Intestinal Toxemia Botulism in Two Young People, Caused by Clostridium butyricum Type E

Lucia Fenicia 0 1 Giovanna Franciosa 0 1 Manoochehr Pourshaban 0 1 Paolo Aureli () 0 1 0 Received 7 May 1998; revised 3 August 1999. Presented in part: Interagency Botulism Research Coordinating Com- mittee Meeting, Centers for Disease Control and Prevention , Atlanta, GA , 13-15 November 1995. nita`, Laboratorio Alimenti, Viale Regina Elena 299, 00161 Rome, Italy 1 From the Food Microbiology Laboratory, National Reference Center for Botulism, Istituto Superiore della Sanita` , Rome, Italy Two unconnected cases of type E botulism involving a 19-year-old woman and a 9-yearold child are described. The hospital courses of their illness were similar and included initial acute abdominal pain accompanied by progressive neurological impairment. Both patients were suspected of having appendicitis and underwent laparotomy, during which voluminous Meckel's diverticula were resected. Unusual neurotoxigenic Clostridium butyricum strains that produced botulinum-like toxin type E were isolated from the feces of the patients. These isolates were genotypically and phenotypically identical to other neurotoxigenic C. butyricum strains discovered in Italy in 1985-1986. No cytotoxic activity of the strains that might explain the associated gastrointestinal symptoms was demonstrated. The clinical picture of the illness and the persistence of neurotoxigenic clostridia in the feces of these patients suggested a colonization of the large intestine, with in vivo toxin production. The possibility that Meckel's diverticulum may predispose to intestinal toxemia botulism may warrant further investigation. - classic food poisoning but also the other clinical forms that result from colonization of the intestinal tract or from tissue infection with in vivo production of the toxin. Because both the infant and adult infectious intestinal forms occasionally involve clostridia other than C. botulinum, a new descriptive term, intestinal toxemia botulism, has been proposed for the infectious intestinal forms to distinguish them from the other 2 forms [8]. In Italy, although foodborne botulism [9], infant botulism [2], and wound botulism [10] have been described, cases of infant-like botulism in older children or adults have not thus far been reported. This last form of botulism has been well documented [7] and prevalently reported in the United States [8], where it has been described in a limited number of cases, almost all of which were associated with gut flora and pH altered by surgery and/or drugs. Herein we describe the clinical and microbiological findings from 2 unconnected cases of type E botulism, 1 in an adult and 1 in an older child, which we suspect resulted from intestinal colonization. The 2 cases occurred 1 year apart in different provinces of northern Italy and were associated with similar illnesses. In both cases, neurotoxigenic C. butyricum was isolated from fecal material. The phenotypic characteristics of the 2 strains (Istituto Superiore di Sanita` [ISS] CL 86 and CL 109), antibiotic resistance, sugar fermentation patterns, and other biochemical profiles were compared. Likewise, the genotypic characteristics of the 2 strains were investigated and compared with those of other C. butyricum strains (ISS CL 20 and CL 21) that produce type Type of botulism Foodborne Wound Infant Infant-like Types of botulism and their features. Ingestion of preformed botulinal toxin contained in improperly preserved food contaminated with spores of Clostridium botulinum type A, B, E, or F or Clostridium butyricum type E Production of toxin by C. botulinum type A or B that infected a wound Production of toxin in the intestine after germination and colonization by spores of C. botulinum type A, B, or C, Clostridium baratii type F, or C. butyricum type E Production of toxin in the intestine after germination and colonization by spores of C. botulinum type A or B, C. baratii type F, or C. butyricum type E E botulinumlike toxin, which were isolated from 2 cases of infant botulism that occurred in Italy 10 years earlier [2]. Finally, to investigate a possible relationship between the gastrointestinal illness observed in both patients and the neurotoxigenic C. butyricum strains isolated, we studied the possible cytotoxic effect of these clostridial culture supernatants on cell cultures [11]. Case Reports Case 1. A 9-year-old boy was hospitalized in Lugo di Romagna, near Ferrara in northern Italy, on 6 December 1994 because of acute abdominal pain and vomiting. Although afebrile, he was diagnosed initially with appendicitis. On day 3 after admission, although he was alert and fully oriented, his examination was notable for worsening diplopia, bilateral mydriasis, dysphonia, dry mouth, dry eyes, constipation, tympanic abdomen, urinary retention, tachycardia, tachypnea, and dyspnea. His worsening condition prompted transfer for intensive care at another hospital, where he immediately underwent laparotomy. He was found to have abundant ascites and a large, inflamed Meckels diverticulum, which was resected along with his appendix. Neither the diverticulum nor the ascites were retained for laboratory analysis. His intestinal loops were distended by gas and liquid feces. He was treated with ceftriaxone (1 g/day) and clavulanic acid (1 g/day) for 15 days after surgery. On day 5, he developed paralysis of the facial muscles and upper limbs and displayed sensory disturbances and dysautonomia. Because of progressive respiratory failure, he was placed on mechanical ventilation for 3 days. Electromyography displayed normal nerve conduction and facial nerve action potential amplitude. Although his physicians suspected either polyradiculoneuritis with dysautonomia or autoimmune disease of the CNS as the cause of weakness, botulism was also considered in the differential diagnosis. His routine blood tests were normal, and stool culture did not detect Salmonella, Shigella, or Campylobacter species, Yersinia enterocolitica, adenovirus, or rotavirus. No antibodies to Clinical description All ages affected; symmetrical descending paralysis that may progress rapidly; common symptoms are diplopia, blurred vision, disphagia, disphonia, and respiratory distress Presence of infected wound in the 2 weeks before onset of symptoms; no suspected food exposure; same symptoms as foodborne form but fever is often present Affects childrens aged !1 year; illness characterized by constipation, poor feeding, weak cry, loss of head control followed by progressive weakness, impaired respiration, and occasionally death Affects patients aged 112 months; illness clinically compatible with botulism, without history of ingestion of suspected food and without wounds CNS antigens were found. Serum obtained at admission and a rectal swab collected on hospital day 5 contained no detectable botulinum toxin or neurotoxigenic clostridia. Regular bowel movements resumed after intestinal canalization on day 13. His general condition then improv (...truncated)