1. Generalized Weakness in a Ten-month-old Infant
Andrew S. Johnson, MD
Pediatric Emergency Medicine
University of Utah

2. Case Presentation
10-month-old healthy female brought to pediatrician due to general weakness for twenty-four hours.
Increased Drooling
“Droopy Eyelids”
Difficulty latching onto breast
Poor suckle
Dx- Otitis media and viral syndrome
Tx- Begun on amoxicillin

3. Case Presentation Returns to E.R. that evening with right arm weakness
PMHx:
Term pregnancy without complications
Frequent middle ear infections
Recent URI in patient and sibling
No other medications or herbal supplements

4. Case Presentation History (cont.) Immunizations current
No recent travel or camping
Recent home remodel and waterline construction near home
No corn syrup or honey exposure
ROS: “loose” stools

5. Case Presentation Physical Exam
Temp °C, P 147, R 34, O2 sat. 99%, BP 126/73
Weakness in hands (R>L)
Poor head control
Difficulty sitting
Face symmetrical, Gag intact, 2+ DTRs

6. Case Presentation Laboratory Radiographs
CBC, Electrolytes, Spinal fluid, Urinalysis
WBC 13,000, CO2 17
Blood, Urine, Stool, and CSF Cultures
Radiographs
Computed tomography of the head without contrast: Normal

7. Case Presentation Hospital Course Admitted to Neurology Service
Progressive Hypotonia
Nasogastric tube placed for feedings
Loss of gag reflex
Loss of facial expressions
Ptosis

8. Botulism 3 distinct clinical infections
Wound
Food borne
Infantile
Adult- compromised host
Clostridium Botulinum (Baratii, Butyricum)
Gram + rod, obligate anaerobe – hardy spores
Most potent toxin known to man

9. Case Presentation Hospital Course (cont.) MRI of head: normal
Diagnostic studies obtained
Definitive treatment initiated

10. Infantile Botulism: Background
Van Ermengem – 19th Century
Botulus - Sausage (Latin)
First infant case reported in 1931
Distinct clinical entity – 1976

11. Infantile Botulism: Pathophysiology
Toxin-infection versus ingestion
Lack of competitive intestinal flora
Neuroparalytic disease caused by heat-labile toxin
Irreversibly binds to presynaptic nerve endings of cranial and peripheral cholinergic nerves
Blocks calcium dependent exocytosis of acetylcholine vesicles

12. Infantile Botulism: Epidemiology
Reservoir: soil (surface of fruit and vegetables), marine life, birds, honey
? Corn Syrup
Seven toxin types (A-G)
90% of cases types A and B
Type A- West of the Mississippi River
Type B- East to West Distribution
85% Indeterminate source
Majority of U.S. cases are Infantile

15. Infantile Botulism Risk Factors
Breast feeding (controversial)
Transition in feedings
Spore density
Local construction or family member working with soil
Honey consumption (4-25%)

16. Infantile Botulism Clinical Presentation
95% of cases occur in the first 6 months of life (range: day of life 6 – 363)
SIDS association
Descending neuromuscular blockade
Cranial nerves
Trunk
Extremities
Diaphragm

17. Infantile Botulism Clinical Presentation
Symptoms
Constipation (most common, 65-95%)
Lack of expression
Weak suck and prolonged feeding
Drooling
Floppiness
Signs
Poor Head Control
Loss of Gag/Suck
Sluggish or nonreactive pupils
Hyptonia/Hyporeflexia
Diminished range of eye movements

18. Infantile Botulism: Physical Exam
Autonomic findings (anticholinergic)
Labile blood pressure and heart rate
Decreased anal sphincter tone
Urinary retention
Flushed skin
Constipation

19. Infantile Botulism: Differential Diagnosis
Sepsis
Myasthenia gravis
Guillain-Barre syndrome (Miller-Fisher variant)
Tick paralysis
Heavy metal/organophosphate poisoning
Werdnig-Hoffman disease
Poliomyelitis
Hypothyroidism

20. Infantile Botulism Diagnosis
Requires isolation of the organism or toxin
Laboratory
laboratories, including CSF, usually show no significant abnormalities
Stool samples for toxin and culture
+ for up to 4 months
Electromyography
Characteristic BSAP (Brief, Small, Abundant motor unit Potentials)
Specific but not sensitive

21. Infant Botulism Diagnostic Testing
Among 309 persons with clinically diagnosed botulism reported to CDC from 1975 to 1988:
Stool cultures for C. botulinum: 51% +
Serum botulinum toxin testing: 37% +
Stool botulinum toxin testing: 23% +
Overall, at least one of the above tests was positive for 65% of all patients

22. Botulism Testing Centers

23. Infantile Botulism Treatment
SUPPORTIVE
Await growth of new nerve endings
Botulinum antitoxin (not used in infants)
Trivalent equine product against types A,B, and E available from CDC
Associated with anaphylaxis and serum sickness
Antibiotics
may increase toxin production or enhance neuromuscular blockade (aminoglycosides)
Botulinum Immunoglobulin (BIG) via CDC
Binds free toxin, halts progression of disease, and shortens length of hospitalization

24. Infantile Botulism Complications
Hyponatremia
Hypoxia
Aspiration pneumonia
Urinary tract infection
Otitis media

25. Infantile Botulism Summary
Consider this uncommon neuroparalytic disease in infants in the first year of life with weakness or cranial nerve deficits
Diagnosis is confirmed by culture or toxin identification at regional centers
Supportive care is the mainstay of early treatment
Administration of BIG will prevent progression of disease

26. Infantile Botulism Case Presentation - Resolution
Stool sample demonstrated toxin
EMG was consistent with botulism
BIG was administered and clinical status stabilized
Patient gradually recovered over two weeks and was discharged to home once gag reflex and feeding abillity had returned to normal