Infant botulism results from germination of swallowed spores of botulinum
toxin–producing clostridia that colonize the large intestine temporarily.
Four cases of type B infant botulism in one New York City (NYC) borough were
diagnosed within a 12-month period during 2001-2002. All four patients resided
in Staten Island (2000 population: 443,728). The annual incidence of infant
botulism in the United States is two cases per 100,000 live births; incidence
in NYC is four cases per 100,000 live births. Staten Island recorded 5,899
live births in 2000; incidence of infant botulism during this 12-month period
was 68 cases per 100,000 live births. This report summarizes the investigation
of these four cases; as expected with infant botulism, a common source of
exposure was not identified. All four patients recovered after treatment and
were discharged from local hospitals. State and local health departments should
be notified promptly when infant botulism is suspected to arrange diagnostic
Infant botulism is a reportable disease in NYC, and the NYC Department
of Health and Mental Hygiene (DOHMH) investigates all suspected cases. Botulism
should be suspected in an infant aged ≤12 months with symptoms including
constipation, lethargy, poor feeding, weak cry, bulbar palsies, and failure
to thrive. These symptoms might be followed by progressive weakness, impaired
respiration, and sometimes death. Laboratory diagnosis of clinically suspect
cases requires detection of botulinum toxin in stool or serum by using the
mouse neutralization assay or the isolation of toxigenic Clostridium botulinum (or related toxigenic clostridia) in the feces
by using enrichment culture techniques.1 When
botulism is suspected, clinical specimens are forwarded to the DOHMH Public
Health Laboratory for toxin detection. Parents or caregivers are interviewed
by using a standardized questionnaire on clinical symptoms and risk factors,
and the physician is interviewed or charts are abstracted for information
on signs and symptoms.
Case 1. In June 2001, a previously healthy breast-fed infant aged 7 weeks with
fever of 105°F (41°C), constipation, listlessness, poor feeding, and
weak head control for 1 day was admitted to a New Jersey hospital. The infant
was irritable and had sluggishly reactive pupils, altered cry, somnolence,
respiratory weakness, and upper airway obstruction that necessitated mechanical
ventilation for 13 days. Botulinum toxin type B was identified 8 days after
illness onset in stool samples at the New Jersey Public Health and Environmental
Laboratories. The family had spent time at residences in Staten Island and
New Jersey before illness onset. The patient was discharged after 26 days
without sequelae and recovered fully.
Case 2. In December 2001, a formula-fed infant aged 10 weeks with a history
of constipation in the first month of life was admitted to a hospital after
having difficulty in sucking and swallowing for 2 days. Mechanical ventilation
was required for 10 days because of respiratory failure. The infant was irritable
and had loss of facial expression, generalized muscle weakness, and constipation.
A diagnosis of infant botulism was established 29 days after onset of symptoms
by detection of toxin type B in stool enrichment cultures. The patient was
treated with Botulism Immune Globulin Intravenous (human) (BIG-IV) and discharged
after 20 days; the infant recovered fully.
Case 3. In May 2002, a previously healthy breast-fed infant aged 18 weeks had
somnolence and difficulty swallowing for 1 day. The infant was admitted to
a hospital and subsequently had altered cry, loss of facial expression, respiratory
muscle weakness, and upper airway obstruction that necessitated mechanical
ventilation for 8 days. The patient also had an intussusception without a
recognizable lead point.2 A diagnosis of
botulism was established 19 days after onset of symptoms by detection of toxin
type B in stool enrichment cultures. The patient was treated with BIG-IV and
discharged after 16 days; the infant recovered fully.
Case 4. In June 2002, a previously healthy infant aged 3 weeks who was both
breast- and formula-fed had constipation, lethargy, and decreased appetite
for 2 days; the infant was brought to a hospital for evaluation and was admitted
the following day. The infant was irritable and had sluggish pupillary reflexes,
difficulty swallowing, altered cry, weak sucking, and peripheral weakness.
A diagnosis of botulism was established 8 days after onset of symptoms by
detection of toxin type B in stool samples. The patient was treated with BIG-IV
and discharged after 10 days; the infant recovered fully.
All four patients received antibiotics during hospitalization. None
had ingested honey or had parents employed in occupations that might increase
exposure to C. botulinum spores in soil and dust
(e.g., construction, plumbing, and farming).3 All
patients had uncomplicated gestational histories and vaginal deliveries. All
resided within a 6-mile radius of each other. All parents reported recent
construction in their neighborhoods during the period (range: 1-31 days) before
illness onset. In the fourth case, the infant's home had been remodeled since
the infant was born.
On May 30, 2002, after three cases had been identified by routine passive
surveillance, DOHMH alerted local physicians by broadcast facsimile and e-mail
about the increased rate of infant botulism in Staten Island. Physicians were
reminded to consider the diagnosis and to report suspected cases to DOHMH
to request assistance with diagnostic testing. Physicians were directed to
contact the California Department of Health Services (CDHS) Infant Botulism
Treatment and Prevention Program about possible treatment with BIG-IV under
a Food and Drug Administration (FDA)–approved protocol.4 All
parents of patients in Staten Island were reinterviewed by using an expanded
questionnaire and visited in their homes and neighborhoods to assess for possible
common sources of exposure; no such source was identified.
V Reddy, MPH, S Balter, MD, D Weiss, MD, M Layton, MD, Bur of Communicable
Diseases; L Kornstein, PhD, Public Health Laboratory, New York City Dept of
Health and Mental Hygiene, New York, New York. I Friberg, MHS, R Schechter,
MD, S Arnon, MD, Infant Botulism Treatment and Prevention Program, California
Dept of Health Svcs. MJ Hung, MSW, E Bresnitz, MD, New Jersey Dept of Health
and Senior Svcs; K Pilot, S Matiuck, New Jersey Public Health and Environmental
Laboratories. J Sobel, MD, Div of Bacterial and Mycotic Diseases, National
Center for Infectious Diseases; M Phillips, MD, EIS Officer, CDC.
Intestinal botulism is the most common form of human botulism in the
United States,1 and approximately 100 cases
are reported among infants in the United States annually
botulism occurs rarely in older children and adults.2,5,6 Intestinal
botulism results from colonization and bacterial production of botulinum toxin
in the colon. Swallowing ambient C. botulinum spores,
which exist worldwide in soil and dust, has been proposed as the principal
route of exposure; honey is an avoidable source of some causative spores.4 A common source of exposure generally is not identified;
apparent clusters such as the four Staten Island cases are rare and often
remain unexplained after investigations are complete.7 In
a cluster of infant botulism cases identified previously in the mid-Atlantic
region of the United States, no common source of exposure was identified.8
Botulism should be suspected in previously healthy infants aged ≤12
months who are constipated and who exhibit weakness in sucking, swallowing,
or crying; hypotonia; and progressive bulbar and extremity muscle weakness.
Approximately half of patients require mechanical ventilation during hospitalization.9 Lumbar puncture and brain imaging generally yield
normal results but can help differentiate among other causes of flaccid weakness.
When infant botulism is suspected, local and state health departments should
be notified promptly to arrange diagnostic stool testing.
The primary therapy for infant botulism is intensive care with mechanically
assisted ventilation when necessary. Prompt diagnosis and treatment of infant
botulism with BIG-IV might reduce the length of time needed for recovery.
In a placebo-controlled trial of BIG-IV, the mean hospital stay of patients
with infant botulism was reduced from 5.6 to 2.6 weeks.4 Therapy
is guided by clinical diagnosis; to avoid delay in treatment, BIG-IV should
be requested and administered without awaiting laboratory confirmation. BIG-IV
can be obtained from the CDHS Infant Botulism Treatment and Prevention Program,
telephone 510-540-2646. Use of BIG-IV under the FDA-approved Treatment Investigational
New Drug open-label protocol requires informed parental consent and coordination
with the hospital's institutional review board (IRB). The license application
for BIG-IV was filed with FDA in 2001; should it be licensed, IRB approval
would no longer be required. Infant botulism is notifiable at the national
level, and physicians should report all cases promptly to state and local
Intestinal botulism is the most common form of human botulism
reported in the United States; approximately 100 cases are reported annually.
The majority of cases occur among infants aged <6 months;
intestinal botulism is seen rarely in adults.
The majority of cases are caused by botulinum toxin types A and B.
The case-fatality rate of hospitalized patients is <1%
Although ingesting honey is a known risk factor, the source of spores for
the majority of cases is unknown.
Ingestion of Clostridium botulinum spores,
which exist worldwide in the soil and dust, is believed to be the principal
route of exposure.
Reporting symptoms range from constipation and mild lethargy
to hypotonia and respiratory insufficiency.
Symptoms in infants aged <12 months include constipation,
lethargy, poor feeding, weak cry, bulbar palsies (e.g., ptosis, expressionless
face, and difficulty swallowing), and failure to thrive.
Presenting symptoms might be followed by progressive weakness,
impaired respiration, and sometimes death.
Differential diagnosis includes sepsis, dehydration, Werdnig-Hoffman
disease, Guillain-Barré syndrome, myasthenia gravis, drug or toxin
ingestions, metabolic disorders, and meningoencephalitis or myelitis.
Laboratory confirmation requires detection of 1) botulinum toxin
in stool or serum by using mouse neutralization assay or 2) isolation of toxigenic C. botulinum (or related clostridia) in the feces by using
stool enrichment culture techniques.
To avoid delay, treatment should be administered without awaiting
Primary therapy is supportive care with mechanically assisted
ventilation when necessary.
Prompt clinical diagnosis and treatment with Botulism Immune
Globulin Intravenous (human) (BIG-IV) might reduce the recovery time. BIG-IV
should be requested without awaiting laboratory confirmation.
BIG-IV can be obtained from the California Department of Health
Services, Infant Botulism Treatment and Prevention Program, telephone 510-540-2646.
Prevention and reporting
Avoid feeding honey to infants aged <12 months.
Report all cases to local and state health departments.
Infant Botulism—New York City, 2001-2002. JAMA. 2003;289(7):834-836. doi:10.1001/jama.289.7.834