A term, previously well, male infant presented to his hospital aged 2 months with a 6-day history of poor feeding, constipation and irritability. He was less interactive with poor fixing and following. He was dehydrated on examination and had fluid resuscitation and antibiotics for presumed sepsis.
He was a thriving, breastfed infant with a regular bowel pattern. Of note, there was a background social history of maternal heroin use in the first trimester and ongoing methadone dependency.
He was transferred to the regional tertiary neurology centre for investigation of generalised weakness. He was found to be globally hypotonic with poor head control and spontaneous but weak movement of all four limbs. There were upper but no lower limb reflexes. He had a poor suck, no rooting reflex and reduced gag reflex. He had no cry though vocalised weakly. Funduscopy revealed no papilloedema or retinal haemorrhages. Pupils were equal and reactive but he was unable to fix or follow.
He was started on gentamicin, cefotaxime, aciclovir and dexamethasone for a presumed diagnosis of encephalopathy. These were discontinued following negative cerebrospinal fluid and blood cultures. Nasopharyngeal secretions were positive for rhinovirus and a chest x-ray (CXR) showed mild right-middle lobe opacification. Investigations for his neurological pathology are detailed in . He became pale and hypotonic a few days later necessitating admission to the high dependency unit. His CXR revealed ongoing right-middle zone changes and he was started on co-amoxiclav and continuous positive airway pressure (CPAP). He continued intermittently on CPAP for a number of days and appeared to show signs of improvement.
On day 16 of admission he had abnormal movements in keeping with a generalised tonic-clonic seizure and was treated with midazolam and phenytoin. His electroencephalography was entirely normal. A tensilon test was performed, which was also normal, and an EMG suggested central rather than peripheral weakness.
He was admitted to the paediatric intensive care unit (PICU) on day 32 following a profound apnoea and desaturation requiring intubation and ventilation. Given the deteriorating nature of his condition and negative investigations at that time, he was thought to have a neurodegenerative condition with a poor prognosis. End-of-life care was discussed with parents and arrangements made for baptism.
In attempts to reach a definitive diagnosis, a MRI scan was repeated and was again normal. Neurophysiology was repeated and revealed a changing pattern in keeping with a peripheral myopathic process. Therefore infantile botulism could not be excluded and a stool sample was sent to the national reference laboratory. On day 41 of admission, Clostridium botulinum type A was isolated by PCR on his stool sample. The diagnosis of infantile botulism was made and public health authorities informed. History from both parents revealed the infant had been getting honey on his soother at home. A sample of the honey from the jar used subsequently tested positive for C botulinum type A. Given the duration of symptoms prior to diagnosis, the decision was made not to administer immunoglobulin.
Seven weeks after initial presentation the infant failed a trial of extubation and showed no signs of sustained neurological improvement having initially recovered some power in his limbs. A tracheostomy was performed and he remained dependant on CPAP ventilation 3 months after presentation. He continued to secrete C botulinum in stool samples tested weekly.