Botulinum toxin type A treatment in neurogenetic syndromes

PURPOSE: To review the use of therapeutic botulinum toxin type A (BoNT-A) treatments in uncommon neurogenetic syndromes. METHOD: A retrospective questionnaire and interview study of a selected case series to assess the efficacy and safety following initial BoNT-A treatment (50-400 units per subject) was conducted to determine the response of families to treatment. Twelve male and six female subjects with ages from 2-19 years were included. The reasons for treatments were based on both patient-related and caregiver-related objectives. Satisfaction with achievement of stated goals was assessed by follow-up interviews. RESULTS: Beneficial effects were reported in 56%, some effects in 22% and no to minimal effects in 22%. The duration of effect ranged from 10 days to 12 months with an average of 3.16 months. Ten families would repeat the injections as needed, four would not and four were not sure. Unanticipated effects of BoNT-A treatments were reported by some families. Adverse effects did not occur with the doses that were used. CONCLUSIONS: The results suggest that obtaining family input may be useful when treating spasticity in unusual circumstances. The use of BTX-A in uncommon neurogenetic syndromes was supported by the majority of families interviewed.     

Staphylococcal scalded skin syndrome related to an exfoliative toxin A- and B-producing strain in preterm infants

A previously well, spontaneously breathing premature infant (gestational age 25 weeks, birth weight 364 g, age 74 days) developed staphylococcal scalded skin syndrome (SSSS). A methicillin-sensitive strain of Staphylococcus aureus producing exfoliative toxins A and B (ETA, ETB) was isolated from a gastric aspirate and a pharyngeal swab. The disease recurred with a milder clinical picture 4 weeks later in the same patient while under steroid treatment. Cultures obtained from conjunctiva and pharynx were again positive for S. aureus. A second premature infant in an adjacent ward developed SSSS 2 weeks after the recurrence in the first patient. No other cases were observed thereafter. A total of 25 individuals who had contact with the first patient were screened for staphylococcal colonisation. S.aureus was isolated from the posterior part of the nasal cavity in 8 of the 25 contacts. These strains and the strain of the first patient were evaluated by PCR for the presence of genes encoding ETA and ETB. Expression of toxins was confirmed by gel electrophoresis and Western blot analysis. Purified toxins were injected into newborn mice to confirm toxin activity. Besides the strain isolated from the first patient, only one isolate from the medical staff was positive for the genes encoding ETA and ETB. CONCLUSION: the carrier of this strain had contact with both patients, suggesting that this individual was the vector between the two patients but not necessarily the source of the original infection. Strict infection control measures were implemented and no further spread of the disease occurred.     

Staphylococcal toxic shock

The authors report the case of a 5 year-old child who, after a sore throat, developed gastrointestinal problems, high fever, scarlatinaform rash, conjunctivitis, shock with renal failure and involvement of liver, pancreas and muscles. No infectious site was detected. However, he had positive blood culture for staphylococcus aureus. The child fully recovered after a period of desquamation of the palms and soles.     

Staphylococcal scalded skin syndrome: exfoliative toxin A (ETA) induces serine protease activity when combined with A431 cells

Staphylococcal scalded skin syndrome is the term used for a spectrum of primarily neonatal blistering skin diseases caused by the exfoliative toxins, ETA and ETB, of Staphylococcus aureus