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Human botulism studied with single-fiber electromyography.

In two cases of mild human botulism, conventional electromyography (EMG) was normal. In one case, the investigation with reptitive nerve stimulation showed slightly abnormal results, but in the other case the findings were within normal limits. Single-fiber EMG showed abnormal neuromuscular function and at later investigations as well, when the patient no longer showed any muscular fatigability. The jitter was frequency dependent and decreased with higher innervation frequency. The single-fiber EMG findings normalized after three months. The results are in agreement with the known disturbance of acetylcholine release.

Adult↗

Botulism in infancy. Report of a case.

A 22-day-old infant developed infant botulism characterized by profound weakness, hypotonia, respiratory arrest, areflexia, ptosis, pupils that responded poorly to light, and absent gag reflex. Stool examination yielded Clostridium botulinum type B organisms and type B toxin. Electromyography provided rapid diagnostic assistance. With supportive care, reovery was complete. This "new" disease probably is more common than now appreciated.

Botulism↗

Wound botulism in pediatrics.

A 6-year-old boy with congenital deafness sustained compound fractures to his left ulna and radius. Six days after the injury, he appeared lethargic, and his illness progressed to respiratory failure within three days. Although the wound at the fracture site appeared benign, cultures obtained when the wound was opened grew Clostridium botulinum, type B. Both the patient's deafness and the appearance of his wound contributed to the delay in diagnosis of wound botulism. Differential diagnosis and treatment of this rare entity are discussed.

Botulism↗

Wound botulism associated with black tar heroin among injecting drug users.

CONTEXT: Wound botulism (WB) is a potentially lethal, descending, flaccid, paralysis that results when spores of Clostridium botulinum germinate in a wound and elaborate neurotoxin. Since 1988, California has experienced a dramatic increase in WB associated with injecting "black tar" heroin (BTH), a dark, tarry form of the drug. OBJECTIVE: To identify risk factors for WB among injecting drug users (IDUs). DESIGN: Case-control study based on data from in-person and telephone interviews. PARTICIPANTS: Case patients (n=26) were IDUs who developed WB from January 1994 through February 1996. Controls (n=110) were IDUs newly enrolled in methadone detoxification programs in 4 counties. MAIN OUTCOME MEASURES: Factors associated with the development of WB. RESULTS: Among the 26 patients, the median age was 41.5 years, 15 (58%) were women, 14 (54%) were non-Hispanic white, 11 (42%) were Hispanic, and none were positive for the human immunodeficiency virus. Nearly all participants (96% of patients and 97% of controls) injected BTH, and the mean cumulative dose of BTH used per month was similar for patients and controls (27 g and 31 g, respectively; P=.6). Patients were more likely than controls to inject drugs subcutaneously or intramuscularly (92% vs 44%, P<.001) and used this route of drug administration more times per month (mean, 67 vs 24, P<.001), with a greater cumulative monthly dose of BTH (22.3 g vs 6.3 g, P<.001). A dose-response relationship was observed between the monthly cumulative dose of BTH injected subcutaneously or intramuscularly and the development of WB (chi2 for linear trend, 26.5; P<.001). In the final regression model, subcutaneous or intramuscular injection of BTH was the only behavior associated with WB among IDUs (odds ratio, 13.7; 95% confidence interval, 3.0-63.0). The risk for development of WB was not affected by cleaning the skin, cleaning injection paraphernalia, or sharing needles. CONCLUSIONS: Injection of BTH intramuscularly or subcutaneously is the primary risk factor for the development of WB. Physicians in the western United States, where BTH is widely used, should be aware of the potential for WB to occur among IDUs.

Adult↗

Cluster of wound botulism in California: clinical, electrophysiologic, and pathologic study.

Over a period of 15 months we have seen 6 patients with long-standing history of subcutaneous heroin injections who experienced acute blurred vision, dysphagia, dysarthria, and generalized weakness. Decreased or absent deep tendon reflexes, pupillary abnormalities, incremental responses to fast repetitive nerve stimulation, and positive serology for Clostridia botulinum toxin A were found, but not in all cases. Muscle biopsies showed variable signs of neurogenic atrophy. In vitro electrophysiology studies revealed decreased end-plate potentials quantal content, confirming the presynaptic nature of the disorder. Mechanical ventilation was required in 5 patients. Half of the patients were treated with polyvalent antitoxiin. Prognosis was favorable, though recovery was slow. In conclusion, acute bulbar weakness with visual symptoms in patients with subcutaneous heroin abuse strongly suggets the possibility of wound botulism. High diagnostic suspicion combined with histology and in vitro electrophysiology confirmation of presynaptic failure, especially in seronegative cases, may significantly improve morbidity.

Adult↗

Neurophysiological assessment in the diagnosis of botulism: usefulness of single-fiber EMG.

We report the clinical, serological, and neurophysiological findings in seven patients with foodborne botulism caused by ingestion of black olives in water. The clinical picture was characterized by mild symptoms with a long latency of onset and by involvement of cranial and upper limb muscles; only one patient, a child, developed respiratory failure. Spores of Clostridium botulinum were found in stools in some but not all cases. Conventional neurophysiological tests had low sensitivity; abnormal findings were present only in the patient with severe clinical involvement, in whom compound muscle action potentials (CMAPs) appeared reduced. Repetitive nerve stimulation at a high rate showed pseudofacilitation and not true posttetanic facilitation, but single-fiber electromyography (SFEMG) showed abnormalities of neuromuscular transmission in every case. Neurophysiological evaluation, particularly SFEMG, is important because it allows rapid identification of abnormal neuromuscular transmission while bioassay studies are in progress.

Action Potentials↗

Botulism in a patient with jejunoileal bypass.

A 45-year-old woman was diagnosed as having the unclassified form of botulism. Her intestines may have been predisposed to colonization with Clostridium botulinum because of a jejunoileal bypass procedure that had been done several years earlier. One other similar case has been reported.

Botulism↗

Reversible esophageal motor dysfunction in botulism.

Two cases of botulism with autonomic and neuromuscular system involvement are presented. In both patients, dryness of the mouth and difficulties in swallowing were predominant symptoms. Esophageal manometry revealed a marked decrease in peristaltic amplitude, which was most pronounced in the upper third of the esophagus. These functional abnormalities returned to normal following recovery from the acute disease.

Adolescent↗

Mosaic type of the nontoxic-nonhemaggulutinin component gene in Clostridium botulinum type A strain isolated from infant botulism in Japan.

The gene encoding the nontoxic-nonhemaggulutinin (NTNH) component was amplified by the PCR technique using two primer sets and the DNA template from Clostridium botulinum type A strain 7I03-H isolated from infant botulism in Japan. The nucleotide sequence revealed that the NTNH gene was composed of 1,193 amino acids with a molecular weight of 130868.08. Furthermore, the N-terminal half side and C-terminal half side of the NTNH component were similar to the NTNH component of type C and type A, respectively. These results indicate that the NTNH component gene codes the mosaic NTNH component composed of type A and type C. The hemaggulutinin gene, aha, and ORF-22 gene, orf-22a, were undetectable in the region upstream of the NTNH component gene, ant. Therefore, orf-22a is not thought to play a key role in the expression of botulinum type A progenitor toxin gene.

Amino Acid Sequence↗

Complete bilateral internal ophthalmoplegia as sole clinical sign of botulism: confirmation of diagnosis by single fibre electromyography.

A case of complete bilateral internal ophthalmoplegia as the sole clinical sign of botulism is reported. Diagnosis was immediately confirmed by single-fibre electromyography (SFEMG), which revealed abnormally high blocking (14.3%), contrasting with moderately increased jitter (mean consecutive difference in the extensor digitorum communis muscle, 43.9 microseconds). After giving equine botulinum antitoxin and simultaneous forced emptying of the bowels, ocular symptoms completely disappeared within 2 days. Six days, 5 weeks and 6 months after the first SFEMG study, the jitter was still abnormal, even becoming more so with time. Blocking, however, was only rarely observed in the follow-up studies. It is concluded that SFEMG may serve as a useful and sensitive method for the rapid diagnosis of botulinum intoxication, even in cases where no clinical signs of general muscular weakness are apparent.

Adult↗

Infant botulism: a rare cause of colonic ileus.

We have recently seen two patients with infant botulism, one of whom had radiologic evidence of autonomic and neuromuscular dysfunction. Both infants had been fed small amounts of honey, which is often contaminated with Clostridium botulinum spores, during the Jewish New Year celebration.

Autonomic Nervous System Diseases↗

Ocular involvement in benign botulism B.

In nine cases of botulism B infection due to food poisoning acute onset of accommodation paresis, mydriasis, and dry-eye symptoms were the prominent clinical findings. Impairment of salivary secretion as a further effect upon cholinergic autonomic innervation was detectable for months. Guanidine treatment had a beneficial effect, whereas administration of antitoxin in the late and benign form was not effective.

Accommodation, Ocular↗

Botulism: emotional impact on patient and family.

During the third largest outbreak of botulism reported in the United States, affective responses of patients and their family members were assessed to monitor and compare the emotional distress experienced by the two groups during the initial, acute phase of the life-threatening illness. Ratings of 12 patients and 16 family members indicated that family members were significantly more fearful and depressed than patients during the first week and as fearful and depressed as patients during the second week of hospitalization/treatment. Anxiety and helplessness decreased significantly in both groups by week 2. Results illustrate the impact of catastrophic illness on the entire family system and provide support for the utility of family-oriented, crisis interventions. In future outbreaks of catastrophic illness, the early identification of emotional distress and informational needs and provision of appropriate counselling to family members as well as patients may be clinically indicated and valuable in facilitating the coping process.

Adult↗

Ineffectiveness of 3,4-diaminopyridine as a therapy for type C botulism.

Clostridium botulinum neurotoxins inhibit acetylcholine release at neuromuscular junctions. Agents stimulating neurotransmitter efflux, such as 3,4-diaminopyridine (3,4-DAP), could be useful for botulism therapy. Treatment with 3,4-DAP (8 mg/kg hourly, beginning 3 hr after toxin injection) failed to increase the survival times of mice receiving 10, 20 or 40 LD50 type C, but did prolong the survival of those receiving 20 LD50 type A. This difference in 3,4-DAP efficacy may reflect variations in the molecular mechanism of action of types A and C botulinum neurotoxins.

4-Aminopyridine↗

Infant botulism: case report and clinical update.

Infant botulism occurs in infants between 1 week and 11 months of age and results from the in vivo production of neurotoxin by Clostridium botulinum. The clinical spectrum ranges from asymptomatic carriers, through various degrees of paralysis, to sudden death. The classic clinical presentation is an afebrile child with constipation and generalized weakness manifested by poor head control, poor suck, and weak cry. Symptoms can progress to include cranial nerve palsies, respiratory arrest, and adynamic ileus. Treatment is supportive in an intensive care setting. Antibiotics and antitoxin are not indicated. The morbidity and mortality is less than 3% in hospitalized patients and complete recovery can be expected. The environmental and dietary factors associated with infection are discussed.

Botulism↗

Infant botulism: a case series and review of the literature.

Little has been written about infant botulism in the emergency medicine literature, despite increasing references in the pediatric journals. We describe three cases diagnosed at The Children's Hospital in Denver, Colorado, over an 8-month period. A review of the literature follows, to discuss the clinical manifestations, differential diagnosis, diagnostic workup and treatment of the disease.

Acute Disease↗

[Botulism in a neonate].

Botulism was suspected in a 17-day-old breastfed infant who developed over 2 days progressive muscular weakness and hypoventilation. The patient also presented with pupil dilation and light unresponsiveness. The electroencephalogram was normal. Full recovery was obtained after 85 days of artificial ventilation. Diagnosis was confirmed by the presence of the botulin toxin B in the patient serum. The source of the infection was not identified.

Botulinum Toxins↗

Potentiation of neuromuscular weakness in infant botulism by aminoglycosides.

A retrospective study of ten patients with infant botulism who received gentamicin or kanamycin suggests that aminoglycoside antibiotics potentiate muscular weakness and precipitate respiratory failure as late as 27 days after onset of the disease. Although it is difficult to separate progression of the disease from the effects of antibiotics, the rapidity of deterioration following aminoglycoside treatment and the rapidity of recovery following cessation of aminoglycoside therapy is highly suggestive. A review of five patients who received only penicillin or a semisynthetic derivative of penicillin did not reveal any temporal deterioration with onset of penicillin therapy or improvement with cessation of penicillin therapy.

Ampicillin↗