Bickerstaff's brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. It is considered to be a variant of Miller Fisher syndrome and Guillain-Barré syndrome but is differentiated from the two by the presence of central nervous system involvement, commonly in the form of impaired consciousness. We present an unusual case of Bickerstaff's encephalitis, where the patient presented with pseudobulbar affect.
Two infants with severe varicella are reported. They received varicella zoster immunoglobulin (VZIG) without concurrent information to parents or carers regarding further care. In both these cases there was a three day delay between the onset of symptoms and initiation of aciclovir. This delay was due to lack of awareness of the high risk of varicella in these infants.Infants born to mothers with onset of chickenpox 4 days before to 2 days after delivery are at risk of fatal varicella, despite the use of VZIG prophylaxis. (Arch Dis Child Fetal Neonatal Ed 1999;81:F69-F70) Keywords: varicella zoster; immunoglobulin; prophylaxis
Case reports
CASE 1An Asian infant boy was taken to the local hospital at 11 days of age, by which time he had had a three day history of increasing feeding diYculties and lethargy. On the day of admission he developed diarrhoea and a rash. He had been delivered at term, one day after his mother had developed varicella. The baby had been given 250 mg intramuscular varicella zoster immunoglobulin (VZIG) at 4 hours of age and was breastfed.Examination showed that the baby had respiratory failure, was shocked, and jaundiced. He had an extensive erythematous maculo-papular rash with one vesicle present. He required immediate intubation, ventilation, and circulatory support and was given intravenous aciclovir and broad spectrum antibiotics.He required six days of ventilation, including three days of high frequency oscillatory ventilation (HFOV). Once extubated, he required facial oxygen for a further week.Immunofluorescence on endotracheal aspirate was strongly positive for varicella zoster virus (VZV), and VZV DNA was demonstrated by polymerase chain reaction of endotracheal aspirate, cerebrospinal and vesicle fluids. There were no positive bacterial cultures. The baby received a total of 16 days of intravenous aciclovir and made a complete recovery. He remained well 20 months later.
CASE 2An Asian boy was delivered normally at term, two days after his mother developed varicella. He was given 250 mg intramuscular VZIG on day 1. He first developed a rash at 10 days of age and was seen by the family practioner, who diagnosed heat rash. The rash persisted and two days later he again saw the GP, who prescribed calamine lotion and paracetamol. He became increasingly unwell and presented to the local accident and emergency department the following day with respiratory distress, irritability, poor feeding and hypoglycaemia. He had an extensive erythematous maculo-papular and vesicular rash. He required fluid resuscitation, intubation, and ventilation. He was ventilated for 14 days, including nine days of HFOV. He required intermittent nasal continuous positive airway pressure (nCPAP) for a further 12 days, and subsequently low flow oxygen via nasal cannulae for six weeks. He developed pancytopenia and clotting derangement and was treated with granulocyte colony stimulating factor, intravenous immunoglobulin, and received several blood transfusions.The baby also developed seizures. Computed...
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