1-3 The Terrace P.O Box 5013 Wellington Date: Tuesday 6th August, 2013 To: General Practitioners, Practice Nurses, Practice Managers, Health Professionals From: Dr Fran McGrath, Acting Director of Public Health Dr Pat Tuohy, Chief Advisor - Child & Youth Health Subject: Information on Botulism Pages: 1 of 3 Please regularly monitor the Ministry for Primary Industries (www.mpi.govt.nz) and Ministry of Health (www.health.govt.nz) websites for more information. Information for the public is available on the MoH website. Botulism is caused by the Botulinum neurotoxin (BoNT) produced by the bacteria Clostridium botulinum. There are 7 known types of BoNT (A to G) but only subtypes A, B, E and rarely F are known to cause disease in humans. The neurotoxin causes illness by irreversibly binding to presynaptic cholinergic receptors at motor nerve terminals. This leads to a disruption in the release of acetylcholine from the presynaptic nerve that is needed to excite the muscle. Clostridium botulinum bacteria are found throughout the environment in soil, dust and some marine environments. Clostridium botulinum spores are occasionally found in honey. Clinical presentation There are four naturally occurring forms of botulism; infant, foodborne, wound, and adult intestinal toxaemia botulism. Classical presentation is of a person who develops acute, bilateral cranial neuropathies with symmetrical descending weakness. Fever is usually absent, the neurologic complications are symmetrical, the patient remains responsive, the heart rate is slow or normal, and sensory deficits do not occur apart from blurred vision. Infant botulism: This more commonly affects under 6 month olds but can affect babies up to the age of 1 year. In this situation the intestinal flora is not fully developed and the spores ingested mature and start to release BoNT, which is absorbed by the intestine. Infant botulism may be difficult to recognise because of its insidious onset. Symptoms appear 3-30 days following ingestion. The sequence of symptoms and signs may vary but prolonged constipation (usually more than 3 days and can precede weakness by several weeks) is usually the first indication. Subsequent symptoms and clinical signs include: Poor feeding/weak suck Lethargy and floppiness Weak cry Facial weakness Impaired gag reflex 1 Over a week these may progress to include: Poor head control Trouble swallowing saliva with excessive drooling Generalised muscle weakness Loss of ocular motility, ptosis and sluggish pupils Difficulty breathing and respiratory difficulties are a late sign and can quickly lead to respiratory arrest. The condition progresses for 1-2 weeks, and then stabilises for 2-3 weeks before recovery starts The symptoms are those of acute flaccid paralysis and have a differential diagnosis of infantile poliomyelitis, sepsis, meningitis, encephalitis, Guillain-Barre syndrome, some metabolic disorders, poisoning from a range of toxins, and some other neuromuscular disorders. Foodborne botulism: This occurs when food has not been adequately processed or stored. The Clostridium botulinum bacteria prefer a low acid, low oxygen environment commonly found in preserved vegetables, meat and fish. This can affect anyone who eats these products at any age. The food will not taste or look spoiled. In this situation the bacteria in the food produce BoNT which is directly ingested. Symptoms occur within 12-36 hours post ingestion. Initial symptoms include: Nausea and vomiting Abdominal cramps and diarrhoea Symptoms progress and then include: Constipation Dry mouth Blurred vision and diplopia Dysphagia Dysarthria Hypoglossal weakness Respiratory dysfunction that may require ventilation. Hypothermia Urinary retention Loss of autonomic responsiveness to hypotension Recovery may not begin for 100 days and may take months to resolve. Wound botulism This occurs when an injury is infected with C. botulinum. The bacteria produce the neurotoxin which is absorbed through the wound. It has a similar presentation to foodborne botulism but without the initial gastric symptoms. 2 Adult Intestinal toxaemia botulism This is similar to infant botulism but affects adults and children who have altered gastrointestinal anatomy and microflora i.e. after intestinal surgery, inflammatory bowel disease, or congenital abnormalities. It is related to spore ingestion. Investigations Take a full history including suspicious food and other people potentially infected. Consider alternate diagnosis for acute flaccid paralysis. Take a stool sample if possible. If the differential diagnosis suggests C. botulinum sepsis then be aware that aminoglycosides can worsen the effects of the neurotoxin. If there is evidence of respiratory distress emergency management and respiratory support should not be delayed. Discuss with your local lab and microbiologist for preferred testing methods. Treatment Botulism can be treated and with prompt assessment and care. The majority fully recover. All suspected cases of botulism should be discussed with hospital specialist and microbiology/local infectious disease specialists, and reported to the local Medical Officer of Health - suspected botulism can be notified under the Health Act 1956 as acute flaccid paralysis (suspect polio) or acute gastroenteritis. All cases of Acute Flaccid Paralysis should be reported to the Paediatric Surveillance Unit in Dunedin. Contact details for the PSU are: Tel 3 474 7825 Fax 3 474 7817 Email nzpsu@otago.ac.nz The affected person will be monitored and assessed and a decision will be made regarding the appropriate use of the botulism antitoxin ‘Botulism Immune Globulin Intravenous’ (BIG-IV), Baby BIG® for infants. The neurotoxin effects wear off over time but the affected person may require intensive care and ventilation during this process. Antitoxin is available overseas if required. For more information see: http://www.foodsafety.govt.nz/elibrary/industry/Clostridium_Botulinum-Neurotoxins_Produced.pdf http://www.health.alberta.ca/documents/Guidelines-Botulism-2013.pdf http://emedicine.medscape.com/article/961833-overview CDC (US) > www.cdc.gov/nczved/divisions/dfbmd/diseases/botulism 3