P3C023: Don’t Sweat It: Bulbar Weakness and Dyspnea in a 21-year-old Male with Recent Travel to Iran

Introduction: Although there is a broad differential for weakness in the emerging adult, careful history and examination can help detect serious causes which require urgent intervention.

Case Description: A 21-year-old male with no medical history presented for dyspnea and dysphagia that began 7 days previously while visiting Iran. He traveled back to the U.S. 5 days prior to presentation and since then also developed diplopia and difficulty with distance vision. He denied animal/insect bites, contaminated food/water exposure, or substance use. Family history was negative for autoimmune or neuromuscular junction disease. On further history, he reported that while in Iran he underwent a total of 2,000 IU AbobotulinumtoxinA (Dysport) injections in his bilateral hands after which he developed mild hand weakness.

On presentation, the patient had a hoarse, tremulous voice and bilateral ptosis. He had 4/5 strength in neck flexion, trunk/hip extension, and deltoid muscles bilaterally. Sensation, reflex, and gait were normal.

Negative Inspiratory Force (NIF) was -60. Given concern for respiratory collapse, he was admitted to the ICU but not intubated. The CDC was contacted. Stool botulinum toxin was sent and he received botulinum antitoxin on the day of admission. EMG demonstrated a local presynaptic process impacting the muscles of the left hand without evidence of myasthenia gravis. Acetylcholine receptor and striated muscle antibodies were negative. He had no further worsening of symptoms following antitoxin administration and was discharged home on hospital day 4 where he continues to receive physical rehabilitation.

Discussion: Botulism is a rare but commonly misdiagnosed illness due to the broad differential including Myasthenia Gravis, Guillain-Barre Syndrome, and Miller-Fisher Syndrome (1). Although food-borne and infant botulism are more common, iatrogenic botulism is important for Med-Peds physicians to recognize given increasing botulinum toxin use in children and adults for indications including dystonia, sialorrhea, spasticity, and cosmetic procedures (2). Patients present with descending paralysis and bulbar weakness due to inhibition of neurotransmitter release and often have normal reflexes.

Botulinum toxin dilution, dosing, and administration are strictly regulated in the United States. Thus, many cases are in the setting of inappropriate dilution and dosing due to poor adherence to regulations, or in regions without such regulations (3-5). Our patient received 2,000 IU of Botulinum toxin, which is far over the recommended 25 units/kg or maximum dose of 400-600 IU (2). Careful history and examination facilitate prompt reporting of this illness to government agencies to obtain antitoxin, which significantly reduces mortality. Antitoxin administration does not reverse existing paralysis and patients often require prolonged physical rehabilitation (1).

Conclusion: Med-Peds physicians should consider iatrogenic botulism in patients with descending paralysis and bulbar symptoms who are returning from international travel or who have complex medical conditions receiving therapeutic botulinum toxin.