Infantile botulism: an atypical case of an uncommon disease.

Abstract

* Abbreviations: IB = : infantile botulism • EDT = : electrodiagnostic testing • WBC = : white blood cell count • RBC = : red blood cell count Most cases of infantile botulism (IB) occur between 2 to 6 months of age, have a history of constipation, and a descending flaccid paralysis with cranial nerve palsies. Electrodiagnostic testing (EDT) with electromyography and nerve conduction velocity testing has characteristic findings. Many cases of IB lack this classical presentation, however, and a wide spectrum of clinical manifestations and disease severity exists. The spectrum of presentations and extremely low incidence accounts for much of the difficulty pediatricians face in recognizing this disease entity. The flaccid paralysis of IB manifested by either hypotonia or weakness is one of the more consistent features. Various studies report it to be present in 88 to 100% of patients with IB.1–4 The diagnosis of IB requires isolation of Clostridium botulinum or its toxin from the stool. Recognition of atypical presentations of this uncommon disease is essential. Early in the disease course, stool must be obtained to allow for culture and toxin analysis. Appropriate supportive care must be instituted. We present a case of IB with the initial complaint of hypotonia to emphasize the several atypical features. The diagnosis of IB was somewhat delayed secondary to these atypical features. This breastfed, previously healthy, 9-month-old presented with several hours of acute hypotonia, lethargy, irritability, and head lag. There was no history of fever, vomiting, rash, honey ingestion, tick or toxin exposure, travel, snakebite, or antecedent illness. Constipation was not present and her normal stool pattern of 1 or 2 soft, yellow stools each day had been maintained. Family history was unremarkable for neurologic disorders. Immunizations were current, none given recently, and she was on no medications. Her developmental milestones were normal and there were no recent dietary changes. On initial evaluation, she was afebrile and nontoxic. Her neurologic examination showed an … Address correspondence to James R. Rick, Maj, MC, USAF, San Antonio Military Pediatrics Center, Wilford Hall Medical Center (MMNP), 2200 Bergquist Dr, Suite 1, Lackland AFB, TX 78236-5300. E-mail:jrick{at}sa-chcs.mednet.af.mil