D Spencer Mangum, PGY3 8/16/13

2 month old previously healthy male, brought to the PCMC ED for concerns of “fussiness” x1 day with difficulty consoling him. • Also has poor feeding x1 day:
• Seems hungry, but is refusing the bottle (will push away with his

tongue and start crying)
• Question as to if has a weak cry?

• Taken only 6 ounces over past 24 hours compared to his normal 20-24

ounces (fed similac) • 4-5 wet diapers over past 24 hours (normally has 6-7 wet diapers) • Parents wonder if he has abdominal pain?
• Recent congestion, no cough, increased WOB, fever. • No vomiting, diarrhea, normal stooling with exception that has

not stooled in past day. • No known sick contacts with exception of mother who has a history of cold sores for which she takes valacyclovir (had a cold sore as recently as last Tuesday).

PMHx: Healthy. Full-term, NSVD, no complications with pregnancy or delivery. Imm’s UTD. No regular medications. NKDA. FHx: Healthy. No history of metabolic, genetic, neuromuscular, or congenital problems. SHx: He is visiting with his family from Phoenix. Lives with mom, dad, and maternal grandparents (no siblings).

T 36.5, HR157, RR 44, BP 96/50, O2 Sat 94% on RA WEIGHT - 4.2 Kg, (7%ile) HEIGHT - 56 cm, (25%ile) GENERAL: Laying on bed, “lethargic” appearing. Not fussy or crying. HEENT: NC/AT, AFOSF, conjunctivae are clear, PERRL, TM’s clear, +nasal congestion without rhinorrhea, “tacky” mucus membranes, no oral lesions, posterior oropharynx without redness. No LAD. Neck is supple. PULM: No increased WOB. CTAB with symmetric breath sounds. CARDIOVASCULAR: RRR, +S1 and S2, no murmur / gallop ABD: Soft, NT/ND, normoactive bowel sounds, liver palpable finger width below costal margin, spleen not palpable GU: Penis is without redness, swelling, or discharge. Testes descended b/l. No masses or hernia. EXT: WWP x4, full ROM SKIN: Petechial rash over abdomen and chest. NEUROLOGIC: CN 2-12 grossly intact. Arousable but diffusely hypotonic. No tongue writhing / abnormal tongue movements.

Differential Diagnosis
• 2 mo infant with fussiness and poor feeding x1 day, now

found to be lethargic / hypotonic with a petechial rash.

Differential Diagnosis
• • • • • • • • • • • • •

1. Bacterial Sepsis 2. Bacterial Sepsis 3. Bacterial Sepsis Viral illness with dehydration CHF Ingestion Botulism Spinal Muscular Atrophy Miller Fisher Variant of Guillain-Barre (5% of GBS) Any lesion leading to spinal cord compression (ie Neuroblastoma) Metabolic genetic disorders NOS Encephalitis (brainstem or metabolic) Other Neuromuscular disorders: hereditary motor sensory neuropathy, congenital myasthenia gravis and Duchenne's muscular dystrophy • Cerebral infarction • Hypermagnesemia (blocks Ca++ release -> inhibits presynaptic acetylcholine release, typically at birth w/ mom’s who received mag sulfate) • Aminoglycoside toxicity (Can cause presynaptic block as well)

Patient Hospital Course
ED Course: Full rule out sepsis performed (Blood, Urine, and CSF obtained), no urine was initially obtained on cath and was subsequently given two 20 ml/kg NS boluses. Started on ceftriaxone and acyclovir. VRP, BMP and Chest X-ray obtained. Only notable finding of platelets of 101. Hospital Course: Patient had progressive hypotonia and lost his gag reflex after first day of hospitalization. Neuro was consulted. While team was discussing possible initiation of Botulism IgG (BabyBIG) with family, patient became apneic requiring intubation and was transferred to the PICU. Neuro recommended obtaining stool to eval for botulism and first rule out inborn errors of metabolism, infection, other causes prior to initiating BabyBIG. BabyBIG given on hospital day 4 after other tests negative. Botulism was confirmed by hospital day 6. Patient remained intubated x2 weeks, hospitalized at PCMC x1.5 months. Ultimately transferred to Phoenix Children’s hospital for parental convenience / continued monitoring of his ability to protect his airway and nutritional support.

Botulism: Overview
• Clostridium botulinim is a gram positive rod that produces

a potent neurotoxin
• Toxin irreversibly inhibits acetylcholine release by presynaptic

nerve terminals but does not cross the blood brain barrier
• Thus affects only the peripheral cholinergic nervous system • Inhibits neuromuscular junctions (descending paralysis), decreased GI

motility (constipation), urinary retention…
• Return of function requires creation of a new synapse – which

takes approximately 6 months.

Botulism: Infection Routes
• Ingestion of spores with toxin made in vivo: • Infantile botulism (72% of all botulism cases)
• Ingestion of environmental dust containing C. botulinum spores • Pennsylvania, Utah, and California are hotspots in North America • Living near activities that disturb the soil (construction or agricultural cultivation) may be risk factors

• Honey also to be a known source of botulinum spores.

• Can occur in adults (rarely reported)
• Adult GI tracts should be resistant to intestinal colonization by

Clostridium botulinum (hence honey is OK after 1 year)

Botulism: Infection Routes
• Ingestion of preformed toxin without spores • Foodborne (25% of all botulism)
• Sausage: Initially discovery and recent outbreak in China • Latin word botulus means sausage • Home canned foods

• Wound infection with toxin made in vivo (3% of all cases) • Most common with drug users, notably those who inject heroin “black tar” IM

Botulism: Clinical Presentation
• Infantile Botulism: • Constipation: typically the first symptom • Descending paralysis:
• Poor feeding (poor suck / swallow), Poor head control, ptosis • Weak Cry • Generalized weakness / floppiness

• Can progress to apnea and in rarer cases will occur rapidly

• Foodborne Botulism: • Onset of GI symptoms within 12-36 hours
• Nausea, vomiting, abdominal pain, diarrhea

• Followed by neurologic symptoms (descending paralysis: CN deficits /

blurry vision / dysarthria then trunk muscles) • Quite variable in degree of severity
• Anywhere from only mild complaints to rapid progression leading to death

• Wound Botulism: • Same as foodborne minus GI complaints

Botulism: Treatment
• Suspect infantile botulism? • Hospitalize immediately and monitor for signs of respiratory failure. • BabyBIG (for infants less than 1 year) should be administered as early as possible to prevent further irreversible nerve damage
• IgG antibodies against toxin, collected / pooled from donor’s with high titers • Early intervention leads to significant decreases in length of hospitalization

and need for mechanical ventilation / ICU care. • You should not wait for confirmatory testing (takes at least 4-6 days)
• Make sure you fully evaluate for other possible etiologies of symptoms

as well
• Cost: • BabyBIG is expensive (~$45,000 per vial), but so is prolonged hospitalization. When BabyBIG is used with botulism, reduces total hospital cost by ~$90,000 on average • Equine anti-toxin is used for patients over 1 year of age • Anaphylaxis and serum sickness may occur • Need to contact state health department prior to use

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