Generalized Weakness in a Tenmonthold Infant

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Generalized Weakness in a Ten-month-old Infant

• Andrew S. Johnson, MD
• Pediatric Emergency Medicine
• University of Utah

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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

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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

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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

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Case Presentation

• Physical Exam
• Temp. 36.6 C, P 147, R 34, O2 sat. 99, BP
  126/73
• Weakness in hands (RgtL)
• Poor head control
• Difficulty sitting
• Face symmetrical, Gag intact, 2 DTRs

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Case Presentation

• Laboratory
• 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

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Case Presentation

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

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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

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Case Presentation

• Hospital Course (cont.)
• MRI of head normal
• Diagnostic studies obtained
• Definitive treatment initiated

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Infantile Botulism Background

• Van Ermengem 19th Century
• Botulus - Sausage (Latin)
• First infant case reported in 1931
• Distinct clinical entity 1976

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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

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Infantile BotulismEpidemiology

• 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

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Infantile BotulismRisk Factors

• Breast feeding (controversial)
• Transition in feedings
• Spore density
• Local construction or family member working with
  soil
• Honey consumption (4-25)

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Infantile BotulismClinical 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

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Infantile BotulismClinical 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

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Infantile BotulismPhysical Exam

• Autonomic findings (anticholinergic)
• Labile blood pressure and heart rate
• Decreased anal sphincter tone
• Urinary retention
• Flushed skin
• Constipation

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Infantile BotulismDifferential Diagnosis

• Sepsis
• Myasthenia gravis
• Guillain-Barre syndrome (Miller-Fisher variant)
• Tick paralysis
• Heavy metal/organophosphate poisoning
• Werdnig-Hoffman disease
• Poliomyelitis
• Hypothyroidism

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Infantile BotulismDiagnosis

• 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

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Infant BotulismDiagnostic 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

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Botulism Testing Centers
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Infantile BotulismTreatment

• 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

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Infantile BotulismComplications

• Hyponatremia
• Hypoxia
• Aspiration pneumonia
• Urinary tract infection
• Otitis media

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Infantile BotulismSummary

• 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

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Infantile BotulismCase 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