spores is ever identified. Infant botulism typically affects previously well infants within the first 4 to 6 months of life (median=10 weeks) and is caused by ingestion of spores that germinate and produce toxins in the GI tract. Seven antigenically distinct types of botulinum toxins have been identified although all cases of infant botulism have been caused by types A or B. The toxin irreversibly blocks the peripheral cholinergic synapses throughout the body, most importantly at the neuromuscular junction. The toxin does not cross the blood-brain barrier. Epidemiologic studies have implicated honey, and to a lesser extent, corn syrup as factors. The significance of breast-feeding as a risk factor is controversial. It is clear that many infants who are diagnosed with infantile botulism are primarily breast-fed. However, it it not clear whether it confers some protection, slowing the process of the disease and allowing the infant to reach the hospital alive. Preexisting host factors such as frequency of bowel movement may be other risk factors for the disease
DIAGNOSIS
Infant botulism may be difficult to recognize in its early stages, and even today, the most common admitting diagnosis is "rule out sepsis." It usually displays a spectrum in its clinical severity. Almost all recognized cases have been sufficiently hypotonic and weak toward hospitalization. The onset of infant botulism ranges from insidious to abrupt. In the classic case the first sign of illness is almost always constipation, yet the constipation is often overlooked. Less than 5 percent will present without constipation.
The diagnosis is confirmed by the detection of the organism or its toxin in the infant's stool. Toxin isolation and identification are accomplished via mouse lethality testing, with typing confirmed by neutralization of toxin by specific sera. EMG if abnormal, typically show a pattern of brief duration, small-amplitude potentials with a decremental response at low repetitive (3-10 Hz), and an incremental or "staircase" response at high repetitive stimulation. The differential diagnosis of infant botulism includes other causes of paralysis such as Guillan-Barré syndrome (symmetric ascending paralysis with an elevated CSF protein), poliomyelitis (asymmetric paralysis, fever, and CSF pleocytosis), and myasthenia gravis (muscle fatigability with reversal or ptosis with tensilon). In addition, bacterial sepsis and meningitis must always be excluded in any infant presenting with fever, lethargy, and poor feeding. The absence of a gag reflex, profound hypotonia, and hyporeflexia help to differentiate infant botulism from bacterial sepsis.
DURATION
Infants with infant botulism may require hospital-based support for an extended period. In severe cases of infant botulism, the child may require several weeks of hospitalization. Infant botulism is a self-limited disease lasting a total of 2 to 6 weeks with progressive symptoms for 1 to 2 weeks followed by gradual recovery of motor function over 3-4 weeks, as a result of the production of new nerve terminals and motor-end plates. Relapse has been reported after apparent recovery, thus necessitating close supervision and follow-up.
CONTAGIOUSNESS
No special isolation or precautions are needed since this infection is not transmitted from person to person.
PREVENTION
Most cases of infant botulism cannot be prevented. Like many germs, the bacteria that cause botulism in infants are everywhere in the environment. They are in dust and dirt. They are even in the air. Experts do not know why some infants end up consuming the airborne bacteria and contracting botulism while others do not.
One of the only things you can do to reduce the risk of botulism is to avoid giving your infant honey or corn syrup before his or her first birthday. Honey is a proven source of the bacteria and it has led to botulism in infants who ingested it, while some researchers think that light or dark corn syrups that are not sterilized may also harbor the bacteria spores and could lead to cases of botulism. This is the reason it is universally recommended that parents can eliminate one risk factor by not feeding honey or corn syrup to children under age 1 year.
Other helpful suggestions may be to make sure that if you are using a formula (or other baby foods) to feed your baby that all containers and utensils are thoroughly clean and the water (if you use water to mix with the formula) is clean and pure. Bottled water or steam distilled water is recommended, if necessary. Avoid tap water. If you are breastfeeding your baby, make sure you clean your nipples prior to feeding and again after feeding is finished. Make sure any toys are clean that are used by your baby (and are inserted into your baby's mouth).
WHEN TO CALL YOUR HEALTH CARE PROVIDER
Call your health care provider immediately if your infant has trouble breathing or if he/she seems to have trouble swallowing and is drooling abnormally. Also call your health care provider if your infant does not seem to be feeding well, cries weakly, has trouble holding her head up, or has stopped sucking normally.
Although it may be just a minor constipation problem, you may want to check with your health care provider if your infant has not had a bowel movement in 3 days.
PROFESSIONAL TREATMENT
Health Care Providers make the diagnosis of infant botulism by checking the infant's stool for clostridium bacteria or clostridium botulinum toxin. A child with infant botulism is treated in a hospital, usually in an intensive care unit, where your health care provider will try to limit the problems the toxin causes in the baby's body.
Because the toxin can affect the breathing and swallowing muscles, aggressive respiratory and nutritional care (nasojejunal tube) are the mainstays of treatment. Many infants require intubation and prolonged mechanical ventilation (ventilator). Continuous nasogastric feedings are preferred over bolus feedings to minimize the risk of aspiration. The baby may be given intravenous (IV) fluids to provide nourishment. Small volumes increased over a few days are well tolerated and decrease the need for central hyperalimentation. Physical and occupational therapies are crucial in maintaining range of motion and functional positioning in patients. Antibiotics have not shown to ameliorate the course of the disease. Aminoglycosides such as gentamicin should be avoided for they may potentiate the neuromuscular blockade. The role of human botulism immune globulin (BIG) to modify the course of the disease if administered early is being explored.
HOME TREATMENT
Infant botulism is treated in a hospital in virtually all cases. Once discharged, infants with botulism usually require routine care.
PROGNOSIS
Prognosis for complete recovery is excellent with meticulous supportive medical care. The infant will likely recover once the effects of the toxin wear off. The parents need to understand the clinical course is reliable for small improvements and deterioration. Movement of the fingers provides an early sign of returning muscle strength and is closely followed by incomplete eye opening. Return of a weak cough and improvement in swallowing will be accompanied by decrease in need for suctioning. When the strength of cough improves and suction becomes unnecessary, the patient can be safely extubated.
In the absence of hypoxic cerebral complications, full and complete recovery of strength and tone is expected. Since the toxin does not cross the BBB to any functional degree, the intelligence and personality remain unaffected. Reinfection with the same or different toxins does not occur.
At present, the recommendation is to avoid feeding honey and corn syrup to infants, Breast feeding may help moderate the rapidity of onset and severity of illness.
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